Sample records for hemolytic anemia due

  1. Hemolytic anemias due to erythrocyte enzyme deficiencies.

    PubMed

    Jacobasch, G; Rapoport, S M

    1996-04-01

    Red blood cells can only fulfil their functions over the normal period of approximately 120 days with 1.7 x 10(5) circulatory cycles efficiently if they withstand external and internal loads. This requires ATP and redox equivalents, which have to be permanently regenerated by the energy and redox metabolism. These pathways are necessary to maintain the biconcave shape of the cells, their specific intracellular cation concentrations, the reduced state of hemoglobin with a divalent iron and the sulfhydryl groups of enzymes, glutathione and membrane components. If an enzyme deficiency of one of these metabolic pathways limits the ATP and/or NADPH production, distinct membrane alterations result causing a removal of the damaged cells by the monocyte-macrophage system. Most metabolic needs of erythrocytes are covered by glycolysis, the oxidative pentose phosphate pathway (OPPP), the glutathione cycle, nucleotide metabolism and MetHb reductase. Hereditary enzyme deficiencies of all these pathways have been identified; those that cause non-spherocytic hemolytic anemia are listed in Table 4. Their frequencies differ markedly both with respect to the affected enzyme and geographic distribution. Glucose-6-phosphate dehydrogenase enzymopathies (G6PD) are with more than 400 million cases by far the most common deficiency. The highest gene frequency has been found with 0.7 among Kurdish Jews. G6PD deficiencies are furthermore prevalent with frequencies of about 0.1 among Africans, Black Americans, and populations of Mediterranean countries and South East Asia. In Middle and Northern Europe the frequency of G6PD is much lower, and with approximately 0.0005, comparable with the frequency of pyruvate kinase (PK) enzymopathies, the most frequent enzyme deficiency in glycolysis in this area (Luzzatto, 1987; Beutler and Kuhl, 1990). The relationship between the degree of enzyme deficiency and the extent of metabolic dysfunction in red blood cells and other tissues depend on several factors: on the importance of the affected enzyme; its expression rate; the stability of the mutant enzyme against proteolytic degradation and functional abnormalities; the possibility to compensate the deficiency by an overexpression of the corresponding isoenzyme or by the use of an alternative metabolic pathway. Difficulties in estimating the quantitative degree of disorder in severe cases are due to the fact that these populations contain many reticulocytes, which generally have higher enzyme activities and concentrations of intermediates than erythrocytes. An alternative approach to predict metabolic changes is the analysis by mathematical modeling. Mathematical modeling of the main metabolic pathways of human erythrocytes has reached an advanced level (Rapoport et al., 1976; Holzhütter et al., 1985; Schuster et al., 1988). Models have been successfully employed to describe stationary and time-dependent metabolic states of the cell under normal conditions as well as in the presence of enzyme deficiencies. Figure 5 shows computational results of erythrocyte enzyme deficiencies. This analysis is based on the comprehensive mathematical model of the energy and redox metabolism for human erythrocyte presented in Fig. 6. Stationary states of the cell metabolism have been calculated by varying the activity of each of the participating enzymes by several orders of magnitude. To predict consequences of enzyme deficiencies a performance function has been introduced (Schuster and Holzhütter, 1995). It takes into account the homeostasis of three essential metabolic variables: the energetic state (ATP), the reductive capacity (reduced glutathione) and the osmotic state. From the data given in Fig. 5 one can conclude that generally the metabolic impairment resulting in deficiencies occurs earlier for enzymes with high control coefficients than for those catalyzing equilibrium reactions. On the other hand the flux curves of latter enzymes decrease more steeply below a critica PMID:8813716

  2. Coomb’s Positive Hemolytic Anemia Due To Insect Bite

    PubMed Central

    2007-01-01

    Hemolytic anemia has occasionally been described in association with insect bites. The venom of certain spiders, bees and wasps, and some snakes can rarely cause intravascular hemolysis. We report here a case of Coombs positive hemolytic anemia due to an insect bite. These bites often pose diagnostic challenges and when associated with systemic manifestations necessitate early intervention. This communication reviews the clinico- hematologic spectrum in these cases and also emphasizes the need to capture the insect as identification would help in early diagnosis and management. PMID:22400097

  3. [Autoimmune hemolytic anemia].

    PubMed

    Marmont, A; Zanella, A

    2000-10-01

    Autoimmune hemolytic anemias (AIHAs) are the most ancient and well known example of clinical autoimmunity. They may be still distinguished in two main groups, that is with "warm" or "cold" antibodies, according to the optimal temperature at which they react with the erythrocyte antigens in vivo and in vitro. There is also a subgroup where both kinds of autoantibodies coexist. AIHAs may be idiopathic or secondary. The immunologic techniques for the demonstration of the antibodies are well established, but one must remember that there are infrequent cases with negative DAT (Coombs) test when performed with conventional procedures. The fundamental concepts of therapy are discussed, and the detailed review of the principal procedures is performed, including blood transfusions (when and how), plasma exchanges, high-dose immunoglobulins, glycocorticosteroids, splenectomy, cytotoxic agents and stem cell transplantation, autologous and allogeneic. PMID:11072741

  4. Fatal autoimmune hemolytic anemia due to immunoglobulin g autoantibody exacerbated by epstein-barr virus.

    PubMed

    Fadeyi, Emmanuel A; Simmons, Julie H; Jones, Mary Rose; Palavecino, Elizabeth L; Pomper, Gregory J

    2015-01-01

    Most cases of autoimmune hemolytic anemia (AIHA) are caused by the production of an autoantibody that targets determinants on red blood cells (RBCs). This autoantibody can be immunoglobulin (Ig) G, IgM, or IgA. Some autoantibodies react optimally at 0° to 4°C (ie, cold agglutinin) and usually are clinically insignificant. High-titer cold agglutinins are associated with IgM autoantibody and complement fixation induced by infectious agents, including the Epstein-Barr virus (EBV). This case report describes a 31-year-old man who had jaundice, a hemoglobin of 6.0 gdL, and was diagnosed with a hemolytic crisis of AIHA. He received a total of 11 RBC transfusions during a 15-hour period without sustained response and later died. The direct antiglobulin test results for this patient were positive, whereas the cold-agglutinin-testing results were negative. We detected EBV DNA in blood via polymerase chain reaction (PCR). We report a rare case of AIHA associated with an IgG autoantibody and exacerbated by EBV infection, causing a fatal hemolytic anemia. PMID:25617394

  5. Hemolytic anemia in dogs and cats due to erythrocyte enzyme deficiencies.

    PubMed

    Owen, Jennifer L; Harvey, John W

    2012-01-01

    Erythrocyte enzyme deficiencies do not usually shorten life expectancy except for PK deficiency in dogs and the potential for PFK-deficient dogs to die during hemolytic crises. In addition, erythrocyte enzyme deficiencies are uncommon or rare, so they are generally not seriously considered in the differential diagnosis of anemia until common causes of anemia have been excluded. However, unique clinical and/or laboratory findings like sporadic hemoglobinuria in English Springer spaniels (PFK deficiency) may quickly point to the possibility of an inherited erythrocyte enzyme defect. The ability to diagnose deficient or carrier animals allows for the possibility of eliminating these undesirable traits in future breeding. Continued research is needed to document additional enzyme deficiencies that likely occur and to develop additional DNA-based assays that are especially important in the recognition of heterozygous or carrier animals that have no clinical signs. PMID:22285158

  6. Drug-induced immune hemolytic anemia

    MedlinePLUS

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... In some cases, a drug can cause the immune system to mistake your own red blood cells for foreign substances. The body responds by making ...

  7. Thymoma with Autoimmune Hemolytic Anemia

    PubMed Central

    Suzuki, Kensuke; Inomata, Minehiko; Shiraishi, Shiori; Hayashi, Ryuji; Tobe, Kazuyuki

    2014-01-01

    A 38-year-old Japanese male was referred to our hospital with abnormal chest X-ray results and severe Coombs-positive hemolytic anemia. He was diagnosed with a stage IV, WHO type A thymoma and was treated with oral prednisolone (1 mg/kg/day) and subsequent chemotherapy. After chemotherapy, the patient underwent surgical resection of the thymoma. Hemolysis rapidly disappeared and did not return after the discontinuation of oral corticosteroids. Corticosteroid therapy may be preferable to chemotherapy or thymoma surgical resection in the management of autoimmune hemolytic anemia with thymoma. PMID:25722666

  8. Mutations in the R-type pyruvate kinase gene and altered enzyme kinetic properties in patients with hemolytic anemia due to pyruvate kinase deficiency

    Microsoft Academic Search

    M. Lakomek; P. Huppke; B. Neubauer; A. Pekrun; H. Winkler; W. Schröter

    1994-01-01

    Summary The biochemical properties of erythrocyte pyruvate kinase (PK) together with mutations found in the coding sequence of the R-PK gene in five patients with severe hemolytic anemia due to PK deficiency are described. The enzyme variants were designated PK ‘Mosul’ (homozygote), PK ‘Bukarest1,2’, PK ‘Hamburg1’, PK ‘Köln1’, and PK ‘Essen’ (compound heterozygote). PK ‘Mosul’ showed normal positive cooperative substrate

  9. Treatment of autoimmune hemolytic anemias

    PubMed Central

    Zanella, Alberto; Barcellini, Wilma

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. The first-line therapy for warm AIHA are corticosteroids, which are effective in 70–85% of patients and should be slowly tapered over a time period of 6–12 months. For refractory/relapsed cases, the current sequence of second-line therapy is splenectomy (effective approx. in 2 out of 3 cases but with a presumed cure rate of up to 20%), rituximab (effective in approx. 80–90% of cases), and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases. In CAD, rituximab is now recommended as first-line treatment. PMID:25271314

  10. Treatment of autoimmune hemolytic anemias.

    PubMed

    Zanella, Alberto; Barcellini, Wilma

    2014-10-01

    Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. The first-line therapy for warm AIHA are corticosteroids, which are effective in 70-85% of patients and should be slowly tapered over a time period of 6-12 months. For refractory/relapsed cases, the current sequence of second-line therapy is splenectomy (effective approx. in 2 out of 3 cases but with a presumed cure rate of up to 20%), rituximab (effective in approx. 80-90% of cases), and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases. In CAD, rituximab is now recommended as first-line treatment. PMID:25271314

  11. [Autoimmune hemolytic anemia and CREST syndrome].

    PubMed

    Jordana, R; Tolosa, C; Selva, A; Ordi, J

    1990-05-19

    There are few studies addressing the frequency and etiology of anemia in progressive systemic sclerosis. Anemia is present in about 25% of these patients. Among the implicated causes are ferropenia, generally associated with gastrointestinal hemorrhage, and renal failure. In a considerable number of patients, the specific etiology of anemia remained unknown. We report a 59-year-old female with autoimmune hemolytic anemia and incomplete CREST syndrome. We emphasize the rarity of autoimmune hemolysis as a cause of anemia in systemic sclerosis, and we discuss its significance as indirectly supporting the possible autoimmune pathogenesis of that condition. PMID:2201843

  12. How Is Hemolytic Anemia Treated?

    MedlinePLUS

    ... medicines rituximab and cyclosporine. If you have severe sickle cell anemia , your doctor may recommend a medicine ... hemoglobin that newborns have. In people who have sickle cell anemia, fetal hemoglobin helps prevent red blood ...

  13. Immunotherapy Treatments of Warm Autoimmune Hemolytic Anemia

    PubMed Central

    Gu, Wangang

    2013-01-01

    Warm autoimmune hemolytic anemia (WAIHA) is one of four clinical types of autoimmune hemolytic anemia (AIHA), with the characteristics of autoantibodies maximally active at body temperature. It produces a variable anemia—sometimes mild and sometimes severe. With respect to the absence or presence of an underlying condition, WAIHA is either idiopathic (primary) or secondary, which determines the treatment strategies in practice. Conventional treatments include immune suppression with corticosteroids and, in some cases, splenectomy. In recent years, the number of clinical studies with monoclonal antibodies and immunosuppressants in the treatment of WAIHA increased as the knowledge of autoimmunity mechanisms extended. This thread of developing new tools of treating WAIHA is well exemplified with the success in using anti-CD20 monoclonal antibody, Rituximab. Following this success, other treatment methods based on the immune mechanisms of WAIHA have emerged. We reviewed these newly developed immunotherapy treatments here in order to provide the clinicians with more options in selecting the best therapy for patients with WAIHA, hoping to stimulate researchers to find more novel immunotherapy strategies. PMID:24106518

  14. Diagnosis and classification of autoimmune hemolytic anemia.

    PubMed

    Bass, Garrett F; Tuscano, Emily T; Tuscano, Joseph M

    2014-01-01

    Uncompensated autoantibody-mediated red blood cell (RBC) consumption is the hallmark of autoimmune hemolytic anemia (AIHA). Classification of AIHA is pathophysiologically based and divides AIHA into warm, mixed or cold-reactive subtypes. This thermal-based classification is based on the optimal autoantibody-RBC reactivity temperatures. AIHA is further subcategorized into idiopathic and secondary with the later being associated with a number of underlying infectious, neoplastic and autoimmune disorders. In most cases AIHA is confirmed by a positive direct antiglobulin test (DAT). The standard therapeutic approaches to treatment of AIHA include corticosteroids, splenectomy, immunosuppressive agents and monoclonal antibodies. PMID:24418298

  15. Hemolytic Anemia Following Rasburicase Administration: A Review of Published Reports

    PubMed Central

    Nguyen, Annhien P.

    2014-01-01

    Tumor lysis syndrome (TLS) is a potentially lethal complication of anticancer treatment. It is caused by the rapid death of malignant cells after initiation of cytotoxic therapy and is typically observed in patients with bulky or highly proliferative malignancies. Currently, rasburicase is one of the recommended therapies for this oncologic emergency. Although this drug is generally well tolerated among patients, there have been several reports of hemolytic anemia following rasburicase infusions. With drug-induced hemolytic anemia, the condition usually resolves shortly after the offending agent is discontinued. However, anemia that is prolonged or severe can lead to problems such as splenomegaly and rapid heart rate. This paper will review primary literature identified through PubMed, International Pharmaceutical Abstracts, and Embase concerning the incidence of hemolytic anemia with rasburicase use. From the available data, the occurrence of hemolytic anemia will be discussed.

  16. Dapsone-induced methemoglobinemia and hemolytic anemia.

    PubMed

    Erstad, B L

    1992-09-01

    The treatment of two common adverse effects of dapsone (methemoglobinemia and hemolytic anemia) is discussed, and a case of acute dapsone intoxication is described. A pregnant 29-year-old woman was admitted to an emergency room three hours after ingesting 50 tablets of dapsone (100 mg each) and six alcoholic drinks. One hour after admission 50 g of activated charcoal was given p.o., and 65 mg of methylene blue was given i.v. The patient was found to have a methemoglobin concentration of 25.1%. Arterial blood gases while the patient was breathing 4 L/min of oxygen by nasal cannula were PO2, 136 mm Hg (72.1% saturation); PCO2, 28.9 mm Hg; bicarbonate content, 18.9 mmol/L; and pH, 7.42. Oxygen therapy was changed to 100% oxygen by face mask, 50 g of activated charcoal in sorbitol was administered p.o., and another 65 mg of methylene blue was given i.v. Two more 50-g doses of activated charcoal in sorbitol were given (18.5 and 22 hours after dapsone ingestion). Methylene blue 130 mg was given 14 hours after dapsone ingestion, and 65 mg was given 21, 36, and 55.5 hours after ingestion. Methemoglobin concentrations never rose above 20% after the sixth dose of methylene blue. On hospital days 2 and 3, laboratory values were consistent with a diagnosis of hemolytic anemia; the patient received two units of packed red blood cells. The hematocrit decreased over the next three days to 23.9%, and the patient received four units of packed red blood cells.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1521404

  17. Current approaches for the treatment of autoimmune hemolytic anemia.

    PubMed

    Jaime-Pérez, José Carlos; Rodríguez-Martínez, Marisol; Gómez-de-León, Andrés; Tarín-Arzaga, Luz; Gómez-Almaguer, David

    2013-10-01

    Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell destruction. Correct diagnosis and classification of this condition are essential to provide appropriate treatment. AIHA is divided into warm and cold types according to the characteristics of the autoantibody involved and by the presence of an underlying or associated disorder into primary and secondary AIHA. Due to its low frequency, treatment for AIHA is largely based on small prospective trials, case series, and empirical observations. This review describes in detail the different treatment approaches for autoimmune hemolytic anemia. Warm antibody type AIHA should be treated with steroids, to which most patients respond, although relapse can occur and maintenance doses are frequently required. Splenectomy is an effective second line treatment and can provide long-term remission without medication. Rituximab is a useful alternative for steroid refractory patients, those requiring high maintenance doses and unfavorable candidates for surgery. Promising therapeutic modifications with this monoclonal antibody are emerging including drug combinations, lower doses, and long-term use. Primary cold agglutinin disease has been recognized as having a lymphoproliferative monoclonal origin. It is unresponsive to both steroids and splenectomy. Rituximab is currently the best therapeutic alternative for this condition, and several treatment regimens are available with variable responses. PMID:23689532

  18. Autoimmune hemolytic anemia: From lab to bedside.

    PubMed

    Chaudhary, R K; Das, Sudipta Sekhar

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT) still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The "best match" or "least incompatible units" can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue "best match" packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services. PMID:24678166

  19. Autoimmune hemolytic anemia: From lab to bedside

    PubMed Central

    Chaudhary, R. K.; Das, Sudipta Sekhar

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT) still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The “best match” or “least incompatible units” can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue “best match” packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services. PMID:24678166

  20. Autoimmune hemolytic anemia in patients with ?-thalassemia major.

    PubMed

    Xu, Lu-Hong; Fang, Jian-Pei; Weng, Wen-Jun; Huang, Ke; Zhang, Ya-Ting

    2012-04-01

    Hemolysis is a common feature in patients with ?-thalassemia major. As a result, autoimmune hemolytic anemia complicating ?-thalassemia is easily overlooked. Here, the authors described the clinical features and management of 7 patients with ?-thalassemia major and autoimmune hemolytic anemia. These patients had fever, cough, and tea-colored urine on admission. The laboratory investigations showed a significant drop in hemoglobin and increased serum bilirubin. Coombs' tests revealed that anti-immunoglobulin G (IgG) and anti-C3 was positive in 7 and 5 cases, respectively, whereas anti-Rh E alloantibody was positive in 3 cases. All the patients received corticosteroids treatments and blood transfusions. Patients with anti-Rh E alloantibodies also received immunoglobulin treatments. Six of the patients responded well to the management, but 1 patient developed recurrent autoimmune hemolytic anemia that required cyclosporin A treatment. All the patients remained well by following up for more than 6 months. PMID:22475299

  1. Impairment of Bone Health in Pediatric Patients with Hemolytic Anemia

    PubMed Central

    Schündeln, Michael M.; Goretzki, Sarah C.; Hauffa, Pia K.; Wieland, Regina; Bauer, Jens; Baeder, Lena; Eggert, Angelika; Hauffa, Berthold P.; Grasemann, Corinna

    2014-01-01

    Introduction Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may also display impaired bone health. Study Design To assess bone health in pediatric patients with chronic hemolytic anemia, a cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i.e., 17 homozygous sickle cell disease and 14 hereditary spherocytosis patients). Biochemical, radiographic and anamnestic parameters of bone health were assessed. Results Vitamin D deficiency with 25 OH-vitamin D serum levels below 20 ng/ml was a common finding (80.5%) in this cohort. Bone pain was present in 31% of patients. Analysis of RANKL, osteoprotegerin (OPG) and osteocalcin levels indicated an alteration in bone modeling with significantly elevated RANKL/OPG ratios (control: 0.08+0.07; patients: 0.26+0.2, P?=?0.0007). Osteocalcin levels were found to be lower in patients compared with healthy controls (68.5+39.0 ng/ml vs. 118.0+36.6 ng/ml, P?=?0.0001). Multiple stepwise regression analysis revealed a significant (P<0.025) influence of LDH (partial r2?=?0.29), diagnosis of hemolytic anemia (partial r2?=?0.05) and age (partial r2?=?0.03) on osteocalcin levels. Patients with homozygous sickle cell anemia were more frequently and more severely affected by impaired bone health than patients with hereditary spherocytosis. Conclusion Bone health is impaired in pediatric patients with hemolytic anemia. In addition to endocrine alterations, an imbalance in the RANKL/OPG system and low levels of osteocalcin may contribute to this impairment. PMID:25299063

  2. Red blood cell vesiculation in hereditary hemolytic anemia

    PubMed Central

    Alaarg, Amr; Schiffelers, Raymond M.; van Solinge, Wouter W.; van Wijk, Richard

    2013-01-01

    Hereditary hemolytic anemia encompasses a heterogeneous group of anemias characterized by decreased red blood cell survival because of inherited membrane, enzyme, or hemoglobin disorders. Affected red blood cells are more fragile, less deformable, and more susceptible to shear stress and oxidative damage, and show increased vesiculation. Red blood cells, as essentially all cells, constitutively release phospholipid extracellular vesicles in vivo and in vitro in a process known as vesiculation. These extracellular vesicles comprise a heterogeneous group of vesicles of different sizes and intracellular origins. They are described in literature as exosomes if they originate from multi-vesicular bodies, or as microvesicles when formed by a one-step budding process directly from the plasma membrane. Extracellular vesicles contain a multitude of bioactive molecules that are implicated in intercellular communication and in different biological and pathophysiological processes. Mature red blood cells release in principle only microvesicles. In hereditary hemolytic anemias, the underlying molecular defect affects and determines red blood cell vesiculation, resulting in shedding microvesicles of different compositions and concentrations. Despite extensive research into red blood cell biochemistry and physiology, little is known about red cell deformability and vesiculation in hereditary hemolytic anemias, and the associated pathophysiological role is incompletely assessed. In this review, we discuss recent progress in understanding extracellular vesicles biology, with focus on red blood cell vesiculation. Also, we review recent scientific findings on the molecular defects of hereditary hemolytic anemias, and their correlation with red blood cell deformability and vesiculation. Integrating bio-analytical findings on abnormalities of red blood cells and their microvesicles will be critical for a better understanding of the pathophysiology of hereditary hemolytic anemias. PMID:24379786

  3. Idiopathic immune-mediated hemolytic anemia in a calf.

    PubMed

    Fenger, C K; Hoffsis, G F; Kociba, G J

    1992-07-01

    Severe anemia was found in a 4-month-old heifer, which was admitted with a 1-day history of anorexia, signs of depression, and recumbency. A diagnosis of immune-mediated hemolytic anemia (IHA) was made on the basis of a Coomb's titer of 1:128 and decreased resistance to osmotic stress, as determined by an RBC fragility test. Anaplasmosis and leptospirosis were ruled out as possible causes of the IHA. Other causes of hemolytic anemia, including intoxication by copper, water, Brassica spp, or drugs were ruled out. Therefore the IHA was considered idiopathic. Treatment consisted of supportive therapy, oxytetracycline, and dexamethasone. After 60 days of treatment, CBC, Coomb's test result, and RBC fragility were within normal limits. PMID:1644656

  4. Hemolytic anemia and metabolic acidosis: think about glutathione synthetase deficiency.

    PubMed

    Ben Ameur, Salma; Aloulou, Hajer; Nasrallah, Fehmi; Kamoun, Thouraya; Kaabachi, Naziha; Hachicha, Mongia

    2015-02-01

    Glutathione synthetase deficiency (GSSD) is a rare disorder of glutathione metabolism with varying clinical severity. Patients may present with hemolytic anemia alone or together with acidosis and central nervous system impairment. Diagnosis is made by clinical presentation and detection of elevated concentrations of 5-oxoproline in urine and low glutathione synthetase activity in erythrocytes or cultured skin fibroblasts. The prognosis seems to depend on early diagnosis and treatment. We report a 4 months old Tunisian male infant who presented with severe metabolic acidosis with high anion gap and hemolytic anemia. High level of 5-oxoproline was detected in her urine and diagnosis of GSSD was made. Treatment consists of the correction of acidosis, blood transfusion, and supplementation with antioxidants. He died of severe metabolic acidosis and sepsis at the age of 15 months. PMID:25166299

  5. [Hemolytic anemia and thrombocytopenia associated with anti-omeprazole antibody].

    PubMed

    Hayashibara, T

    1998-06-01

    An 80-year-old woman was admitted with anemia, jaundice and a bleeding tendency about 5 weeks after starting omeprazole. On admission, the hemoglobin was 6.4 g/dl, platelets 0.1 x 10(4)/microliter, leukocyte count 7,500/microliter, and reticulocyte count 325/1000. The total bilirubin was 1.9 mg/dl, indirect bilirubin 0.6 mg/dl, lactate dehydrogenase 572 IU/l, and haptoglobin < 10 mg/dl. Both the direct and the indirect Coombs' tests were positive. The platelet-associated IgG (PAIgG) was 1,100.0 ng/10(7) cells. A decrease in the complement value was observed. There was an increase in the number of megakaryocytes and erythroblasts in the marrow film. After omeprazole administration was halted, her hemoglobin and platelet levels gradually returned to normal. On the 27th hospital day, the direct Coombs' test was positive but the indirect Coombs' test became negative. The PAIgG value also returned to normal, and she was discharged on the 59th hospital day. The acute phase of the drug-induced lymphocyte stimulation test was negative, however, we detected the IgG antibody to omeprazole. In the recovery phase, the IgG value decreased. Forty days after discharge, the direct Coombs' test had become negative. This is apparently the first report of a patient with acute hemolytic anemia and thrombocytopenia due to omeprazole through an immune complex mechanism. PMID:9695674

  6. Hemolytic anemia with impaired hexokinase activity

    PubMed Central

    Keitt, Alan S.

    1969-01-01

    Analyses of key glycolytic intermediates in freshly drawn red cells from six related individuals suggest that decreased hexokinase activity underlies the hemolytic process in the two members with overt hemolysis. Low red cell glucose 6-phosphate (G6P) was observed not only in the anemic patients but in the presumptive heterozygotes as well and served as a useful marker for the presence of the trait. Hexokinase activity was labile in distilled water hemolysates but was only slightly low when protected by glucose, mercaptoethanol, and ethylenediaminetetraacetate (EDTA). Normal red cell hexokinase was demonstrated to be dependent on glucose for maintenance of activity after heating to 45°C. The cells of the proposita are unable to utilize glucose efficiently at glucose concentrations lower than 0.2 mmole/liter whereas normal cells maintain linear glucose consumption to at least 0.05 mM glucose. These qualitative abnormalities could result from the presence of a mutant hexokinase with an abnormally reactive sulfhydryl group and altered substrate affinity in the red cells of this kindred. PMID:4980929

  7. Molecular basis of erythroenzymopathies associated with hereditary hemolytic anemia: tabulation of mutant enzymes.

    PubMed

    Miwa, S; Fujii, H

    1996-02-01

    Molecular abnormalities of erythroenzymopathies associated with hereditary hemolytic anemia have been determined by means of molecular biology. Pyruvate kinase (PK) deficiency is the most common and well-characterized enzyme deficiency in the glycolytic pathway, and it causes hereditary hemolytic anemia. To date, 47 gene mutations have been identified. We identified one base deletion, one splicing mutation, and six distinct missense mutations in 12 unrelated families with a homozygous PK deficiency. Mutations located near the substrate or fructose-1,6- diphosphate binding site may change the conformation of the active site, resulting in a drastic loss of activity and severe clinical symptoms. Glucose-6-phosphate dehydrogenase (G6PD)deficiency is the most common metabolic disorder, and it is associated with chronic hemolytic anemia and/or drug- or infection-induced acute hemolytic attack. An estimated 400 million people are affected worldwide. The mutations responsible for about 78 variants have been determined. Some have polymorphic frequencies in different populations. Most variants are produced by one or two nucleotide substitutions. Molecular studies have disclosed that most of the class 1 G6PD variants associated with chronic hemolysis have the mutations surrounding either the substrate or the NADP binding site. Among rare enzymopathies, missense mutations have been determined in deficiencies of glucosephosphate isomerase, (TPI), phosphoglycerate kinase, and adenylate kinase. Compound heterozygosity with missense mutation and base deletion has been determined in deficiencies of hexokinase and diphosphoglyceromutase. Compound heterozygosity with missense and nonsense mutations has been identified in TPI deficiency. One base junction mutations resulting in abnormally spliced PFK-M mRNA have been identified in homozygous PFK deficiency. An exception is hemolytic anemia due to increased adenosine deaminase activity. The basic abnormality appears to result from the overproduction of a structurally normal enzyme. PMID:8579052

  8. [Mechanisms of congenital erythrocyte enzyme deficiencies associated with hemolytic anemia].

    PubMed

    Boivin, P; Kahn, A

    1976-01-01

    The search for a mechanism for red cell enzyme deficiency associated with congenital hemolytic anemia, requires one to determine the kinetic and thermodynamic properties of the enzyme reaction and study the physico-chemical and immunological characteristics of the protein which supports enzyme activity. The technique of iso-electric focalisation and the use of specific anti-enzyme antibodies, is the reason for recent progress in the understanding of the mechanism of these deficiencies. Examples of application of these techniques are given in relation to glucose-6-dehydrogenase, pyruvate kinase, glucose phosphate isomerase, phosphofructokinase and phosphoglycerate kinase of deficiencies showing the multiplicity of the molecular mechanisms. PMID:135522

  9. Hemolytic anemia in wild seaducks caused by marine oil pollution.

    PubMed

    Yamato, O; Goto, I; Maede, Y

    1996-04-01

    Clinico-pathological examinations were conducted on wild white-winged scoters (Melanitta fusca) contaminated with fuel oil (Bunker C oil) from a capsized cargo ship in February 1993 in Japan. The erythrocyte count, hemoglobin concentration and hematocrit value in the oiled seaducks all were decreased and numerous immature erythrocytes were observed in blood smears. In addition, hemosiderosis was observed in the liver, kidney, and lung of some birds. We propose that the sea-ducks suffered from hemolytic anemia induced by ingestion of oil, which occurs when the birds preen their oiled plumage. PMID:8722285

  10. Pleural solitary fibrous tumor complicated with autoimmune hemolytic anemia.

    PubMed

    Takahashi, Hiroshi; Ohkawara, Hiroshi; Ikeda, Kazuhiko; Harada-Shirado, Kayo; Furukawa, Miki; Sukegawa, Masumi; Shichishima-Nakamura, Akiko; Noji, Hideyoshi; Wakamatsu, Saho; Tasaki, Kazuhiro; Suzuki, Hiroyuki; Ogawa, Kazuei; Takeishi, Yasuchika

    2014-01-01

    We herein report a 74-year-old woman who presented with autoimmune hemolytic anemia (AIHA) associated with pleural solitary fibrous tumor (SFT). Her AIHA was initially treated with 1 mg/kg daily of oral prednisolone (PSL) for 2 months, which had a limited effect. However, after surgical tumor resection, the patient showed remarkable improvement of AIHA with normalizations of serum lactate dehydrogenase and bilirubin levels, and we were able to rapidly reduce the PSL dosage. This is the first description of a case of AIHA caused by SFT. PMID:25030571

  11. An Imported Case of Severe Falciparum Malaria with Prolonged Hemolytic Anemia Clinically Mimicking a Coinfection with Babesiosis

    PubMed Central

    Na, Young Ju; Chai, Jong-Yil; Jung, Bong-Kwang; Lee, Hyun Jung; Song, Ji Young; Je, Ji Hye; Seo, Ji Hye; Park, Sung Hun; Choi, Ji Seon

    2014-01-01

    While imported falciparum malaria has been increasingly reported in recent years in Korea, clinicians have difficulties in making a clinical diagnosis as well as in having accessibility to effective anti-malarial agents. Here we describe an unusual case of imported falciparum malaria with severe hemolytic anemia lasting over 2 weeks, clinically mimicking a coinfection with babesiosis. A 48-year old Korean man was diagnosed with severe falciparum malaria in France after traveling to the Republic of Benin, West Africa. He received a 1-day course of intravenous artesunate and a 7-day course of Malarone (atovaquone/proguanil) with supportive hemodialysis. Coming back to Korea 5 days after discharge, he was readmitted due to recurrent fever, and further treated with Malarone for 3 days. Both the peripheral blood smears and PCR test were positive for Plasmodium falciparum. However, he had prolonged severe hemolytic anemia (Hb 5.6 g/dl). Therefore, 10 days after the hospitalization, Babesia was considered to be potentially coinfected. A 7-day course of Malarone and azithromycin was empirically started. He became afebrile within 3 days of this babesiosis treatment, and hemolytic anemia profiles began to improve at the completion of the treatment. He has remained stable since his discharge. Unexpectedly, the PCR assays failed to detect DNA of Babesia spp. from blood. In addition, during the retrospective review of the case, the artesunate-induced delayed hemolytic anemia was considered as an alternative cause of the unexplained hemolytic anemia. PMID:25548419

  12. An imported case of severe falciparum malaria with prolonged hemolytic anemia clinically mimicking a coinfection with babesiosis.

    PubMed

    Na, Young Ju; Chai, Jong-Yil; Jung, Bong-Kwang; Lee, Hyun Jung; Song, Ji Young; Je, Ji Hye; Seo, Ji Hye; Park, Sung Hun; Choi, Ji Seon; Kim, Min Ja

    2014-12-01

    While imported falciparum malaria has been increasingly reported in recent years in Korea, clinicians have difficulties in making a clinical diagnosis as well as in having accessibility to effective anti-malarial agents. Here we describe an unusual case of imported falciparum malaria with severe hemolytic anemia lasting over 2 weeks, clinically mimicking a coinfection with babesiosis. A 48-year old Korean man was diagnosed with severe falciparum malaria in France after traveling to the Republic of Benin, West Africa. He received a 1-day course of intravenous artesunate and a 7-day course of Malarone (atovaquone/proguanil) with supportive hemodialysis. Coming back to Korea 5 days after discharge, he was readmitted due to recurrent fever, and further treated with Malarone for 3 days. Both the peripheral blood smears and PCR test were positive for Plasmodium falciparum. However, he had prolonged severe hemolytic anemia (Hb 5.6 g/dl). Therefore, 10 days after the hospitalization, Babesia was considered to be potentially coinfected. A 7-day course of Malarone and azithromycin was empirically started. He became afebrile within 3 days of this babesiosis treatment, and hemolytic anemia profiles began to improve at the completion of the treatment. He has remained stable since his discharge. Unexpectedly, the PCR assays failed to detect DNA of Babesia spp. from blood. In addition, during the retrospective review of the case, the artesunate-induced delayed hemolytic anemia was considered as an alternative cause of the unexplained hemolytic anemia. PMID:25548419

  13. Cryptococcal meningitis in patients with autoimmune hemolytic anemia.

    PubMed

    Yang, YaLi; Sang, Junjun; Pan, Weihua; Du, Lin; Liao, Wanqing; Chen, Jianghan; Zhu, Yuanjie

    2014-08-01

    To summarize the epidemiology, clinical features, treatment, and outcome of cryptococcal meningitis (CM) in autoimmune hemolytic anemia (AIHA) patients and to provide a reference for the prevention and control of AIHA complicated with CM, we evaluated five cases of CM in patients with AIHA treated in our hospital from 2003 to 2013 and eight related foreign cases. All of the clinical isolates were Cryptococcus neoformans var. grubii and grouped into the VNI genotype and serotype A. The clinical features exhibit significant features. Headache, nausea, and fever are common symptoms of AIHA complicated with CM. The early clinical manifestations lack specificity, which may lead to delayed diagnosis and treatment. Long-term use of prednisone (?15 mg day(-1)), poor control of anemia, and splenectomy are risk factors for AIHA complicated with cryptococcal infection. The combination of intravenous amphotericin B and oral 5-fluorocytosine remains the preferred treatment for AIHA complicated with CM. PMID:24952011

  14. Molecular aspects of erythroenzymopathies associated with hereditary hemolytic anemia.

    PubMed

    Miwa, S; Fujii, H

    1985-07-01

    Since the discovery of glucose 6-phosphate dehydrogenase (G6PD) and of pyruvate kinase deficiencies, erythroenzymopathies associated with hereditary hemolytic anemia have been extensively investigated. Kinetic and electrophoretic studies have shown that most, if not all, erythroenzymopathies are caused by the production of a mutant enzyme. Except for a few enzymes that are abundant in blood and tissues, it is difficult to obtain enough sample to study the functional and structural abnormalities of mutant enzymes associated with genetic disorders in man. The primary structures of only two normal red cell enzymes which can cause hereditary hemolytic anemia, phosphoglycerate kinase (PGK) and adenylate kinase, have been determined. Single amino acid substitutions of PGK variants have been found, and the identification of the exact molecular abnormalities of such variants has helped us to understand the accompanying functional abnormality. Gene cloning makes possible the identification of the DNA sequence that codes for enzyme proteins. Recently, human complementary DNA (cDNA) for aldolase, PGK, G6PD, and adenosine deaminase (ADA) have been isolated, and the nucleotide sequences for PGK and ADA determined. In the near future, human cDNA sequencing should permit identification of the gene alteration that gives rise to the mutant enzymes. PMID:2990202

  15. The first report of cabergoline-induced immune hemolytic anemia in an adolescent with prolactinoma.

    PubMed

    Gürbüz, Fatih; Ya?c?-Küpeli, Begül; Kör, Y?lmaz; Yüksel, Bilgin; Zorludemir, Suzan; Gürbüz, Berrak Bilginer; Küpeli, Serhan

    2014-01-01

    Prolactinomas are common pituitary tumors that can cause gonadal dysfunction and infertility related to hyperprolactinemia. Dopamine agonists are the first-line treatment in these patients. Cabergoline leads to significant reduction in serum prolactin levels and tumor size in patients with prolactinoma. Dopamine agonists have been associated with adverse effects such as nausea, vomiting and psychosis. We report here a case with cabergoline-induced immune hemolytic anemia. The patient had cabergoline treatment history for prolactinoma and presented with weakness, fatigue, nausea, and paleness. Laboratory findings revealed severe anemia-related immune hemolysis. There were no causes identified to explain hemolytic anemia except cabergoline. Therefore, cabergoline therapy was stopped and subsequently hemolytic anemia resolved and did not occur again. This is the first reported pediatric case with prolactinoma and cabergoline-induced hemolytic anemia. Clinicians should be watchful for this rare side effect induced by cabergoline. PMID:23945126

  16. Two cases of autoimmune hemolytic anemia secondary to brucellosis: a review of hemolytic disorders in patients with brucellosis.

    PubMed

    Eskazan, Ahmet Emre; Dal, Mehmet Sinan; Kaya, Safak; Dal, Tuba; Ayyildiz, Orhan; Soysal, Teoman

    2014-01-01

    Brucellosis is a worldwide zoonotic disease associated with hemolytic complications, including thrombotic microangiopathy (TMA) and hemolytic anemia. Autoimmune hemolytic anemia (AIHA) is a rare clinical presentation of this disease. In this report, we describe the cases of two patients with brucellosis who presented with Coombs-positive AIHA. We also include a review of the literature on the hemolytic complications of brucellosis. Both patients were successfully treated with a combination of doxycycline and rifampicin in addition to steroids. In the medical literature, there are several cases of TMA associated with brucellosis, although only a few cases of Coombs test-positive AIHA have been reported. Antibiotic therapy is the mainstay of treatment, and the selection of antibiotics and duration of treatment do not differ between brucellosis patients with and without hemolysis. Although rare, the potential for brucellosis should always be kept in mind in patients who present with hemolysis, especially those living in areas where brucellosis is endemic. PMID:24881740

  17. A Fetal Hemolytic Anemia in a Child with Cytomegalovirus Infection

    PubMed Central

    Hosseeini, S; Ansari, Sh; Kalantar, E; Sabzechian, M; Alibeik, A; Dorgalaleh, A

    2014-01-01

    Background Autoimmune hemolytic anemia is a hematologic disorder that is rarely observed in infants and young children. Most of the cases are associated with viral or bacterial infections. In some cases, AIHA can be characterized by a chronic course and an unsatisfactory control of hemolysis, thus requiring prolonged immunosuppressive therapy. Case report Especially in children younger than 2 years of age, the clinical course of the disease may show either resistance to steroids or dependence on high-dose steroids. We report here an infant fatal autoimmune Conclusion This case suggests that investigation for the presence of CMV infection in infantile AIHA should be considered. Severe hemolysis is rare but could be a potentially life-threatening complication of CMV infection described mostly in immune compromised adults and children. PMID:25002930

  18. A toxicogenomic approach revealed hepatic gene expression changes mechanistically linked to drug-induced hemolytic anemia.

    PubMed

    Rokushima, Masatomo; Omi, Kazuo; Araki, Akiko; Kyokawa, Yoshimasa; Furukawa, Naoko; Itoh, Fumio; Imura, Kae; Takeuchi, Kumiko; Okada, Manabu; Kato, Ikuo; Ishizaki, Jun

    2007-02-01

    A variety of pharmaceutical compounds causes hemolytic anemia as a significant adverse effect and this toxicity restricts the clinical utility of these drugs. In this study, we applied microarray technology to investigate hepatic gene expression changes associated with drug-induced hemolytic anemia and to identify potential biomarker genes for this hematotoxicity. We treated female Sprague-Dawley rats with two hemolytic anemia-inducing compounds: phenylhydrazine and phenacetin. Hepatic gene expression profiles were obtained using a whole-genome oligonucleotide microarray with pooled RNA samples from individual rats within each dose group and analyzed in comparison with hepatic histopathology, hematology, and blood chemistry data. We identified a small subset of genes that were commonly deregulated in all the severe hemolytic conditions, some of which were considered to be involved in hepatic events characteristic of hemolytic anemia, such as hemoglobin biosynthesis, heme metabolism, and phagocytosis. Among them, we selected six upregulated genes as putative biomarkers, and their expression changes from microarray measurements were confirmed by quantitative real-time PCR using RNAs from individual animals. They were Alas2, beta-glo, Eraf, Hmox1, Lgals3, and Rhced. Expression patterns of all these genes showed high negative and positive correlation against erythrocyte counts and total bilirubin levels in circulation, respectively, suggesting that these genes may be the potential biomarkers for hemolytic anemia. These findings indicate that drug-induced hemolytic anemia may be detected based on hepatic changes in the expression of a subset of genes that are mechanistically linked to the hematotoxicity. PMID:17082564

  19. Aortic valve replacement for a patient with glucose-6-phosphate dehydrogenase deficiency and autoimmune hemolytic anemia.

    PubMed

    Tas, Serpil; Donmez, Arzu Antal; Kirali, Kaan; Alp, Mete H; Yakut, Cevat

    2005-01-01

    Autoimmune hemolytic anemia and deficiency of glucose-6-phosphate deyhdrogenase (G6PD) result in severe hemolysis with different mechanisms. In patients with both pathologies, the effects of cardiopulmonary bypass on red blood cells and thrombocytes demand special care before and after open heart surgery. We evaluated the preoperative management and postoperative care of a patient with severe aortic insufficiency associated with G6PD deficiency and autoimmune hemolytic anemia who underwent aortic valve replacement. PMID:15985145

  20. Hemolytic-Anemia-Associated Pulmonary Hypertension: Sickle-Cell-Disease- and Thalassemia-Associated Pulmonary Hypertension

    Microsoft Academic Search

    Elizabeth S. Klings; Mark T. Gladwin

    \\u000a Pulmonary hypertension (PH) is now recognized as a complication of both chronic and acquired hemolytic anemias. The process\\u000a of hemolysis appears to be central to disease pathogenesis. Sickle cell disease (SCD), a congenital hemoglobinopathy affecting\\u000a as many as 30 million individuals worldwide, is the best characterized hemolytic anemia associated with PH. Multiple clinical\\u000a studies have demonstrated a 10–30% prevalence of

  1. Erythropoietin May Improve Anemia in Patients with Autoimmune Hemolytic Anemia Associated with Reticulocytopenia

    PubMed Central

    Arbach, Olga; Funck, Robert; Seibt, Frank; Salama, Abdulgabar

    2012-01-01

    Background Management of patients with autoimmune hemolytic anemia (AIHA) and reticulocytopenia remains challenging. Case Reports Two patients with decompensated AIHA who were receiving immunosuppressive drugs were treated with erythropoietin (EPO). Administration of EPO increased reticulocyte counts and hemoglobin concentrations in both cases. One patient completely recovered following a short course of treatment. Hemolysis could be compensated in the second patient using only mild doses of immunosuppressive drugs in combination with EPO. Conclusion The administration of EPO should be considered in patients with therapy-refractory AIHA, particularly in the presence of reticulocytopenia. PMID:22851939

  2. Erythropoietin May Improve Anemia in Patients with Autoimmune Hemolytic Anemia Associated with Reticulocytopenia.

    PubMed

    Arbach, Olga; Funck, Robert; Seibt, Frank; Salama, Abdulgabar

    2012-06-01

    BACKGROUND: Management of patients with autoimmune hemolytic anemia (AIHA) and reticulocytopenia remains challenging. CASE REPORTS: Two patients with decompensated AIHA who were receiving immunosuppressive drugs were treated with erythropoietin (EPO). Administration of EPO increased reticulocyte counts and hemoglobin concentrations in both cases. One patient completely recovered following a short course of treatment. Hemolysis could be compensated in the second patient using only mild doses of immunosuppressive drugs in combination with EPO. CONCLUSION: The administration of EPO should be considered in patients with therapy-refractory AIHA, particularly in the presence of reticulocytopenia. PMID:22851939

  3. Hemolytic disease of the newborn due to isoimmunization with anti-E antibodies: a case report.

    PubMed

    Sarici, S Umit; Alpay, Faruk; Ye?ilkaya, Ediz; Ozcan, Okan; Gökçay, Erdal

    2002-01-01

    Minor blood group hemolytic disease is extremely rare, since the overall potency of minor blood groups in inducing antibodies is significantly lower when compared with that of Rh (D) antigen. We hereby report a very rare case of severe neonatal anti-E hemolytic disease due to E minor blood group incompatibility. A term newborn born to a 27-year-old, gravida 3, para 3 mother was referred due to a high and increasing serum bilirubin level despite phototherapy on the 4th day of life. On admission physical examination was normal except for the jaundice, and results of the laboratory investigation demonstrated a moderate-to-severe anemia (hemoglobin 7.8 g/dl) and a severe hemolytic hyperbilirubinemia (serum total and indirect bilirubin levels 36 mg/ dl and 32.8 mg/dl, respectively; reticulocyte count 15%; and a positive direct antiglobulin test). As there was no apparent cause of the hemolytic disease such as Rh or ABO incompatibilities, further investigation (a positive indirect antiglobulin test and a positive irregular anti-E antibody in both the patient and mother, and minor blood group antigen profiles in family members compatible with E minor blood group isoimmunization) revealed the presence of anti-E hemolytic disease due to E minor blood group incompatibility. Two exchange transfusions with a 12-hour-interval were performed with minor blood group compatible fresh whole blood, and the patient was discharged in a healthy condition on the 10th postnatal day. If the most common causes of severe neonatal hemolytic disease such as Rh and ABO incompatibilities cannot be demonstrated in a newborn with significant hemolytic hyperbilirubinemia, anti-E hemolytic disease should strongly be considered in differential diagnosis. It should be kept in mind that a very severe from of minor group antibody hemolytic disease characterized by anemia and severe hyperbilirubinemia many exchange transfusions may be encountered during the course of the disease. PMID:12405439

  4. Maternal anti-M induced hemolytic disease of newborn followed by prolonged anemia in newborn twins

    PubMed Central

    Arora, Satyam; Doda, Veena; Maria, Arti; Kotwal, Urvershi; Goyal, Saurabh

    2015-01-01

    Allo-anti-M often has an immunoglobulin G (IgG) component but is rarely clinically significant. We report a case of hemolytic disease of the fetus and newborn along with prolonged anemia in newborn twins that persisted for up to 70 days postbirth. The aim was to diagnose and successfully manage hemolytic disease of newborn (HDN) due to maternal alloimmunization. Direct antiglobulin test (DAT), antigen typing, irregular antibody screening and identification were done by polyspecific antihuman globulin cards and standard tube method. At presentation, the newborn twins (T1, T2) had HDN with resultant low reticulocyte count and prolonged anemia, which continued for up to 70 days of life. Blood group of the twins and the mother was O RhD positive. DAT of the both newborns at birth was negative. Anti-M was detected in mothers as well as newborns. Type of antibody in mother was IgG and IgM type whereas in twins it was IgG type only. M antigen negative blood was transfused thrice to twin-1 and twice to twin-2. Recurring reduction of the hematocrit along with low reticulocyte count and normal other cell line indicated a pure red cell aplastic state. Anti-M is capable of causing HDN as well as prolonged anemia (red cell aplasia) due to its ability to destroy the erythroid precursor cells. Newborns with anemia should be evaluated for all the possible causes to establish a diagnosis and its efficient management. Mother should be closely monitored for future pregnancies as well. PMID:25722586

  5. Autoimmune hemolytic anemia in a patient with Malaria.

    PubMed

    Sonani, Rajesh; Bhatnagar, Nidhi; Maitrey, Gajjar

    2013-07-01

    Autoimmune Hemolytic Anemia (AIHA), a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb) was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct) were high. This patient's blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT), antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2(nd) day of starting treatment. Hb was raised to 6.1 gm% on 4(th) day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this. PMID:24014948

  6. Successful treatment with rituximab of an infant with refractory autoimmune hemolytic anemia.

    PubMed

    Moriya, Kunihiko; Matsuhashi, Tetsuro; Onuma, Masaei; Niizuma, Hidetaka; Rikiishi, Takeshi; Asada, Hiroshi; Suzuki, Jun; Sasahara, Yoji; Kure, Shigeo

    2013-08-01

    Autoimmune hemolytic anemia (AIHA) is a rare disease in infants, for which steroids are recognized as a first-line therapy for patients. Rituximab, a humanized monoclonal antibody raised against CD20, has been used in the treatment of autoimmune diseases, including AIHA, in adults and children. Due to limited follow-up study of the use of rituximab in the treatment for AIHA, its long-term efficacy, adverse effects, and immunological reconstitution of B cells have not been fully evaluated in infants. Here, we report a 3-month-old female patient with refractory AIHA, who was successfully treated with rituximab. Hemolytic anemia improved rapidly, and there were no severe adverse effects caused by rituximab. After 4.5 months following rituximab treatment, peripheral B cells were gradually reconstituted and required no intravenous immunoglobulin replacement thereafter. The patient has remained disease-free for more than 30 months without any additional treatment. This case suggests that rituximab may be a valuable therapeutic option, given its efficacy and minimal adverse effects in infants with therapy-resistant AIHA. PMID:23702915

  7. A case of recurrent autoimmune hemolytic anemia during remission associated with acute pure red cell aplasia and hemophagocytic syndrome due to human parvovirus B19 infection successfully treated by steroid pulse therapy with a review of the literature

    PubMed Central

    Sekiguchi, Yasunobu; Shimada, Asami; Imai, Hidenori; Wakabayashi, Mutsumi; Sugimoto, Keiji; Nakamura, Noriko; Sawada, Tomohiro; Komatsu, Norio; Noguchi, Masaaki

    2014-01-01

    The patient was a 47-year-old man diagnosed as having autoimmune hemolytic anemia (AIHA) in April 2011. He also had a congenital chromosomal abnormality, a balanced translocation. Treatment with prednisolone (PSL) 60 mg/day resulted in resolution of the AIHA, and the treatment was completed in November 2011. While the patient no longer had anemia, the direct and indirect Coombs tests remained positive. In May 2013, he developed recurrent AIHA associated with acute pure red cell aplasia (PRCA) and hemophagocytic syndrome (HPS) caused by human parvovirus B19 (HPV B19) infection. Tests for anti-erythropoietin and anti-erythropoietin receptor antibodies were positive. Steroid pulse therapy resulted in resolution of the AIHA, PRCA, as well as HPS. The serum test for anti-erythropoietin antibodies also became negative after the treatment. However, although the serum was positive for anti-HPV B19 IgG antibodies, the patient continued to have a low CD4 lymphocyte count (CD4, <300/?L) and persistent HPV B19 infection (HPV B19 DNA remained positive), suggesting the risk of recurrence and bone marrow failure. PMID:24966977

  8. A case of recurrent autoimmune hemolytic anemia during remission associated with acute pure red cell aplasia and hemophagocytic syndrome due to human parvovirus B19 infection successfully treated by steroid pulse therapy with a review of the literature.

    PubMed

    Sekiguchi, Yasunobu; Shimada, Asami; Imai, Hidenori; Wakabayashi, Mutsumi; Sugimoto, Keiji; Nakamura, Noriko; Sawada, Tomohiro; Komatsu, Norio; Noguchi, Masaaki

    2014-01-01

    The patient was a 47-year-old man diagnosed as having autoimmune hemolytic anemia (AIHA) in April 2011. He also had a congenital chromosomal abnormality, a balanced translocation. Treatment with prednisolone (PSL) 60 mg/day resulted in resolution of the AIHA, and the treatment was completed in November 2011. While the patient no longer had anemia, the direct and indirect Coombs tests remained positive. In May 2013, he developed recurrent AIHA associated with acute pure red cell aplasia (PRCA) and hemophagocytic syndrome (HPS) caused by human parvovirus B19 (HPV B19) infection. Tests for anti-erythropoietin and anti-erythropoietin receptor antibodies were positive. Steroid pulse therapy resulted in resolution of the AIHA, PRCA, as well as HPS. The serum test for anti-erythropoietin antibodies also became negative after the treatment. However, although the serum was positive for anti-HPV B19 IgG antibodies, the patient continued to have a low CD4 lymphocyte count (CD4, <300/?L) and persistent HPV B19 infection (HPV B19 DNA remained positive), suggesting the risk of recurrence and bone marrow failure. PMID:24966977

  9. Acute ventricular septal perforation in a patient with autoimmune hemolytic anemia

    Microsoft Academic Search

    Hitoshi Matsumoto; Toshiyuki Yuda; Takayuki Ueno; Akira Taira

    1998-01-01

    A 71-year-old woman with autoimmune hemolytic anemia underwent an emergency endocardial patch repair for ventricular septal\\u000a perforation after acute myocardial infarction. Use of washed red blood cells was effective in averting hemolytic crisis throughout\\u000a perioperative period. In spite of improvement of her hemodynamics, liver dysfunction which had been present preoperatively\\u000a deteriorated after the operation. Finally she died of hepatic failure

  10. [Autoimmune hemolytic anemia with a paroxysmal nocturnal hemoglobinuria-like defect: report of one case].

    PubMed

    Tong, Juxian; Kou, Wei; Chen, Qi; Xiao, Duan

    2013-12-01

    Both autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria are common hemolytic diseases. The former causes hemolysis because of immune disorder, and the latter is an acquired clonal hematologic disorder of stem cells. The two entities are often separate diseases, but can also occur concomitantly or secondary to each other. paroxysmal nocturnal haemoglobinuria-like defect-like defect is a special type of autoimmune haemolytic anaemia and should be distinguished from typical paroxysmal nocturnal haemoglobinuria-like defect. PMID:24369265

  11. Incompatible blood transfusion: Challenging yet lifesaving in the management of acute severe autoimmune hemolytic anemia

    PubMed Central

    Das, Sudipta Sekhar; Zaman, Rafiq Uz; Safi, Mohammad

    2014-01-01

    Background and Aim: Autoimmune hemolytic anemia (AIHA) is characterized by the production of autoantibodies directed against red cell antigens. Most patients of AIHA arrive in the emergency or out-patient department (OPD) with severe anemia requiring urgent blood transfusion. Here we share our experience of managing these patients with incompatible blood transfusions and suggest the minimal test required to assure patient safety. Materials and Methods: A total of 14 patients admitted with severe anemia, diagnosed with AIHA and requiring blood transfusion urgently were included in the study. A series of immunohematological investigations were performed to confirm the diagnosis and issue best match packed red blood cells (PRBC) to these patients. Results: A total of 167 PRBC units were crossmatched for 14 patients of which 46 units (28%) were found to be best match ones and 26 (56.5%) of these units were transfused. A mean turn around time of 222 min was observed in issuing the “best match” blood. Severe hemolysis was observed in all patients with a median hemoglobin increment of 0.88 g/dl after each unit PRBC transfusion. Conclusion: Decision to transfuse in AIHA should be based on the clinical condition of the patient. No critical patient should be denied blood transfusion due to serological incompatibility. Minimum investigations such as direct antiglobulin test (DAT), antibody screening and autocontrol should be performed to ensure transfusion safety in patients. All transfusion services should be capable of issuing “best match” PRBCs in AIHA. PMID:25161349

  12. Acute hemolytic anemia in glucose-6-phosphate dehydrogenase deficiency complicated by Ginkgo biloba.

    PubMed

    Lai, Shiue-Wei; Chen, Jia-Hong; Kao, Woei-Yau

    2013-01-01

    We report on a patient with glucose-6-phosphate dehydrogenase (G6PD) deficiency who developed acute hemolytic anemia after having received an injection of Ginkgo biloba for dementia prophylaxis without medical advice. She suddenly developed general malaise, generalized yellowish skin color, and tea-colored urine. Intravenous fluid infusion and cessation of G. biloba quickly relieved her clinical symptoms. To the best of our knowledge, this is the first case report of G. biloba-induced acute hemolytic anemia in vivo. PMID:23970095

  13. Alpha-Methyldopa-Induced Autoimmune Hemolytic Anemia in the Third Trimester of Pregnancy

    PubMed Central

    Tympa, Aliki; Liapis, Angelos; Hassiakos, Dimitrios; Bakas, Panagiotis

    2013-01-01

    Alpha-methyldopa has been demonstrated to be safe for use during pregnancy and is now used to treat gestational hypertension. In pregnancy, alpha-methyldopa-induced autoimmune hemolytic anemia does not have typical features and the severity of symptoms ranges from mild fatigue to dyspnea, respiratory failure, and death if left untreated. A case of alpha-methyldopa-induced autoimmune hemolytic anemia in a 36-year-old gravida 2, para 1 woman at 37+6 weeks of gestation is reported herein along with the differential diagnostic procedure and the potential risks to the mother and the fetus. PMID:24175105

  14. Fatal carboplatin-induced immune hemolytic anemia in a child with a brain tumor

    PubMed Central

    Haley, Kristina M; Russell, Thomas B; Boshkov, Lynn; Leger, Regina M; Garratty, George; Recht, Michael; Nazemi, Kellie J

    2014-01-01

    Drug-induced immune hemolytic anemia (DIIHA) is an uncommon side effect of pharmacologic intervention. A rare mediator of DIIHA, carboplatin is an agent used to treat many pediatric cancers. We describe here, the first case of fatal carboplatin induced DIIHA in a pediatric patient and a brief review of the literature. Our patient developed acute onset of multi-organ failure with evidence of complement activation, secondary to a drug induced red cell antibody. Early recognition of the systemic insult associated with carboplatin induced hemolytic anemia may allow for future affected patients to receive plasmapheresis, a potentially effective therapy. PMID:24868179

  15. Hereditary Hemolytic Anemia with Human Erythrocyte Pyrimidine 5?-Nucleotidase Deficiency

    PubMed Central

    Valentine, William N.; Fink, Kay; Paglia, Donald E.; Harris, Susan R.; Adams, William S.

    1974-01-01

    A severe deficiency of a red cell pyrimidine 5?-nucleotidase was found to be associated with hereditary hemolytic anemia in four members of three kindreds. The syndrome was characterized by marked increases above normal in red cell basophilic stippling, total nucleotides, and GSH and by a fairly severe deficiency of ribosephosphate pyrophosphokinase (EC 2.7.6.1.). Patient erythrocytes uniquely contained large amounts of pyrimidine 5?-ribonucleotides. In earlier studies, these were erroneously considered to be adenosine phosphates, since all previous investigations of the nucleotides of human red cells and reticulocytes have shown 97% or more to contain adenine. Total nucleotides in patient cells were present in amounts 3-6 times greater than normal, and approximately 80% contained pyrimidine. The ultraviolet spectral curves of deproteinized red cell extracts exhibited a shift in maximum absorbance from the usual 256-257 nm to approximately 266-270 nm, and absorbance at 250, 270, 280, and 290 nm, expressed as a ratio of that at 260 nm, differed greatly from normal. The spectral characteristics of extracts provide the basis of a readily performed screening procedure, which does not require enzyme assay. The nucleotidase activity in deficient red cells assayed less than 14%, and usually less than 10%, of normal and much less in terms of reticulocyte-rich blood, where it was consistently found to be increased. The enzyme has a pH optimum of 7.5-8.0, is inhibited by EDTA, and does not utilize purine 5?-ribonucleotides or ?-glycerophosphate as substrates. While comparatively few family members have been available thus far for study, initial data are compatible with an autosomal, recessive mode of transmission of the deficiency. The pyrimidine 5?-ribonucleotides are presumably derived from RNA degradation and, not being diffusible, accumulate when the enzyme catalyzing their dephosphorylation is deficient. It is postulated that the prominent basophilic stippling results from retarded ribosomal RNA degradation secondary to accumulation of degradation products, namely pyrimidine 5?-ribonucleotides. Ribosephosphate pyrophosphokinase deficiency is considered to be an epiphenomenon. The mechanism responsible for increased red cell GSH is unknown. Images PMID:4372252

  16. Attending rounds: microangiopathic hemolytic anemia with renal insufficiency.

    PubMed

    Clark, William F; Hildebrand, Ainslie

    2012-02-01

    The classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy-thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and disseminated intravascular coagulation-encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TTP, recognition of the absence of ADAMTS13 inhibition in diarrheal HUS, identification of complement abnormalities in atypical HUS, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from HUS and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy. PMID:22193233

  17. Deficiencies of glycolytic enzymes as a possible cause of hemolytic anemia.

    PubMed

    Martinov, M V; Plotnikov, A G; Vitvitsky, V M; Ataullakhanov, F I

    2000-03-01

    The critical minimum values of Na,K-ATPase and glycolytic enzyme activities at which the erythrocyte viability is lost were calculated using the mathematical model of the erythrocyte, which included all reactions of glycolysis, adenylate metabolism, ionic balance, and osmotic regulation of erythrocyte volume. The criterion for cell death was an increase in its volume to the level at which it is sequestrated from the circulation or is lysed. In hemolytic anemia associated with hexokinase or pyruvate kinase deficiency, activities of these enzymes measured in patient erythrocytes appeared to be close to the calculated critical values. By contrast, in hemolytic anemia associated with phosphofructokinase, glucosephosphate isomerase, triosephosphate isomerase, or phosphoglycerate kinase deficiency, activities of these enzymes measured in patient erythrocytes were significantly greater than the calculated critical values. In this case, if the deficient enzyme were stable, i.e. its activity in the cell were low, but constant in time, the deficiency observed would not account for the erythrocyte destruction observed and the development of hemolytic anemia. It was shown, however, that in phosphofructokinase, glucosephosphate isomerase, triosephosphate isomerase, or phosphoglycerate kinase deficiency, hemolytic anemia can arise because of the instability of these enzymes in time. PMID:10699493

  18. Brown recluse spider (Loxosceles reclusa) envenomation leading to acute hemolytic anemia in six adolescents.

    PubMed

    McDade, Jenny; Aygun, Banu; Ware, Russell E

    2010-01-01

    Loxosceles reclusa (brown recluse spider) bites often cause local envenomation reactions; however, acute hemolysis from systemic loxoscelism is rare. To highlight this important diagnostic consideration for unexplained hemolysis in areas endemic for brown recluse spiders, we report on 6 adolescents with acute hemolytic anemia from presumed L reclusa bites. PMID:20006769

  19. Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia

    E-print Network

    Speakers Mark; T. Gladwin; Md Richard Lottenberg; Mark C. Walters; Mark T. Gladwin; Gregory J. Kato

    Medical advances in the management of patients with sickle cell disease, thalassemia, and other hemolytic anemias have led to significant increases in life expectancy. Improved public health, neonatal screen-ing, parental and patient education, advances in red cell transfusion medicine, iron

  20. A case of primary ovarian lymphoma with autoimmune hemolytic anemia achieving complete response with Rituximab-based combination chemotherapy

    PubMed Central

    Ghadyalpatil, N. S.; Chandrasekar, R.; Snehalatha, D.; Reddy, B. M.

    2011-01-01

    Ovarian involvement as primary or secondary lymphomatous process is extremely uncommon. In most cases, the diagnosis is usually not suspected initially and is confirmed only after detailed histopathological evaluation. We report a patient with primary ovarian diffuse large B-cell lymphoma (DLBCL) and associated auto-immune hemolytic anemia (AIHA) who achieved complete remission after treatment with Rituximab-cyclophosphamide-doxorubicin-vincristine and prednisolone (R-CHOP) chemotherapy. This patient was a 50 year old female, who presented with fever, abdominal pain, vomiting, weight loss and anemia. Computed tomography scan of the abdomen and pelvis revealed a large left ovarian mass with bilateral hydronephrosis. We performed exploratory laparotomy and partial resection of the mass was done due to the adhesions. Histopathology confirmed the diagnosis of DLBCL. After six R-CHOP chemotherapy cycles, patient achieved complete response with correction of anemia. To our knowledge, this may be the first case report till date of primary ovarian DLBCL with AIHA treated with R-CHOP chemotherapy who achieved complete remission in terms of primary disease as well as hemolytic anemia. PMID:22563154

  1. Effect of hemolytic and iron-deficiency anemia on intestinal absorption and tissue accumulation of cadmium.

    PubMed

    Min, Kyong-Son; Iwata, Naoyuki; Tetsutikawahara, Noriko; Onosaka, Satomi; Tanaka, Keiichi

    2008-06-10

    Abnormal iron (Fe) metabolism induces iron-deficiency anemia (FeDA) and also affects body cadmium (Cd) accumulation. However, whether hemolytic anemia also affects Cd metabolism is not known. We compared the intestinal absorption and tissue accumulation of Cd after oral administration of Cd to mice with hemolytic anemia induced by treatment with phenylhydrazine (PHA mice) to that in mice with FeDA. Although the hematocrit decreased significantly in mice with either type of anemia, the Fe concentration decreased in the livers and kidneys of FeDA mice, but increased in those of PHA mice. After an oral administration with various amounts of Cd, hepatic and renal Cd concentrations significantly increased in both FeDA and PHA mice. An intraduodenal injection of Fe raised the hepatic Fe content in FeDA mice to the control level and raised the hepatic Fe content in PHA mice to 2.4 times that in control mice. Intestinal divalent metal transporter 1 (DMT1) expression increased significantly in mice with both types of anemia. These data indicate that, despite the accumulation of hepatic Fe associated with PHA, PHA also significantly increases hepatic and renal Cd accumulation according to an stimulation of intestinal DMT1 expression, as occurs in FeDA mice. This suggests that anemia may be a risk factor for Cd accumulation. PMID:18485624

  2. Pure red-cell aplasia and autoimmune hemolytic anemia in a patient with acute hepatitis A

    PubMed Central

    Chang, Hyo Jeong; Cho, Sung Gyun; Oh, Tae Hoon; Jeon, Tae Joo; Shin, Won Chang; Choi, Won Choong

    2014-01-01

    Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA. PMID:25032187

  3. Pure red-cell aplasia and autoimmune hemolytic anemia in a patient with acute hepatitis A.

    PubMed

    Chang, Hyo Jeong; Sinn, Dong Hyun; Cho, Sung Gyun; Oh, Tae Hoon; Jeon, Tae Joo; Shin, Won Chang; Choi, Won Choong

    2014-06-01

    Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA. PMID:25032187

  4. Coombs-Negative Autoimmune Hemolytic Anemia in Crohn’s Disease

    PubMed Central

    Park, Bong Soo; Park, Sihyung; Jin, Kyubok; Kim, Yeon Mee; Park, Kang Min; Lee, Jeong-Nyeo; Kamesaki, Toyomi; Kim, Yang Wook

    2014-01-01

    Patient: Female, 41 Final Diagnosis: Coombs negative autoimmune hemolytic anemia Symptoms: Dark urine • dizziness • dyspnea Medication: — Clinical Procedure: Immunoradiometric assay for RBC-IgG Specialty: Hematology Objective: Rare disease Background: Anemia is a common, important extraintestinal complication of Crohn’s disease. The main types of anemia in patients with Crohn’s disease are iron deficiency anemia and anemia of chronic disease. Although patients with Crohn’s disease may experience various type of anemia, autoimmune hemolytic anemia (AIHA) in patients with Crohn’s disease, especially Coombs-negative AIHA, is very rare. Case Report: A 41-year-old woman with Crohn’s disease presented to our emergency room (ER) with dark urine, dizziness, and shortness of breath. The activity of Crohn’s disease had been controlled, with Crohn’s disease activity index (CDAI) score below 100 point. On physical examination, the patient had pale conjunctivae and mildly icteric sclerae. Serum bilirubin was raised at 3.1 mg/dL, lactate dehydrogenase (LDH) level was 1418 U/L and the haptoglobin level was <3 mg/dL. Results of direct and the indirect Coombs tests were all negative. We then measured the RBC-IgG to evaluate the possibility of Coombs-negative AIHA. The result revealed that RBC-IgG level was 352 IgG molecules/cell, with the cut-off value at 78.5 IgG molecules/cell. Conclusions: We report a case of Coombs-negative AIHA in a patient with Crohn’s disease with chronic anemia, diagnosed by red blood cell-bound immunoglobulin G (RBC-IgG) and treated with steroids therapy. PMID:25488633

  5. [A case of acute autoimmune hepatitis associated with autoimmune hemolytic anemia].

    PubMed

    Hanai, Tatsunori; Naiki, Takafumi; Takamatsu, Manabu; Imai, Kenji; Kitagawa, Junichi; Suetsugu, Atsushi; Takai, Koji; Shiraki, Makoto; Shimizu, Masahito; Hirose, Yoshinobu; Tsurumi, Hisashi; Moriwaki, Hisataka

    2013-10-01

    A 61-year-old female was admitted to our hospital with severe jaundice and anemia. She was diagnosed with severe acute hepatitis secondary to autoimmune hepatitis (AIH) on the basis of positive anti-nuclear antibody titers, high serum IgG levels, and liver biopsy. Autoimmune hemolytic anemia (AIHA) was diagnosed because of the presence of reticulocytosis, decreased haptoglobin, positive direct Coombs test, and erythroid hyperplasia in the bone marrow. Although AIH occurs in association with various immunological disorders, an association with AIHA is rarely reported. We report a rare case of severe AIH associated with AIHA. PMID:24097153

  6. A Thermolabile Aldolase A Mutant Causes Fever-Induced Recurrent Rhabdomyolysis without Hemolytic Anemia

    PubMed Central

    Mamoune, Asmaa; Bahuau, Michel; Hamel, Yamina; Serre, Valérie; Pelosi, Michele; Habarou, Florence; Nguyen Morel, Marie-Ange; Boisson, Bertrand; Vergnaud, Sabrina; Viou, Mai Thao; Nonnenmacher, Luc; Piraud, Monique; Nusbaum, Patrick; Vamecq, Joseph; Romero, Norma; Ottolenghi, Chris; Casanova, Jean-Laurent; de Lonlay, Pascale

    2014-01-01

    Aldolase A deficiency has been reported as a rare cause of hemolytic anemia occasionally associated with myopathy. We identified a deleterious homozygous mutation in the ALDOA gene in 3 siblings with episodic rhabdomyolysis without hemolytic anemia. Myoglobinuria was always triggered by febrile illnesses. We show that the underlying mechanism involves an exacerbation of aldolase A deficiency at high temperatures that affected myoblasts but not erythrocytes. The aldolase A deficiency was rescued by arginine supplementation in vitro but not by glycerol, betaine or benzylhydantoin, three other known chaperones, suggesting that arginine-mediated rescue operated by a mechanism other than protein chaperoning. Lipid droplets accumulated in patient myoblasts relative to control and this was increased by cytokines, and reduced by dexamethasone. Our results expand the clinical spectrum of aldolase A deficiency to isolated temperature-dependent rhabdomyolysis, and suggest that thermolability may be tissue specific. We also propose a treatment for this severe disease. PMID:25392908

  7. Anemia Due to Excessive Bleeding

    MedlinePLUS

    ... Blood Cell Disorders Plasma Cell Disorders Leukemias Lymphomas Myeloproliferative Disorders Spleen Disorders Topics in Anemia Overview of ... stomach or small intestine and diverticulosis, polyps, or cancers in the large intestine. Other sources of chronic ...

  8. Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features

    Microsoft Academic Search

    Francesca R. Mauro; Robert Foa; Raffaella Cerretti; Diana Giannarelli; Serelina Coluzzi; Franco Mandelli; Gabriella Girelli

    2000-01-01

    Fifty-two cases of autoimmune hemolytic anemia (AHA) were observed within a series of 1203 patients (4.3%) with chronic lymphocytic leukemia (CLL) followed at a single institution. Nineteen were ob- served at the time of CLL diagnosis and 33 during the clinical follow-up. Ninety percent of the patients with CLL\\/AHA showed active CLL and 25% had been treated previously. The antierythrocyte

  9. Interferon-? induced psoriatic arthritis and autoimmune hemolytic anemia during chronic hepatitis C treatment.

    PubMed

    Oliveira, T L; Caetano, A Z; Belem, J M; Klemz, B C; Pinheiro, M M

    2014-01-01

    Chronic hepatitis C (CHC) can occur simultaneously with a myriad of rheumatic diseases or can induce autoimmunity. Inflammatory arthropathy is the main extra-hepatic manifestation of infection by virus C. In addition, the treatment for CHC with INF-? and ribavirin is also able to cause some immune-mediated events. The present case report describes an unusual case of psoriatic arthritis (PsA) and autoimmune hemolytic anemia (AIHA) during therapy for CHC. PMID:25341062

  10. Successful rituximab treatment of refractory hemophagocytic lymphohistiocytosis and autoimmune hemolytic anemia associated with systemic lupus erythematosus.

    PubMed

    So, Min Wook; Koo, Bon San; Kim, You Jae; Kim, Yong-Gil; Lee, Chang-Keun; Yoo, Bin

    2014-09-01

    High-dose steroids, immunosuppressants such as cyclophosphamide and cyclosporine, and high-dose intravenous immunoglobulin have all been used to control hemophagocytic lymphohistiocytosis (HLH) or autoimmune hemolytic anemia (AIHA) associated with systemic lupus erythematosus (SLE); however, some patients are refractory to treatment. Rituximab has successfully resolved many of the refractory manifestations of SLE. Here, we report a case of HLH and AIHA associated with SLE that was refractory or intolerable to conventional therapy, but was successfully treated with rituximab. PMID:24517558

  11. Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties

    Microsoft Academic Search

    Ami A Shah; John P Higgins; Eliza F Chakravarty

    2007-01-01

    Background A 19-year-old woman with newly diagnosed systemic lupus erythematosus (SLE) presented with hemolytic anemia, thrombocytopenia, hypertension, tonic-clonic seizures, blurry vision, nephrotic syndrome and renal insufficiency.Investigations At a general hospital, the investigations included brain MRI, echocardiography, laboratory tests including measurement of the amount of protein excreted daily, platelet count, levels of lactate dehydrogenase, creatinine and anticardiolipin antibodies, direct Coombs' test,

  12. Occurrence of hemolytic anemia in patients with GBS treated with high-dose IVIg

    PubMed Central

    Biliciler, Suur; Wahed, Amer; Sheikh, Kazim

    2014-01-01

    Objective: We describe an underrecognized side effect of high-dose IV immunoglobulin (IVIg), hemolytic anemia. Background: There are no established guidelines on treating patients with Guillain-Barré syndrome (GBS) who relapse or do not improve after a standard course of treatment (IVIg or plasma exchange). Some centers will opt for a second course of the initial treatment. There is an ongoing trial of a second course of IVIg in patients with severe GBS. Methods: We retrospectively reviewed 4 patients with severe GBS who received high-dose IVIg. One patient inadvertently received a high dose of IVIg for Miller Fisher syndrome. All patients received a total of at least 2 courses of the standard dose of IVIg (total >4 g/kg). We review their clinical course and side effects. Results: All patients with non-O blood types developed clinically significant hemolytic anemia requiring blood transfusion. Conclusion: Hemolytic anemia may limit doses of IVIg for treatment of severe GBS in patients with non-O blood types. PMID:25520957

  13. Clinicopathologic features of young and old sphha/sphha mice. Mutants with congenital hemolytic anemia.

    PubMed Central

    Maggio-Price, L.; Russell, R.; Wolf, N. S.; Alpers, C. E.; Engel, D.

    1988-01-01

    A colony of mice with congenital hemolytic anemia, sphha/sphha, were evaluated over a 3-year period. Prominent findings included decreased survivability, reticulocytosis, increased peripheral blood leukocytes, extramedullary hematopoiesis in liver and spleen, lymphoid hyperplasia and membranoproliferative glomerulonephritis. Older (12 to 21 months) anemic animals had elevated serum levels of IgG1 and IgA. There was deposition of C3, IgG, IgM, and IgA in renal glomeruli of both control and anemic mice, but deposition of IgM and IgA was more prominent and widely distributed in anemic animals and correlated with mesangial expansion and the presence of electron dense deposits in the mesangium and in glomerular capillary walls. Prominent renal tubular hemosiderosis was noted in young and old anemic mice. The relation between the hemolytic anemia and glomerular disease is unclear but these mice may be an animal model useful for exploration of changes attendant with chronic hemolysis and evaluation of renal disease that accompanies hemolytic anemia. Images Figure 1 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 PMID:3414779

  14. Severe refractory autoimmune hemolytic anemia with both warm and cold autoantibodies that responded completely to a single cycle of rituximab: a case report

    Microsoft Academic Search

    Shilpi Gupta; Anita Szerszen; Fadi Nakhl; Seema Varma; Aaron Gottesman; Frank Forte; Meekoo Dhar

    2011-01-01

    Introduction  Mixed warm and cold autoimmune hemolytic anemia runs a chronic course with severe intermittent exacerbations. Therapeutic\\u000a options for the treatment of hemolysis associated with autoimmune hemolytic anemia are limited. There have been only two reported\\u000a cases of the effective use of rituximab in the treatment of patients with mixed autoimmune hemolytic anemia. We report a case\\u000a of severe mixed autoimmune

  15. Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy

    PubMed Central

    Butler, Matthew J.; Yin, Ming; Quddus, Fahd

    2014-01-01

    Hemolysis is an uncommon and usually late complication of malignancy, and very rarely the presenting feature. Cancer-associated hemolysis may be immune-mediated, or may result from thrombotic microangiopathy accompanied by thrombocytopenia. We describe an unusual case of isolated hemolysis in the setting of occult metastatic breast cancer. The patient initially presented with symptomatic anemia, with evidence of hemolysis but with negative direct antiglobulin testing and a normal platelet count. Subsequent investigation discovered metastatic adenocarcinoma of the breast involving bone marrow. Hemolysis worsened despite initial treatment with cytotoxic chemotherapy and a trial of corticosteroids, but later resolved with aromatase inhibitor therapy. PMID:24711918

  16. The Effect of Erythropoiesis-Stimulating Agents in Patients with Therapy-Refractory Autoimmune Hemolytic Anemia

    PubMed Central

    Salama, Abdulgabar; Hartnack, Dirk; Lindemann, Hans-Walter; Lange, Hans-Joachim; Rummel, Mathias; Loew, Andreas

    2014-01-01

    Summary Background Many patients with autoimmune hemolytic anemia (AIHA) do not respond to standard therapy and/or may develop severe complications which can be of fatal outcome. There is some evidence that erythropoiesis-stimulating agents (ESAs) may be helpful in the management of such patients. Methods We describe the effect of ESAs in 12 new patients with therapy-refractory AIHA (7 of warm type and 5 of cold type) and review 5 previously reported cases. Serological testing was performed using standard methods. Results All patients responded well to treatment with ESAs. At least 5 of the 17 patients demonstrated complete recovery, and none of the patients developed significant adverse reactions due to treatment with ESAs. Conclusion The mechanism by which ESAs improves hemolysis in AIHA is not completely clear. In addition to increased production and prolonged RBC survival, it may inhibit eryptosis (programmed cell death). ESAs represent a new option in the treatment of decompensated and/or refractory AIHA of warm and cold type. However, more information is required to assess which patients can be treated with ESAs. PMID:25670934

  17. Enzymatic diagnosis in non-spherocytic hemolytic anemia.

    PubMed

    Hirono, A; Forman, L; Beutler, E

    1988-03-01

    Blood samples from 722 unrelated patients with anemia and/or reticulocytosis were submitted to our laboratory for red cell enzyme assay during the past 7 years. Among these 722 cases, we found 82 cases of 7 different red cell enzyme deficiencies and 2 of unstable hemoglobin. Abnormalities of pyruvate kinase (PK) were found to cause hemolysis in 55 patients. Although their average PK activity was about 35% of the normal level, 5 showed normal and 2 demonstrated high PK activity. Among 17 patients in whom pyruvate kinase assays or screening tests had been carried out in routine laboratories, the correct diagnoses had been made in only 4. Glucose-6-phosphate dehydrogenase (G6PD) deficiency was found in 15 patients, pyrimidine 5'-nucleotidase deficiency in 5, glucose phosphate isomerase deficiency in 3, adenylate kinase deficiency in 2, phosphoglycerate kinase deficiency in 1, and glutathione synthetase deficiency in 1 patient. Even after we performed a panel of over 20 different red cell enzyme assays, 519 patients still remained undiagnosed. PMID:3352512

  18. Rare hereditary red blood cell enzymopathies associated with hemolytic anemia - pathophysiology, clinical aspects, and laboratory diagnosis.

    PubMed

    Koralkova, P; van Solinge, W W; van Wijk, R

    2014-06-01

    Hereditary red blood cell enzymopathies are genetic disorders affecting genes encoding red blood cell enzymes. They cause a specific type of anemia designated hereditary nonspherocytic hemolytic anemia (HNSHA). Enzymopathies affect cellular metabolism, which, in the red cell, mainly consists of anaerobic glycolysis, the hexose monophosphate shunt, glutathione metabolism, and nucleotide metabolism. Enzymopathies are commonly associated with normocytic normochromic hemolytic anemia. In contrast to other hereditary red cell disorders such as membrane disorders or hemoglobinopathies, the morphology of the red blood cell shows no specific abnormalities. Diagnosis is based on detection of reduced specific enzyme activity and molecular characterization of the defect on the DNA level. The most common enzyme disorders are deficiencies of glucose-6-phosphate dehydrogenase (G6PD) and pyruvate kinase (PK). However, there are a number of other enzyme disorders, often much less known, causing HNSHA. These disorders are rare and often underdiagnosed, and the purpose of this review. In this brief review, we provide an overview of clinically relevant enzymes, their function in red cell metabolism, and key aspects of laboratory diagnosis. PMID:24750686

  19. Cholesterol granulomas of the lungs associated with microangiopathic hemolytic anemia and thrombocytopenia in pulmonary hypertension.

    PubMed

    Fischer, E G; Marek, J M; Morris, A; Nashelsky, M B

    2000-12-01

    Cholesterol granulomas unrelated to endogenous lipoid pneumonia, pulmonary alveolar proteinosis, or cholesterol pneumonia are a rare finding during pneumectomy or autopsy. They have been occasionally reported in association with pulmonary hypertension. We report a case where these lesions were associated with long-standing pulmonary hypertension and microangiopathic hemolytic anemia and thrombocytopenia. Plexiform lesions were present in the pulmonary vasculature secondary to pulmonary hypertension, causing hemolysis and thrombocytopenia. We suggest that destruction of red blood cells and platelets could provide membrane lipids that are taken up by phagocytic cells, which promotes the formation of these cholesterol deposits. PMID:11100063

  20. Pseudo-hypoproteinemia in a hyperbilirubinemic dog with immune-mediated hemolytic anemia.

    PubMed

    Garner, Bridget C; Priest, Heather; Smith, Jo

    2014-06-01

    A 10-year-old spayed female Miniature Poodle was presented to the University of Georgia veterinary teaching hospital for evaluation of lethargy, vomiting and anorexia of 4 days' duration. Physical examination, history and a minimum database led to a diagnosis of immune-mediated hemolytic anemia accompanied by marked hyperbilirubinemia. Refractometric protein determination was within the reference interval, whereas the biuret method indicated hypoproteinemia. This discrepancy was attributed to interference of bilirubin and biliverdin with the spectrophotometric read-out of the biuret total protein assay. The albumin concentration, determined by bromcresol green, and refractometric total protein were less affected by this interference. PMID:24627963

  1. [Successful treatment with rituximab in a patient with refractory mixed-type autoimmune hemolytic anemia].

    PubMed

    Ono, Kaoru; Sato, Tsutomu; Iyama, Satoshi; Tatekoshi, Ayumi; Hashimoto, Akari; Kamihara, Yusuke; Horiguchi, Hiroto; Kikuchi, Shohei; Takada, Kohichi; Hayashi, Tsuyoshi; Miyanishi, Koji; Sato, Yasushi; Takimoto, Rishu; Kobune, Masayoshi; Kato, Junji

    2013-11-01

    The evidence that rituximab is effective therapy for refractory warm or cold autoimmune hemolytic anemia (AIHA) has been accumulating; however, the efficacy of rituximab for mixed-type AIHA is not evident. Herein, we report a case of mixed-type AIHA refractory to corticosteroids and splenectomy, but successfully treated with rituximab (375 mg/m(2)/day, once weekly, four times). She achieved a complete response, which has been maintained for 16 months, to date, despite steroid tapering. Our case suggests that rituximab therapy should be considered for refractory AIHA even of mixed-type. PMID:24305538

  2. First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia

    PubMed Central

    Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo

    2014-01-01

    To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed. PMID:25024635

  3. Splenic infarction in a patient with autoimmune hemolytic anemia and protein C deficiency

    PubMed Central

    Park, Min Yong; Kim, Jung A; Yi, Seong Yoon; Chang, Sun Hee; Um, Tae Hyun

    2011-01-01

    Splenic infarction is most commonly caused by cardiovascular thromboembolism; however, splenic infarction can also occur in hematologic diseases, including sickle cell disease, hereditary spherocytosis, chronic myeloproliferative disease, leukemia, and lymphoma. Although 10% of splenic infarction is caused by hematologic diseases, it seldom accompanies autoimmune hemolytic anemia (AIHA). We report a case of a 47-year-old woman with iron deficiency anemia who presented with pain in the left upper abdominal quadrant, and was diagnosed with AIHA and splenic infarction. Protein C activity and antigen decreased to 44.0% (60-140%) and 42.0% (65-140%), respectively. Laboratory testing confirmed no clinical cause for protein C deficiency, such as disseminated intravascular coagulation, sepsis, hepatic dysfunction, or acute respiratory distress syndrome. Protein C deficiency with splenic infarction has been reported in patients with viral infection, hereditary spherocytosis, and leukemia. This is a rare case of splenic infarction and transient protein C deficiency in a patient with AIHA. PMID:22259634

  4. First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia.

    PubMed

    Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo

    2014-07-14

    To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed. PMID:25024635

  5. Vitamin B12 and Vitamin D Deficiencies: An Unusual Cause of Fever, Severe Hemolytic Anemia and Thrombocytopenia

    PubMed Central

    Mishra, Vikas A.; Harbada, Rishit; Sharma, Akhilesh

    2015-01-01

    The array of diagnostic workup for pyrexia of unknown origin (PUO) generally revolves in searching for infections, inflammatory/autoimmune, and endocrine etiologies. A differential diagnosis of fever, hemolytic anemia, and thrombocytopenia can have etiologies varying from infections like malaria, dengue, cytomegalovirus, Ebstein barr virus, Parvovirus, infective endocarditis, to autoimmune disorder (systemic lupus erythromatosis), vasculitis, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (TTP), autoimmune hemolytic anemia/Evan's syndrome, paroxysmal nocturnal hemoglobinuri (PNH), or drugs. Nutritional deficiencies (especially vitamin B12 deficiency) as a cause of fever, hemolytic anemia, and thrombocytopenia are very rare and therefore rarely thought of. Severe vitamin B12 deficiency may cause fever and if accompanied by concurrent hyper-homocysteinemia and hypophosphatemia can sometimes lead to severe hemolysis mimicking the above-mentioned conditions. We present a case that highlights vitamin B12 and vitamin D deficiency as an easily treatable cause of PUO, hemolytic anemia, and thrombocytopenia, which should be actively looked for and treated before proceeding with more complicated and expensive investigation or starting empiric treatments.

  6. Anemia hemolítica em cães e gatos

    Microsoft Academic Search

    Rafael Almeida Fighera

    Hemolytic anemia is the reduction in the numbers of an individual's red blood cells (RBCs) due to shortening of the life span of these cells. There are several hemolytic disorders caused by infectious agents in dogs and cats, namely babesiosis, rangeliosis, trypanoso- miasis, cytauxzoonosis, hemobartonellosis, dirofilariasis and hemolytic anemia associated with the infection by the feline leukemia virus. Non- infectious

  7. Warm autoimmune hemolytic anemia secondary to Plasmodium ovale infection: a case report and review of the literature.

    PubMed

    Johnson, Adam S; Delisca, Gadini; Booth, Garrett S

    2013-12-01

    A three year old male from the Democratic Republic of the Congo was admitted to Monroe Carell Jr. Children's Hospital at Vanderbilt with a 10-day history of fever, emesis, and diarrhea. Examination demonstrated scleral icterus, splenomegaly, and anemia. By peripheral blood smear, the patient was diagnosed with Plasmodium ovale. Immunohematology demonstrated a positive direct antiglobulin test (DAT) for IgG and C3d with pan-agglutination on eluate. These findings, in combination with hemolytic labs, signified presence of an autoimmune hemolytic anemia (AIHA). We believe this to be the first reported case of P. ovale infection-mediated AIHA. PMID:24148713

  8. Clinical outcomes of splenectomy in children: Report of the splenectomy in congenital hemolytic anemia registry.

    PubMed

    Rice, Henry E; Englum, Brian R; Rothman, Jennifer; Leonard, Sarah; Reiter, Audra; Thornburg, Courtney; Brindle, Mary; Wright, Nicola; Heeney, Matthew M; Smithers, Charles; Brown, Rebeccah L; Kalfa, Theodosia; Langer, Jacob C; Cada, Michaela; Oldham, Keith T; Scott, J Paul; St Peter, Shawn; Sharma, Mukta; Davidoff, Andrew M; Nottage, Kerri; Bernabe, Kathryn; Wilson, David B; Dutta, Sanjeev; Glader, Bertil; Crary, Shelley E; Dassinger, Melvin S; Dunbar, Levette; Islam, Saleem; Kumar, Manjusha; Rescorla, Fred; Bruch, Steve; Campbell, Andrew; Austin, Mary; Sidonio, Robert; Blakely, Martin L

    2015-03-01

    The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (?30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1?±?1.8 g/dl, 52 week 12.8?±?1.6 g/dl; mean?±?SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process. Am. J. Hematol. 90:187-192, 2015. © 2014 Wiley Periodicals, Inc. PMID:25382665

  9. Giant cell hepatitis with autoimmune hemolytic anemia in a nine month old infant.

    PubMed

    Bouguila, Jihene; Mabrouk, Sameh; Tilouche, Samia; Bakir, Dajla; Trabelsi, Amel; Hmila, Amel; Boughammoura, Lamia

    2013-04-27

    Giant cell hepatitis (GCH) with autoimmune hemolytic anemia is a rare entity, limited to young children, with an unknown pathogenesis. We report the case of 9-mo old who presented with fever, diarrhea and jaundice four days before hospitalization. Physical examination found pallor, jaundice and hepatosplenomegaly. The laboratory workup showed serum total bilirubin at 101 ?mol/L, conjugated bilirubin at 84 ?mol/L, hemolytic anemia, thrombocytopenia and immunoglobulin G (IgG) and anti-C3d positive direct Coombs' test. The antinuclear, anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies, antiendomisium antibodies were negative. Serological assays for viral hepatitis B and C, cytomegalovirus, herpes simplex and Epstein Barr virus were negative. The association of acute liver failure, Evan's syndrome, positive direct Coomb's test of mixed type (IgG and C3) and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH. The diagnosis was confirmed by a needle liver biopsy. The patient was treated by corticosteroids, immunomodulatory therapy and azathioprine but died with septicemia. PMID:23671728

  10. Heinz-body hemolytic anemia from the ingestion of crude oil: a primary toxic effect in marine birds

    SciTech Connect

    Leighton, F.A. (Cornell Univ., Ithaca, NY); Peakall, D.B.; Butler, R.G.

    1983-05-20

    Hemolytic anemia developed in young herring gulls and Atlantic puffins given daily oral doses of a Prudhoe Bay crude oil. Anemia developed 4 to 5 days after the initiation of oil ingestion and was accompainied by Heinz-body formation and a strong regenerative response. The data evince a toxic effect on circulating red blood cells involving an oxidative biochemical mechanism and the first clear evidence of a primary mechanism of toxicity from the ingestion of crude oil by birds.

  11. [Study on blood ABO typing in patients with autoimmune hemolytic anemia].

    PubMed

    Zhu, Jian-Ying; Lan, Jiong-Cai; Hu, Li-Ya; Meng, Qing-Bao; Luo, Hong-Qing

    2004-08-01

    To explore effect of autoantibody on identification of ABO and RhD blood group, the blood samples of 38 patients with autoimmune hemolytic anemia (AIHA) were identified by routine typing and typing after chloroquine elution test as well as PCR. The results showed that out of 38 patients with AIHA, 11 cases (31.6%) of ABO blood group were difficulty typed, indirect antiglobulin test were positive, and contradiction between cells typing and sera typing were observed. 1 case of RhD(-) was mistyped as RhD(+) and anti-D was found in its serum. The blood group of these cases were typed correctly by chloroquine elution test. It is concluded that blood group identification of patients with AIHA can be interfered by autoantibody, and the correct typing for blood group of these patients may be determined by using combination of several methods to ensure safe transfusion. PMID:15363147

  12. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia

    PubMed Central

    Arroyo-Ávila, Mariangelí; Fred-Jiménez, Ruth M.; Vilá, Luis M.

    2015-01-01

    Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L) after the second weekly rituximab infusion (375?mg/m2 weekly × 4) given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections.

  13. Anti B cell targeted therapy for autoimmune hemolytic anemia in an infant.

    PubMed

    Makadia, Darshak; Siddaiahgari, Sirisha Rani; Latha, M S

    2013-01-01

    Autoimmune hemolytic anemia (AIHA) is an immune mediated destruction of erythrocytes, which has a good prognosis in children. It is known to have chronic, remitting or relapsing course, especially in infants and adolescents. Treatment of refractory or relapsing AIHA is a challenge as the other aim of the treatment is to avoid prolonged exposure to steroids or other immunosuppressants in small children. Rituximab is used in patients who are non-responsive to conventional treatment such as steroids, intravenous immunoglobulins and transfusion therapy. It has varying therapeutic success rate. We report a case of AIHA in a 4-month-old infant who had ill-sustained response to conventional therapy, but responded to rituximab. PMID:24130393

  14. Erythrocytic Pyruvate Kinase Mutations Causing Hemolytic Anemia, Osteosclerosis, and Secondary Hemochromatosis in Dogs

    PubMed Central

    Gultekin, G. Inal; Raj, K.; Foureman, P.; Lehman, S.; Manhart, K.; Abdulmalik, O.; Giger, U.

    2013-01-01

    Background Erythrocytic pyruvate kinase (PK) deficiency, first documented in Basenjis, is the most common inherited erythroenzymopathy in dogs. Objectives To report 3 new breed-specific PK-LR gene mutations and a retrospective survey of PK mutations in a small and selected group of Beagles and West Highland White Terriers (WHWT). Animals Labrador Retrievers (2 siblings, 5 unrelated), Pugs (2 siblings, 1 unrelated), Beagles (39 anemic, 29 other), WHWTs (22 anemic, 226 nonanemic), Cairn Terrier (n = 1). Methods Exons of the PK-LR gene were sequenced from genomic DNA of young dogs (<2 years) with persistent highly regenerative hemolytic anemia. Results A nonsense mutation (c.799C>T) resulting in a premature stop codon was identified in anemic Labrador Retriever siblings that had osteosclerosis, high serum ferritin concentrations, and severe hepatic secondary hemochromatosis. Anemic Pug and Beagle revealed 2 different missense mutations (c.848T>C, c.994G>A, respectively) resulting in intolerable amino acid changes to protein structure and enzyme function. Breed-specific mutation tests were developed. Among the biased group of 248 WHWTs, 9% and 35% were homozygous (affected) and heterozygous, respectively, for the previously described mutation (mutant allele frequency 0.26). A PK-deficient Cairn Terrier had the same insertion mutation as the affected WHWTs. Of the selected group of 68 Beagles, 35% were PK-deficient and 3% were carriers (0.37). Conclusions and Clinical Importance Erythrocytic PK deficiency is caused by different mutations in different dog breeds and causes chronic severe hemolytic anemia, hemosiderosis, and secondary hemochromatosis because of chronic hemolysis and, an as yet unexplained osteosclerosis. The newly developed breed-specific mutation assays simplify the diagnosis of PK deficiency. PMID:22805166

  15. CNS intravascular large cell lymphoma in a patient with autoimmune hemolytic anemia.

    PubMed

    Alexandrescu, Sanda; Orengo, James P; Toossi, Shahed; Perry, Arie; Treseler, Patrick; Hess, Christopher; Margeta, Marta

    2014-11-01

    Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality. PMID:25378202

  16. Peritoneal EMH in a dog with immune-mediated hemolytic anemia.

    PubMed

    Brenner, Karen; Pohlman, Lisa; Muldowney, Ian; Petersen, Don; Schermerhorn, Thomas

    2013-01-01

    Extramedullary hematopoiesis (EMH) is the process by which normal blood cells are produced outside the bone marrow. In humans, EMH effusions are rare and are characterized by the presence of megakaryocytes, immature erythrocytes, immature leukocytes, or combinations of those cells. To the authors' knowledge, this is the first report to describe a case of peritoneal EMH effusion in a dog. A 5 yr old castrated male shorthaired dachshund presented with a 2 day history of pigmenturia and inappetence. A complete blood count revealed regenerative anemia with marked agglutination, spherocytosis, and an acute inflammatory leukogram characterized by a neutrophilia, regenerative left shift, and monocytosis. Ultrasound-guided aspiration of peritoneal effusion yielded a sample of high nucleated cellularity predominantly composed of mature and immature neutrophils and erythroid precursor cells. The patient was diagnosed with primary immune-mediated hemolytic anemia with concurrent EMH peritoneal effusion. The following case description and discussion explore the clinical findings associated with the unusual effusion and outline the possible pathogenesis by which the EMH effusion may have arisen in the dog. PMID:23690489

  17. Marrow transplantation in the treatment of a murine heritable hemolytic anemia

    SciTech Connect

    Barker, J.E.; McFarland-Starr, E.C.

    1989-05-15

    Mice with hemolytic anemia, sphha/sphha, have extremely fragile RBCs with a lifespan of approximately one day. Neither splenectomy nor simple transplantation of normal marrow after lethal irradiation cures the anemia but instead causes rapid deterioration and death of the mutant unless additional prophylactic procedures are used. In this report, we show that normal marrow transplantation preceded by sublethal irradiation increases but does not normalize RBC count. The mutant RBCs but not all the WBCs are replaced by donor cells. Splenectomy of the improved recipient causes a dramatic decrease in RBC count, indicating that the mutant spleen is a site of donor-origin erythropoiesis as well as of RBC destruction. Injections of iron dextran did not improve RBC counts. Transplantation of primary recipient marrow cells into a secondary host with a heritable stem cell deficiency (W/Wv) corrects the defect caused by residence of the normal cells in the sphha/sphha host. The original +/+ donor cells replace the RBCs of the secondary host, and the RBC count is normalized. Results indicate that the environment in the sphha/sphha host is detrimental to normal (as well as mutant) erythroid cells but the restriction is not transmitted.

  18. Primary bone marrow lymphoma presenting with cold-type autoimmune hemolytic anemia.

    PubMed

    Kosugi, Shigeki; Watanabe, Mai; Hoshikawa, Masahiro

    2014-09-01

    We report a rare case of primary bone marrow lymphoma with cold-type autoimmune hemolytic anemia (AIHA). A 70-year-old Japanese woman with suspected liver disorder presented to our hospital with palpitation. On physical examination, she had jaundice and signs of anemia. No lymphadenopathy or hepatosplenomegaly was noted. A direct antiglobulin test was positive for complement C3b and C3d. Anti-IgG testing was negative. Cold agglutinin was positive with a titer of 1:?8,192, and haptoglobin was absent. A diagnosis of cold-type AIHA was made. Bone marrow biopsy revealed involvement with a population of lymphocytes that were positive for CD20 (L-26), CD79a, and Bcl-2. No lymphoma lesion was detected on computerized tomography or on upper and lower endoscopy. The patient was diagnosed with diffuse large B cell lymphoma (DLBCL) presenting with cold-type AIHA. She was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone, resulting in complete remission after six cycles. As of 22 months after presentation, no signs of cold-type AIHA or lymphoma were present. PMID:25332595

  19. The simultaneous incidence of acute pancreatitis and autoimmune hemolytic anemia: a rare duo in a patient with SLE

    PubMed Central

    Masoodi, Ibrahim

    2014-01-01

    A young female presented with acute abdominal pain of two days duration consistent with acute pancreatitis. During her stay in the hospital she had a sudden drop in hemoglobin to 6 g/dl without any overt blood loss. On evaluation, it was evident that she had acute pancreatitis, in addition to displaying features of autoimmune hemolytic anemia. She had been a known case of systemic lupus erythematosus (SLE) and had discontinued her treatment. She was managed with methylprednisolone pulse therapy. Her clinical condition improved, and she has been regularly attending our clinic for the last 2 years. According to a literature search in Medline, it would appear that this is the first report of a case in which SLE with autoimmune hemolytic anemia has been associated with acute pancreatitis in a single case. PMID:25276114

  20. Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice

    Microsoft Academic Search

    O. Linderkamp; F. Höpner; H. Klose; K. Riegel; W. Ch. Hecker

    1976-01-01

    A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. Blood volume increased from 93.9 ml\\/kg at the age of 5 h to 124.2

  1. [Serological characteristics and transfusion efficacy evaluation in 61 cases of autoimmune hemolytic anemia].

    PubMed

    Yu, Yang; Sun, Xiao-Lin; Ma, Chun-Ya; Guan, Xiao-Zhen; Zhang, Xiao-Juan; Chen, Lin-Fen; Wang, Ke; Luo, Yuan-Yuan; Wang, Yi; Li, Ming-Wei; Feng, Yan-Nan; Tong, Shan; Yu, Shuai; Yang, Lu; Wu, Yue-Qing; Zhuang, Yuan; Pan, Ji-Chun; Fen, Qian; Zhang, Ting; Wang, De-Qing

    2013-10-01

    This study was aimed to analyze the serological characteristics, efficacy and safety of incompatible RBC transfusion in patients with autoimmune hemolytic anemia (AIHA). The patients with idiopathic or secondary AIHA were analyzed retrospectively, then the serological characteristics and the incidence of adverse transfusion reactions were investigated, and the efficacy and safety of incompatible RBC transfusion were evaluated according to the different autoantibody type and infused different RBC components. The results showed that out of 61 cases of AIHA, 21 cases were idiopathic, and 40 cases were secondary. 8 cases (13.1%) had IgM cold autoantibody, 50 cases (82.0%) had IgG warm autoantibody, and 3 cases (4.9%) had IgM and IgG autoantibodies simultaneously. There were 18 cases (29.5%) combined with alloantibodies. After the exclusion of alloantibodies interference, 113 incompatible RBC transfusions were performed for 36 patients with AIHA, total efficiency rate, total partial efficiency rate and total inefficiency rate were 56.6%, 15.1% and 28.3%, respectively. Incompatible RBC transfusions were divided into non-washed RBC group and washed RBC group. The efficiency rate, partial efficiency rate and inefficiency rate in non-washed RBC group were 57.6%, 13.0% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in washed RBC group were 53.6%, 21.4% and 25.0%, respectively. There was no significant difference of transfusion efficacy (P > 0.05) in two groups. Incompatible RBC transfusions were also divided into IgM cold autoantibody group and IgG warm autoantibody group. The efficiency rate, partial efficiency rate and inefficiency rate in IgM cold autoantibody group were 46.2%, 30.8% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in IgG warm autoantibody group were 56.7%, 13.4% and 29.9%, respectively. There was no significant difference of transfusion efficacy (P > 0.05 ) in two groups. Hemolytic transfusion reaction was not observed in all incompatible RBC transfusions. It is concluded that the same ABO type of non-washed RBC transfusion and O type washed RBC transfusion are all relatively safe for the AIHA patients with severe anemia after the exclusion of alloantibodies interference. There is no significant difference of transfusion efficacy in two groups. The same ABO type of non-washed RBC transfusion is more convenient and efficient than washed RBC transfusion, and excessive use of type O RBCs can also be avoided. PMID:24156449

  2. Partial splenectomy for children with congenital hemolytic anemia and massive splenomegaly.

    PubMed

    Diesen, Diana L; Zimmerman, Sherri A; Thornburg, Courtney D; Ware, Russell E; Skinner, Michael; Oldham, Keith T; Rice, Henry E

    2008-03-01

    Partial splenectomy is an alternative to total splenectomy for the treatment of congenital hemolytic anemias (CHAs) in children, although the feasibility of this technique in the setting of massive splenomegaly is unknown. This study was designed to evaluate the safety and efficacy of partial splenectomy in children with CHAs and massive splenomegaly. This retrospective study examined 29 children with CHAs who underwent partial splenectomy. Children were divided into 2 groups based on splenic size: 8 children had splenic volumes greater than 500 mL, whereas 21 children had splenic volumes less than 500 mL. Outcome variables included perioperative complications, transfusion requirements, hematocrits, reticulocyte counts, bilirubin levels, splenic sequestration, and splenic regrowth. All 29 children underwent successful partial splenectomy with 0.02 to 10 years of follow-up. After partial splenectomy, children overall had decreased transfusion requirements, increased hematocrits, decreased bilirubin levels, decreased reticulocyte counts, and elimination of splenic sequestration. Children with massive splenomegaly had similar outcomes compared with children without massive splenomegaly. Long-term complications included 3 mild infections, 4 cases of gallstones requiring cholecystectomy, and 1 child who required completion splenectomy. Partial splenectomy is a safe, effective, and technically feasible option for children with various CHAs, even in the setting of massive splenomegaly. PMID:18358283

  3. [Blood matching and transfusion for 12 acute autoimmune hemolytic anemia patients by extracorporal hemolysis test].

    PubMed

    Yuan, Min; Tang, Cong-Hai; Wu, A-Yang; Yang, Hui-Cong; Gan, Wei-Wei; Zhang, Tian-Xin; Huang, Yan-Xue; Xu, Wei-Ping

    2014-12-01

    In order to screen the compatible red cells by using extracorporal hemolysis test for acute autoimmune hemolytic anemia (AIHA) patients who were difficult to be matched by automatic microcolumn gel indirect antiglobulin test. Twenty-six cases of AIHA were chosen as control group, to whom the same type of donor red blood cells were infused with the weakest blood agglutination; 12 cases of acute AIHA patients were chosen as test group, these patients were difficult to be matched by automatic microcolumn gel indirect antiglobulin test, and the donor red cells without hemolysis by extracoral hemolysis test were transfused for them. The results showed that compared with the control group,the effect of transfusion was better in test group (P < 0.01), with 2.26 U leukocyte-depleted erythrocyte suspension in average, whose hemoglobin, reticulocyte and total bilirubin levels were changed significantly compared with those before blood transfusion (P < 0.01) . It is concluded that the compatible red blood cells for the acute AIHA patients can be screened by the extracorporal hemolysis test, when it is difficult to screen by the automatic microcolumn gel indirect antiglobulin test. PMID:25543503

  4. Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device

    PubMed Central

    Khawandanah, Mohamad O.; Weiss, Susan M.; Cherry, Mohamad A.; Maymani, Hossein; Selby, George B.; Aster, Richard H.; George, James N.; Holter Chakrabarty, Jennifer L.

    2015-01-01

    BACKGROUND Evans syndrome is a rare condition manifested by combined autoimmune hemolytic anemia (AIHA) and thrombocytopenia or neutropenia. It is often associated with other autoimmune disorders, immunodeficiencies, and non-Hodgkin’s lymphoma. CASE REPORT We describe a patient with Evans syndrome that may have been related to exposure to a polyethylene-based intrauterine contraceptive device (IUD). A 26-year-old white female presented with severe, symptomatic AIHA and subsequently developed severe thrombocytopenia. She had a refractory course resistant to multiple treatments including corticosteroids, intravenous immune globulin, rituximab, splenectomy, cyclophosphamide, cyclosporine, eculizumab, and plasma exchange. It was then noticed that her serum autoantibody agglutinated red blood cells (RBCs) in the presence of polyethylene glycol (PEG) but not in the absence of PEG nor when an alternative agglutination enhancing technique, low-ionic-strength solution, was used. Therefore, her polyethylene-containing IUD, which was a polyethylene frame with a levonorgestrel-releasing device, was removed. Norgestrel-dependent, platelet (PLT)-reactive antibodies were not identified by either flow cytometry or in vivo in a NOD/SCID mouse. Testing for PEG-dependent antibodies was not possible. Remission, with no requirement for RBC or PLT transfusions and return of her hemoglobin and PLT counts to normal, followed removal of the IUD. CONCLUSION The patient’s recovery after removal of the IUD and the PEG dependence of RBC agglutination suggested a possibility that the IUD may have been a contributing factor to the etiology of Evans syndrome in this patient. PMID:25208591

  5. Establishment of permanent chimerism in a lactate dehydrogenase-deficient mouse mutant with hemolytic anemia

    SciTech Connect

    Datta, T.; Doermer, P.

    1987-12-01

    Pluripotent hemopoietic stem cell function was investigated in the homozygous muscle type lactate dehydrogenase (LDH-A) mutant mouse using bone marrow transplantation experiments. Hemopoietic tissues of LDH-A mutants showed a marked decreased in enzyme activity that was associated with severe hemolytic anemia. This condition proved to be transplantable into wild type mice (+/+) through total body irradiation (TBI) at a lethal dose of 8.0 Gy followed by engraftment of mutant bone marrow cells. Since the mutants are extremely radiosensitive (lethal dose50/30 4.4 Gy vs 7.3 Gy in +/+ mice), 8.0-Gy TBI followed by injection of even high numbers of normal bone marrow cells did not prevent death within 5-6 days. After a nonlethal dose of 4.0 Gy and grafting of normal bone marrow cells, a transient chimerism showing peripheral blood characteristics of the wild type was produced that returned to the mutant condition within 12 weeks. The transfusion of wild type red blood cells prior to and following 8.0-Gy TBI and reconstitution with wild type bone marrow cells prevented the early death of the mutants and permanent chimerism was achieved. The chimeras showed all hematological parameters of wild type mice, and radiosensitivity returned to normal. It is concluded that the mutant pluripotent stem cells are functionally comparable to normal stem cells, emphasizing the significance of this mouse model for studies of stem cell regulation.

  6. Reactive oxygen species exacerbate autoimmune hemolytic anemia in New Zealand Black mice.

    PubMed

    Konno, Tasuku; Otsuki, Noriyuki; Kurahashi, Toshihiro; Kibe, Noriko; Tsunoda, Satoshi; Iuchi, Yoshihito; Fujii, Junichi

    2013-12-01

    Elevated reactive oxygen species (ROS) and oxidative damage occur in the red blood cells (RBCs) of SOD1-deficient C57BL/6 mice. This leads to autoimmune responses against RBCs in aged mice that are similar to autoimmune hemolytic anemia (AIHA). We examined whether a SOD1 deficiency and/or the human SOD1 transgene (hSOD1) would affect phenotypes of AIHA-prone New Zealand Black (NZB) mice by establishing three congenic strains: those lacking SOD1, those expressing hSOD1 under a GATA-1 promoter, and those lacking mouse SOD1 but expressing hSOD1. Levels of intracellular ROS and oxidative stress markers increased, and the severity of the AIHA phenotype was aggravated by a SOD1 deficiency. In contrast, the transgenic expression of hSOD1 in an erythroid cell-specific manner averted most of the AIHA phenotype evident in the SOD1-deficient mice and also ameliorated the AIHA phenotype in the mice possessing intrinsic SOD1. These data suggest that oxidative stress in RBCs may be an underlying mechanism for autoimmune responses in NZB mice. These results were consistent with the hypothetical role of reactive oxygen species in triggering the autoimmune reaction in RBCs and may provide a novel approach to mitigating the progression of AIHA by reducing oxidative stress. PMID:24095725

  7. Alemtuzumab Plus Cyclosporine Treatment of the Autoimmune Hemolytic Anemia in an Adult Bowel Transplant

    PubMed Central

    Lauro, A.; Stanzani, M.; Finelli, C.; Zanfi, C.; Morelli, M. C.; Pasqualini, E.; Dazzi, A.; Ravaioli, M.; Di Simone, M.; Giudice, V.; Pironi, L.; Pinna, A. D.

    2014-01-01

    An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft. PMID:25177510

  8. Transfusion support of autoimmune hemolytic anemia: how could the blood group genotyping help?

    PubMed

    El Kenz, Hanane; Efira, André; Le, Phu Quoc; Thiry, Claire; Valsamis, Joseph; Azerad, Marie-Agnès; Corazza, Francis

    2014-01-01

    Conventional pretransfusion testing based on hemagglutination assays can be challenging for patients with autoimmune hemolytic anemia (AIHA) because of the presence of auto-antibodies. It has been suggested that deoxyribonucleic acid-based methods could be more efficient in the selection of antigen-matched red blood cell units in those settings. Because of the high risk of alloimmunization of these patients and the labor-intensive nature of adsorption techniques, we decided to evaluate the feasibility of selecting antigen-matched units on the basis of RBC genotyping. We included in our routine RBC genotyping program samples from 7 patients with AIHA presenting a strongly positive direct antiglobulin test. This made the routine compatibility tests difficult. Most patients had previously received transfusions because of warm AIHA. Matched donor units were selected according to the genotype. For all but 1 patient, blood group genotyping could be done on time to allow antigen-matched transfusion. Four patients received antigen-matched red blood cell units based on RBC genotyping and for 1 patient the fact that no matched units were available led us to postpone the transfusion. After each transfusion, the recovery was recorded and considered satisfactory for all transfused patients. PMID:24120494

  9. Autoimmune hemolytic anemia with gel-based immunohematology tests: neural network analysis.

    PubMed

    Lai, Marco; De Stefano, Valerio; Landolfi, Raffaele

    2014-01-01

    In a previous report, we investigated the capability of commercially available immunohematology tests based on gel technology to add useful information for the diagnosis of autoimmune hemolytic anemia (AIHA). In this report, we analyzed the same casuistic to find useful information on the importance of different immunohematology tests for the AIHA diagnosis, but using the artificial neural network (ANN) analysis. We studied 588 samples with a positive direct antiglobulin test (DAT), of which 52 samples came from patients with AIHA. The samples were analyzed with the ANN using the multilayer perceptron with the backpropagation algorithm. Using the ANN in the observed data set, the predictive value for the presence of AIHAs was 94.7%. The rate of DAT-positive cases that were not AIHA and that were correctly classified was 99.4%. The receiver operating curve area for the model was 0.99. The independent variable importance analysis found that the gel centrifugation test anti-IgG titer was an important contributor to the network performance, but other variables such as the IgG subclasses can also be considered important. The use of the ANN permitted us to identify immunohematology tests that were "hidden" with the common statistical models used previously. This was the case for the IgG subclasses. However, it is very likely that the information given to the network from those tests is quantitative rather than qualitative. PMID:24371011

  10. Alemtuzumab plus cyclosporine treatment of the autoimmune hemolytic anemia in an adult bowel transplant.

    PubMed

    Lauro, A; Stanzani, M; Finelli, C; Zanfi, C; Morelli, M C; Pasqualini, E; Dazzi, A; Ravaioli, M; Di Simone, M; Giudice, V; Pironi, L; Pinna, A D

    2014-01-01

    An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft. PMID:25177510

  11. MicroRNA expression in chronic lymphocytic leukemia developing autoimmune hemolytic anemia.

    PubMed

    Ferrer, Gerardo; Navarro, Alfons; Hodgson, Kate; Aymerich, Marta; Pereira, Arturo; Baumann, Tycho; Monzo, Mariano; Moreno, Carol; Montserrat, Emili

    2013-09-01

    Chronic lymphocytic leukemia (CLL) is frequently associated with autoimmune hemolytic anemia (AIHA). However, the mechanisms governing the association between CLL and AIHA are poorly understood. MicroRNAs (miRNAs) have been associated with different clinico-biological forms of CLL and are also known to play a substantial role in autoimmunity. However, there are no studies correlating miRNA expression with the likelihood that patients with CLL will develop AIHA. In this study, we found that malignant B-cells from patients with CLL subsequently developing AIHA present nine down-regulated (i.e. miR-19a, miR-20a, miR-29c, miR-146b-5p, miR-186, miR-223, miR-324-3p, miR-484 and miR-660) miRNAs. Interestingly, two of these miRNAs (i.e. miR-20a and miR-146b-5p) are involved in autoimmune phenomena, and one (i.e. miR-146b-5p) in both autoimmunity and CLL. Furthermore, we demonstrated that miR-146b-5p modulates CD80, a molecule associated with the B-T-cell synapse and in restoration of the antigen presenting cell capacity of CLL cells. PMID:23286334

  12. Acute generalized exanthematous pustulosis and Coombs-positive hemolytic anemia in a child following Loxosceles reclusa envenomation.

    PubMed

    Lane, Leanna; McCoppin, Holly H; Dyer, Jonathan

    2011-01-01

    Previously reported cases of acute generalized exanthematous pustulosis secondary to brown recluse spider bite have been questioned due to lack of identification of the spider or because of the concomitant administration of antibiotics. We report a 9-year-old boy who arrived at the emergency department with a confirmed Loxosceles reclusa bite to the neck. On the third day of hospitalization, he developed hundreds of monomorphous, sterile pustules, initially in intertriginous areas. The eruption disseminated and was followed by pinpoint desquamation typical for acute generalized exanthematous pustulosis. During this he also developed late onset Coombs-positive hemolytic anemia and systemic loxoscelism. Sphingomyelinase in Loxosceles venom induces the production of interleukin-8 and granulocyte-macrophage colony-stimulating factor, cytokines involved in the pathogenesis of acute generalized exanthematous pustulosis, providing a mechanism by which Loxosceles reclusa bite may trigger acute generalized exanthematous pustulosis. We suggest that this case adds Loxosceles envenomation to the spectrum of agents that can trigger acute generalized exanthematous pustulosis. PMID:22082464

  13. Severe viral hepatitis in a patient with chronic lymphocytic leukemia (CLL) complicated with autoimmune hemolytic anemia (AIHA), treated with steroids.

    PubMed

    Orvain, Corentin; Ducancelle, Alexandra; Eymerit-Morin, Caroline; Rousselet, Marie-Christine; Oberti, Frederic; Hunault-Berger, Mathilde; Tanguy-Schmidt, Aline

    2015-01-01

    Infectious complications are a major cause of morbidity and mortality in patients with chronic lymphocytic leukemia (CLL) due to impaired immunity secondary to the disease itself and to the immunosuppressive therapies administered to these patients. We report a 78-year-old woman with CLL who was treated with steroids for autoimmune hemolytic anemia (AIHA). A few weeks later, she was admitted for severe acute hepatitis with disseminated intravascular coagulation (DIC). Despite the symptomatic treatment of DIC, standard reanimation and probabilistic antibiotics, the patient died within 24h with severe hepatic failure. Autopsy was in favor of a disseminated viral infection with esophageal, hepatic and pulmonary cytopathologic lesions with acidophilic intranuclear inclusions suggestive of herpes virus, even though HSV 1 and 2, CMV and HHV6 PCRs were negative. This case of severe viral hepatitis with esophagitis occurring three weeks after the introduction of high-dose steroid treatment for AIHA in a CLL patient calls for anti-herpetic prophylaxis in such patients, immunodepressed by their diseases and the treatment they receive. PMID:25542474

  14. Metabolic compensation for profound erythrocyte adenylate kinase deficiency. A hereditary enzyme defect without hemolytic anemia.

    PubMed Central

    Beutler, E; Carson, D; Dannawi, H; Forman, L; Kuhl, W; West, C; Westwood, B

    1983-01-01

    A child with hemolytic anemia was found to have severe erythrocyte adenylate kinase (AK) deficiency, but an equally enzyme-deficient sibling had no evidence of hemolysis. No residual enzyme activity was found in erythrocytes by spectrophotometric methods that could easily have detected 0.1% of normal activity. However, concentrated hemolysates were shown to have the capacity to generate small amounts of ATP and AMP from ADP after prolonged incubation. Hemolysates could also catalyze the transfer of labeled gamma-phosphate from ATP to ADP. Intact erythrocytes were able to transfer phosphate from the gamma-position of ATP to the beta-position, albeit at a rate substantially slower than normal. They could also incorporate 14C-labeled adenine into ADP and ATP. Thus, a small amount of residual AK-like activity representing about 1/2,000 of the activity normally present could be documented in the deficient erythrocytes. The residual activity was not inhibited by N-ethylmaleimide, which completely abolishes the activity of the normal AK1 isozyme of erythrocytes. The minute amount of residual activity in erythrocytes could represent a small amount of the AK2 isozyme, which has not been thought to be present in erythrocytes, or the activity of erythrocyte guanylate kinase with AMP substituting as substrate for GMP. Peripheral blood leukocytes, cultured skin fibroblasts, and transformed lymphoblasts from the deficient subject manifested about 17, 24, and 74%, respectively, of the activity of the concurrent controls. This residual activity is consistent with the existence of genetically independent AK isozyme, AK2, which is known to exist in these tissues. The cause of hemolysis in the proband was not identified. Possibilities include an unrelated enzyme deficiency or other erythrocyte enzyme defect and intraction of another unidentified defect with AK deficiency. PMID:6308059

  15. Pulse cyclophosphamide therapy in refractory warm autoimmune hemolytic anemia: a new perspective.

    PubMed

    Thabet, Ahmad F; Faisal, Mostafa

    2014-12-01

    Treatment of steroid refractory autoimmune hemolytic anemia (AIHA) is challenging especially with no evidence based consensus guide lines and limited resources. The aim of this study was to evaluate the efficacy of pulse cyclophosphamide therapy in patients with severe refractory warm AIHA. The prospective study was designed to evaluate the efficacy of pulse cyclophosphamide-1 g/month for four consecutive months-in 17 patients (10 males and 7 females) with severe refractory warm AIHA [13 primary AIHA and 4 (females) secondary to SLE], all studied patients failed to respond to high dose of steroid therapy ± azathioprine ± intravenous immunoglobulin ± oral cyclophosphamide. Mean hemoglobin level, reticulocytic count and direct antiglobulin test were assessed before and after cyclophosphamide treatment every month. After the 4th cycle of cyclophosphamide (82 %, 14 patients) achieved partial response while the remaining (17 %, 3 patients) showed no response, while after 6 months follow up 47 % (8 patients) show complete response, while 53 % (9 patients) showed partial response. The mean hemoglobin levels were significantly increased after the 1st, 2nd, 3rd and 4th months of pulse cyclophosphamide therapy when compared to before treatment (P < 0.01, P < 0.001, P < 0.001 and P < 0.001) respectively, and the mean reticulocyte (%) were significantly decreased after the 2nd, 3rd and 4th months (P < 0.05, P < 0.01 and P < 0.001) respectively. We conclude that pulse cyclophosphamide therapy is well tolerated and induces good response in patients with severe refractory warm AIHA. PMID:25435734

  16. The enzyme-linked immunosorbent assay: accurate detection of red blood cell antibodies in autoimmune hemolytic anemia.

    PubMed

    Bodensteiner, D; Brown, P; Skikne, B; Plapp, F

    1983-02-01

    The enzyme-linked immunosorbent assay (ELISA) was employed in the study of red blood cells from patients with autoimmune hemolytic anemia. The ELISA was more sensitive and correlated with severity of hemolysis better than the direct antiglobulin test (DAT). It was helpful in diagnosing and following the clinical course in these patients. This was particularly true in the DAT-negative group, since the ELISA can detect smaller increases in red blood cell IgG than are required for a positive DAT. PMID:6823902

  17. A Puzzle of Hemolytic Anemia, Iron and Vitamin B12 Deficiencies in a 52-Year-Old Male

    PubMed Central

    Liana, Palacci; Ali, Alaa M.; Gilman, Alan D.

    2013-01-01

    A 52-year-old male with no significant past medical history reports increasing generalized fatigue and weakness for the past 2 weeks. Physical examination reveals jaundice and pallor without organomegaly or lymphadenopathy. His hemoglobin was 5.9?g/dL with a mean corpuscular volume of 87.1?fL and elevated red blood cell distribution width of 30.7%. His liver function test was normal except for elevated total bilirubin of 3.7?mg/dL. Serum LDH was 701?IU/L, and serum haptoglobin was undetectable. Further investigation revealed serum vitamin B12 of <30?pg/mL with elevated methylmalonic acid and homocysteine level. In addition, serum ferritin and transferrin saturation were low. The patient was diagnosed with hemolytic anemia secondary to vitamin B12 deficiency with concomitant iron deficiency anemia. PMID:24083040

  18. Pure red cell aplasia accompanied by autoimmune hemolytic anemia in a patient with type A viral hepatitis.

    PubMed

    Koiso, Hiromi; Kobayashi, Satsuki; Ueki, Kazue; Hamada, Tetsuya; Tsukamoto, Norifumi; Karasawa, Masamitsu; Murakami, Hirokazu; Nojima, Yoshihisa

    2009-05-01

    A rare case of acute hepatitis A associated with autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) is reported. A 55-year-old woman consulted a doctor because of common cold-like symptoms and she was referred to our hospital in January 2007. Laboratory findings showed a marked elevation of serum transaminase and total bilirubin levels (AST 9,605 IU/l, ALT 5,546 IU/l and T-bil 4.14 mg/dl), and prolonged prothrombin time, findings which suggested the risk of progression to fulminant hepatitis, and she was treated with plasmapheresis and hemodialysis filtration on the first and second hospital days. She was diagnosed with severe acute hepatitis A based on the elevation of serum IgM anti-hepatitis A virus. On the 20th hospital day, her hemoglobin level began to decrease in spite of improving transaminase levels without any signs of gastrointestinal bleeding. Bilirubin and LDH elevation, haptoglobin decline and a positive direct Coombs test were detected and these findings indicated AIHA complication; however, the reticulocyte count decreased and bone marrow showed marked erythroid hypoplasia so the co-existence of PRCA was diagnosed. After oral prednisolone administration (1 mg/kg/day), her hemolytic anemia rapidly improved. PMID:19483404

  19. Heterozygous pyruvate kinase deficiency and severe hemolytic anemia in a pregnant woman with concomitant, glucose-6-phosphate dehydrogenase deficiency.

    PubMed

    Vives Corrons, J L; García, A M; Sosa, A M; Pujades, A; Colomer, D; Linares, M

    1991-05-01

    The aim of this paper is to describe the clinical and hematological characteristics of a 32-year-old woman with concomitant heterozygous pyruvate kinase (PK) and glucose-6-phosphate dehydrogenase (G6PD) deficiencies and severe hemolytic anemia during pregnancy. In 1964, Oski et al. described a family in which a clinically healthy woman was heterozygous for both PK and G6PD deficiencies. To our knowledge, the present case is the first described in which the same condition is associated with hemolysis. A heterozygous condition for both enzymopathies was clearly demonstrated by family study criteria, and all other causes of hemolytic anemia were eliminated. No evidence of genetic relationship between the two disorders was demonstrated. Since late onset of hemolysis in heterozygous PK-deficient women has been observed in association with pregnancy and the molecular characteristics of the concomitant deficient G6PD enzyme were kinetically favorable, partial PK deficiency is suggested as the major cause of hemolysis in this patient. PMID:2049468

  20. Molecular modelling of human red blood cell pyruvate kinase: structural implications of a novel G1091 to A mutation causing severe nonspherocytic hemolytic anemia

    Microsoft Academic Search

    Wouter W. van Solinge; Rob J. Kraaijenhagen; Gert Rijksen; R ichard van Wijk; Bjarne B. Stoffer; Michael Gajhede; Finn C. Nielsen

    1997-01-01

    We present a novel G1091 to A mutation in the human liver of RBC PK, whereas the G1529 to A mutation leads to the and red blood cell (RBC) pyruvate kinase (PK) gene causing substitution of a conserved arginine residue with glutamine severe hemolytic anemia. In two families, three children in the C-domain. Molecular modelling of human RBC PK, were

  1. Successful treatment of severe immune hemolytic anemia after allogeneic stem cell transplantation with bortezomib: report of a case and review of literature

    PubMed Central

    Hosoba, Sakura; Jaye, David L; Cohen, Cynthia; Roback, John D; Waller, Edmund K

    2015-01-01

    Background Immune hemolytic anemia is a well-known complication after allogeneic hematopoietic stem cell transplantation (HSCT). Posttransplant hemolytic anemia results in increased red blood cell transfusions and medical sequelae including iron overload. Case Report We present a case report of immune hemolytic anemia that occurred after allogeneic HSCT from an ABO major–mismatched, HLA-matched unrelated donor. The patient had high anti-donor A type antibodies that were unresponsive to treatment with steroids and rituximab, resulting in persistent transfusion dependence. A detailed time course of anti-A titers, plasma cell content of the marrow, and B-cell content of the blood is presented. Treatment with bortezomib, a protease inhibitor, eliminated residual host-type plasma cells secreting anti-A and restored normal donor-derived erythropoiesis. Conclusion This report, and a review of literature for treatment of immune hemolytic anemia after allogeneic HSCT, supports the utility of bortezomib as plasma cell–targeted therapy in this setting. PMID:25156334

  2. Delayed-onset hemolytic anemia in patients with travel-associated severe malaria treated with artesunate, france, 2011-2013.

    PubMed

    Jauréguiberry, Stéphane; Thellier, Marc; Ndour, Papa Alioune; Ader, Flavie; Roussel, Camille; Sonneville, Romain; Mayaux, Julien; Matheron, Sophie; Angoulvant, Adela; Wyplosz, Benjamin; Rapp, Christophe; Pistone, Thierry; Lebrun-Vignes, Bénédicte; Kendjo, Eric; Danis, Martin; Houzé, Sandrine; Bricaire, François; Mazier, Dominique; Buffet, Pierre; Caumes, Eric

    2015-05-01

    Artesunate is the most effective treatment for severe malaria. However, delayed-onset hemolytic anemia has been observed in ?20% of travelers who receive artesunate, ?60% of whom require transfusion. This finding could discourage physicians from using artesunate. We prospectively evaluated a cohort of 123 patients in France who had severe imported malaria that was treated with artesunate; our evaluation focused on outcome, adverse events, and postartesunate delayed-onset hemolysis (PADH). Of the 123 patients, 6 (5%) died. Overall, 97 adverse events occurred. Among the 78 patients who received follow-up for >8 days after treatment initiation, 76 (97%) had anemia, and 21 (27%) of the 78 cases were recorded as PADH. The median drop in hemoglobin levels was 1.3 g/dL; 15% of patients with PADH had hemoglobin levels of <7 g/dL, and 1 required transfusion. Despite the high incidence of PADH, the resulting anemia remained mild in 85% of cases. This reassuring result confirms the safety and therapeutic benefit of artesunate. PMID:25898007

  3. Severe refractory autoimmune hemolytic anemia with five-year complete hematologic response to third course of treatment with rituximab: a case report

    PubMed Central

    2014-01-01

    Introduction Rituximab is an emerging treatment for autoimmune hemolytic anemia. We report the case of a patient with a five-year complete hematologic response to a third course of treatment with rituximab. Cases of response to rituximab re-treatments have been reported, but none to our knowledge that failed multiple prior treatments and achieved as durable a response. Case presentation A 45-year-old Hispanic man presented at age 26 with darkening urine and cold intolerance. His blood tests revealed elevated lactic dehydrogenase and bilirubin, a hemoglobin level of 7.4g/dL, and a positive Coombs test for complement C3 and immunoglobulin G antibody. A diagnosis of autoimmune hemolytic anemia was made. After failing multiple therapies including prednisone, splenectomy, immunoglobulin, cyclosporine, danocrine and azathioprine, our patient was treated with a four-week course of rituximab at a dose of 375mg/m2 weekly, 10 years following initial presentation. He achieved a rapid and complete hematologic response that lasted 25 months. Re-treatment with the same course of rituximab prompted a second response that lasted 18 months. A third re-treatment has achieved an ongoing five-year complete hematologic response. Conclusions This is an unusual case of a durable five-year remission of autoimmune hemolytic anemia with rituximab re-treatment following relapse after two prior courses of rituximab and despite the persistence of immunoglobulin G and complement-coated red blood cells. No mechanistic explanations for improved response to rituximab re-treatment in autoimmune hemolytic anemia have been reported in the literature. Future studies of rituximab or other B cell-targeting antibodies in the treatment of autoimmune hemolytic anemia should explore autoantibody immunoglobulin G subclass switching and alterations in complement inhibitory proteins on red blood cell membranes as potential correlates of hematologic response. PMID:24889270

  4. [Enzyme deficiencies in glycolysis and nucleotide metabolism of red blood cells in nonspherocytic hemolytic anemia (author's transl)].

    PubMed

    Waller, H D; Benöhr, H C

    1976-09-01

    The detection of enzyme deficiencies in glycolytic and nucleotide metabolism of human red blood cells has enriched the pathophysiological knowledge on the origin of nonspherocytic hemolytic anemias (NSHA). So far for 11 of 13 glycolytic enzymes deficiencies have been described which are connected with alterations of biochemical enzymatic properties. The most frequent enzyme deficiencies are those of GPI and PK. By performance of special electrophoretic techniques genetic studies allow the demonstration of homozygote and double heterozygote defect carriers. Up to now only adenylate kinase and pyrimidine 5' nucleotidase deficiencies have been detected as genetically determined in altered nucleotide metabolism. The metabolic alterations of several enzymopathies have been characterized so well, that the pathophysiological relations between enzyme deficiency and NSHA probably have been found to be a sufficient explanation. PMID:184346

  5. Reappraisal of the Etiology of Extracorpuscular Non-Autoimmune Acquired Hemolytic Anemia in 2657 Hospitalized Patients with Non-Neoplastic Disease

    PubMed Central

    Kok, Victor C; Lee, Chien-Kuan; Horng, Jorng-Tzong; Lin, Che-Chen; Sung, Fung-Chang

    2014-01-01

    INTRODUCTION Unlike autoimmune hemolytic anemia (AIHA), literature on the etiological study of non-autoimmune hemolytic anemia (non-AIHA) is scarce. The incidence and prevalence of non-AIHA in different geographic regions are largely unknown perhaps owing to the lack of perspective investigation and different profiles of etiologies from different geographic regions. We aimed to examine the real-world etiology or mechanisms of the non-hereditary non-AIHA from a nationwide population-based administrative claim database in Taiwan. PATIENTS AND METHODS The National Health Insurance Research Database of Taiwan was adopted for this research. The studied population was total inpatient claim records including both pediatric and adult patients, contributed by a population of 23 million insured individuals in Taiwan. From 2002 to 2008, we retrieved 3,903 patients having no pre-existing malignancy discharged after inpatient management for acquired hemolytic anemia, which was defined as coding in discharge diagnoses containing ICD-9-CM code 283. By contrast, ICD-9-CM code 282 and all of the sub-codes are for hereditary hemolytic anemias. RESULTS AIHA accounted for 32% of the total cases. Among 2,657 patients with non-AIHA, mechanical or microangiopathic mechanism accounted for 19% of cases; hemolytic-uremic syndrome (HUS) 4%, hemoglobinuria because of hemolysis from external causes such as paroxysmal nocturnal hemoglobinuria (PNH) and march hemoglobinuria 7%, and chronic idiopathic hemolytic anemia or other unspecified non-AIHA 69%. We looked further for specific etiology or mechanism for this group of patients with non-hereditary extrinsic non-AIHA (n = 2,657). The explanatory disease states or conditions were splenomegaly; alcohol use disorder (spur cell hemolysis); heart-valve prosthesis; malignant hypertension; disseminated intravascular coagulation; transfusion reaction; dengue fever-induced hemolytic anemia; direct parasitization; snake, lizard, or spider bite; and Wilson’s disease with internal toxin mechanism. All these cases can explain up to 34.6% of all the non-hereditary extrinsic non-AIHA cases. Fragmentation hemolysis (HUS, heart-valve prosthesis, malignant hypertension, and disseminated intravascular coagulation) accounted for 7.4% of non-AIHA hospitalized patients with non-neoplastic disease. CONCLUSIONS This article is the first one to clearly demonstrate that the non-neoplastic-induced HUS requiring hospitalization cases in Taiwan, which has a population of over 23 million were 110 over a span of seven years, 16 cases per year. Although the etiologies of non-AIHA are well known and described in the literature, this work added the statistical percentages of the various etiologies of non-AIHA in Taiwan. PMID:24808725

  6. [Immune and metabolic effects of carnitine and biotin in hemolytic anemia].

    PubMed

    Lazareva, G A; Prokopenko, L G; Uteshev, B S

    2002-01-01

    Single (80 mg/kg) or multiple (3 mg/kg) intramuscular introduction of phenylhydrazine decreases the functional activity of mononuclear blood cells and the immunological reactivity of the organism. Carnitine and biotin do not significantly influence the immunological response to a single administration of phenylhydrazine but noticeably reduce the expression of changes produced by repeated injections of the hemolytic toxin. PMID:12227094

  7. Age and dose sensitivities in the 2-butoxyethanol F344 rat model of hemolytic anemia and disseminated thrombosis.

    PubMed

    Ramot, Yuval; Lewis, Deborah A; Ortel, Thomas L; Streicker, Mike; Moser, Glenda; Elmore, Susan; Ward, Sandra M; Peddada, Shyamal; Nyska, Abraham

    2007-04-01

    In hemolytic disorders, such as sickle cell disease and beta-thalassemia, the mechanisms of thrombosis are poorly understood. Appropriate animal models would increase the understanding of the pathophysiology of thrombosis. We previously reported that rats exposed to 2-butoxyethanol (2-BE) developed hemolytic anemia and disseminated thrombosis resembling sickle cell disease and beta-thalassemia. To characterize our model further, we investigated age- and dose-related differences in sensitivity to 2-BE. We exposed groups of 6- and 12-week-old F344 rats (5 animals/group) to 62.5, 125, and 250 mg/kg/day of 2-BE for up to 4 days. Blood was collected on days 2-4 for complete blood count and measurement of intracellular adhesion molecule-1 (ICAM-1). Histopathological evaluation was performed to find evidence of disseminated thrombosis. The maximum hemolytic response, resulting in decreased erythrocyte count and higher mean cell volume (MCV) occurred in the 12-week-old rats treated with the highest dose of 2-BE (250 mg/kg, p<0.0001). The highest increase in ICAM-1 levels occurred in the 12-week-old rats treated with 125 and 250 mg/kg 2-BE (p<0.0001). No intravascular thrombi were noted in the 6-week-old 2-BE-treated animals. The majority of intravascular thrombi occurred in the 12-week-old rats treated with 250 mg/kg 2-BE. Because our findings show age- and dose-related sensitivities, we suggest that 12-week-old rats and doses of 250 mg/kg be used in the 2-BE model. PMID:17261363

  8. Serologic profile of alphamethyldopa-induced hemolytic anemia: correlation between cell-bound IgM and hemolysis.

    PubMed

    Lalezari, P; Louie, J E; Fadlallah, N

    1982-01-01

    Erythrocyte-bound immunoglobulins have been characterized by a PVP-potentiated antiglobulin test in 11 patients who had developed antibodies after treatment with alpha-methyldopa. Serologic profiles were recognized that could distinguish between the hemolyzing and nonhemolyzing patients: IgM antibodies together with the first component of complement (C1q) were demonstrated on erythrocytes of all eight hemolyzing patients. By contrast, these immunoproteins were absent from the cells of nonhemolyzing patients and became undetectable when the hemolyzing patients recovered. IgG and its subclasses were variably present on erythrocytes of all patients regardless of hemolytic activity. Eluates prepared from erythrocytes of the hemolyzing patients were shown to contain both IgG and IgM, and fixes C1q, C3, and C4. Eluates from the nonhemolyzing patients contained only IgG. The IgM antibodies differed from the commonly occurring cold agglutinins in that they were warm-reactive and were mainly concentrated on the patients' cells rather than being free in the serum. Because of their nonagglutinating property, it is suggested that they are monomeric IgM. It is concluded that the high affinity, warm-reactive IgM and not the IgG antibodies are primarily responsible for clinically manifest anemia in patients receiving alphamethyldopa and that the hemolytic activity is probably mediated by the classic pathway of complement activation. PMID:7053765

  9. Fetal and neonatal anemia associated with anti-Jr(a) : a case report showing a poorly hemolytic mechanism.

    PubMed

    Sasamoto, Naoko; Tomimatsu, Takuji; Nagamine, Keisuke; Oshida, Machiko; Kashiwagi, Hirokazu; Koyama, Shinsuke; Kanagawa, Takeshi; Arahori, Hitomi; Tomiyama, Yoshiaki; Kimura, Tadashi

    2011-08-01

    Although recently published case reports suggest the significance of Jr(a) alloimmunization in the obstetric setting, the involved mechanism still remains unclear. Here we report a case of severe fetal and neonatal anemia associated with anti-Jr(a) alloimmunization, which was successfully managed using Doppler assessment of peak systolic velocity of the fetal middle cerebral artery (MCA-PSV). A Japanese woman with anti-Jr(a) (titer 1024) was referred to our department at 20 weeks' gestation. As fetal MCA-PSV exceeded 1.5 multiple of median, labor was induced and a female neonate of 1998 g was delivered vaginally at 33 weeks and 5 days of gestation. The infant's hematocrit and hemoglobin levels were 25.4% and 82 g/L, respectively, but her total bilirubin level (15 µmol/L; 0.9 mg/dL) and reticulocyte counts (4.5%) were low. During the course, the infant showed no apparent signs of hemolysis. Jr(a) alloimmunization should be recognized as a possible cause of fetal anemia with no direct hemolytic process. PMID:21481087

  10. Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients.

    PubMed

    Barcellini, Wilma; Fattizzo, Bruno; Zaninoni, Anna; Radice, Tommaso; Nichele, Ilaria; Di Bona, Eros; Lunghi, Monia; Tassinari, Cristina; Alfinito, Fiorella; Ferrari, Antonella; Leporace, Anna Paola; Niscola, Pasquale; Carpenedo, Monica; Boschetti, Carla; Revelli, Nicoletta; Villa, Maria Antonietta; Consonni, Dario; Scaramucci, Laura; De Fabritiis, Paolo; Tagariello, Giuseppe; Gaidano, Gianluca; Rodeghiero, Francesco; Cortelezzi, Agostino; Zanella, Alberto

    2014-11-01

    The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels ?6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb ?6 g/dL; P < .001). Thrombotic events were associated with Hb levels ?6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians. PMID:25232059

  11. A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia

    PubMed Central

    Hanson, Madelyn S.; Xu, Hao; Flewelen, Timothy C.; Holzhauer, Sandra L.; Retherford, Dawn; Jones, Deron W.; Frei, Anne C.; Pritchard, Kirkwood A.; Hillery, Cheryl A.; Hogg, Neil

    2013-01-01

    Hemolysis can saturate the hemoglobin (Hb)/heme scavenging system, resulting in increased circulating cell-free Hb (CF-Hb) in hereditary and acquired hemolytic disease. While recent studies have suggested a central role for intravascular hemolysis and CF-Hb in the development of vascular dysfunction, this concept has stimulated considerable debate. This highlights the importance of determining the contribution of CF-Hb to vascular complications associated with hemolysis. Therefore, a novel Hb-binding peptide was synthesized and linked to a small fragment of apolipoprotein E (amino acids 141–150) to facilitate endocytic clearance. Plasma clearance of hE-Hb-b10 displayed a rapid phase t1/2 of 16 min and slow phase t1/2 of 10 h, trafficking primarily through the liver. Peptide hE-Hb-B10 decreased CF-Hb in mice treated with phenylhydrazine, a model of acute hemolysis. Administration of hE-Hb-B10 also attenuated CF-Hb in two models of chronic hemolysis: Berkeley sickle cell disease (SS) mice and mice with severe hereditary spherocytosis (HS). The hemolytic rate was unaltered in either chronic hemolysis model, supporting the conclusion that hE-Hb-B10 promotes CF-Hb clearance without affecting erythrocyte lysis. Interestingly, hE-Hb-B10 also decreased plasma ALT activity in SS and HS mice. Although acetylcholine-mediated facialis artery vasodilation was not improved by hE-Hb-B10 treatment, the peptide shifted vascular response in favor of NO-dependent vasodilation in SS mice. Taken together, these data demonstrate that hE-Hb-B10 decreases CF-Hb with a concomitant reduction in liver injury and changes in vascular response. Therefore, hE-Hb-B10 can be used to investigate the different roles of CF-Hb in hemolytic pathology and may have therapeutic benefit in the treatment of CF-Hb-mediated tissue damage. PMID:23125208

  12. AMPD3-deficient mice exhibit increased erythrocyte ATP levels but anemia not improved due to PK deficiency.

    PubMed

    Cheng, Jidong; Morisaki, Hiroko; Toyama, Keiko; Ikawa, Masahito; Okabe, Masaru; Morisaki, Takayuki

    2012-11-01

    AMP deaminase (AMPD) catalyzes AMP to IMP and plays an important role in energy charge and nucleotide metabolism. Human AMPD3 deficiency is a type of erythrocyte-specific enzyme deficiency found in individuals without clinical symptoms, although an increased level of ATP in erythrocytes has been reported. To better understand the physiological and pathological roles of AMPD3 deficiency, we established a line of AMPD3-deficient [A3(-/-)] mice. No AMPD activity and a high level of ATP were observed in erythrocytes of these mice, similar to human RBC-AMPD3 deficiency, while other characteristics were unremarkable. Next, we created AMPD3 and pyruvate kinase (PK) double-deficient [PKA(-/-,-/-)] mice by mating A3(-/-) mice with CBA-Pk-1slc/Pk-1slc mice [PK(-/-)], a spontaneous PK-deficient strain showing hemolytic anemia. In PKA(-/-,-/-) mice, the level of ATP in red blood cells was increased 1.5 times as compared to PK(-/-) mice, although hemolytic anemia in those animals was not improved. In addition, we observed osmotic fragility of erythrocytes in A3(-/-) mice under fasting conditions. In contrast, the ATP level in erythrocytes was elevated in A3(-/-) mice as compared to the control. In conclusion, AMPD3 deficiency increases the level of ATP in erythrocytes, but does not improve anemia due to PK deficiency and leads to erythrocyte dysfunction. PMID:23078545

  13. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia

    PubMed Central

    Saleemi, Sarfraz

    2014-01-01

    Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide are living with sickle cell disease, while 60-80 million carry beta thalassemia trait. About 400,000 children are born with severe hemoglobinopathies each year. Cardiovascular complications of hemoglobinopathies include left and right ventricular (RV) dysfunction, arrhythmias, pericarditis, myocarditis, valvular heart disease, myocardial ischemia, and notably pulmonary hypertension (PH). Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established. PMID:25077000

  14. B-cell receptor configuration and adverse cytogenetics are associated with autoimmune hemolytic anemia in chronic lymphocytic leukemia.

    PubMed

    Maura, Francesco; Visco, Carlo; Falisi, Erika; Reda, Gianluigi; Fabris, Sonia; Agnelli, Luca; Tuana, Giacomo; Lionetti, Marta; Guercini, Nicola; Novella, Elisabetta; Nichele, Ilaria; Montaldi, Anna; Autore, Francesco; Gregorini, Anna; Barcellini, Wilma; Callea, Vincenzo; Mauro, Francesca R; Laurenti, Luca; Foà, Robin; Neri, Antonino; Rodeghiero, Francesco; Cortelezzi, Agostino

    2013-01-01

    The development of autoimmune hemolytic anemia (AIHA) in patients with chronic lymphocytic leukemia (CLL) is associated with specific biological features. The occurrence of AIHA was hereby investigated in a retrospective series of 585 CLL patients with available immunoglobulin heavy chain variable (IGHV) gene status. AIHA occurred in 73 patients and was significantly associated with an IGHV unmutated (UM) status (P < 0.0001) and unfavorable [del(17)(p13) and del(11)(q23)] cytogenetic lesions (P < 0.0001). Stereotyped HCDR3 sequences were identified in 29.6% of cases and were similarly represented among patients developing or not AIHA; notably, subset #3 was associated with a significantly higher risk of AIHA than the other patients (P = 0.004). Multivariate analysis showed that UM IGHV, del(17)(p13) and del(11)(q23), but not stereotyped subset #3, were the strongest independent variables associated with AIHA. Based on these findings, we generated a biological risk score for AIHA development according to the presence of none (low risk), one (intermediated risk), or two (high risk) of the independent risk factors. Overall, our data indicate that UM IGHV status and/or unfavorable cytogenetic lesions are associated with the risk of developing secondary AIHA in CLL patients and suggest a possible role of specific stereotyped B-cell receptor subsets in a proportion of cases. PMID:23115077

  15. Red Blood Cell Destruction in Autoimmune Hemolytic Anemia: Role of Complement and Potential New Targets for Therapy

    PubMed Central

    Berentsen, Sigbjørn

    2015-01-01

    Autoimmune hemolytic anemia (AIHA) is a collective term for several diseases characterized by autoantibody-initiated destruction of red blood cells (RBCs). Exact subclassification is essential. We provide a review of the respective types of AIHA with emphasis on mechanisms of RBC destruction, focusing in particular on complement involvement. Complement activation plays a definitive but limited role in warm-antibody AIHA (w-AIHA), whereas primary cold agglutinin disease (CAD), secondary cold agglutinin syndrome (CAS), and paroxysmal cold hemoglobinuria (PCH) are entirely complement-dependent disorders. The details of complement involvement differ among these subtypes. The theoretical background for therapeutic complement inhibition in selected patients is very strong in CAD, CAS, and PCH but more limited in w-AIHA. The optimal target complement component for inhibition is assumed to be important and highly dependent on the type of AIHA. Complement modulation is currently not an evidence-based therapy modality in any AIHA, but a number of experimental and preclinical studies are in progress and a few clinical observations have been reported. Clinical studies of new complement inhibitors are probably not far ahead. PMID:25705656

  16. Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient

    PubMed Central

    Philip, Joseph; Jain, Neelesh

    2014-01-01

    Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies) can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA), and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg) and rituximab (anti-CD20 monoclonal antibody). PMID:25161355

  17. Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient.

    PubMed

    Philip, Joseph; Jain, Neelesh

    2014-07-01

    Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies) can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA), and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg) and rituximab (anti-CD20 monoclonal antibody). PMID:25161355

  18. Deficiency of nicotinamide mononucleotide adenylyltransferase 3 (nmnat3) causes hemolytic anemia by altering the glycolytic flow in mature erythrocytes.

    PubMed

    Hikosaka, Keisuke; Ikutani, Masashi; Shito, Masayuki; Kazuma, Kohei; Gulshan, Maryam; Nagai, Yoshinori; Takatsu, Kiyoshi; Konno, Katsuhiro; Tobe, Kazuyuki; Kanno, Hitoshi; Nakagawa, Takashi

    2014-05-23

    NAD biosynthesis is of substantial interest because of its important roles in regulating various biological processes. Nicotinamide mononucleotide adenylyltransferase 3 (Nmnat3) is considered a mitochondria-localized NAD synthesis enzyme involved in de novo and salvage pathways. Although the biochemical properties of Nmnat3 are well documented, its physiological function in vivo remains unclear. In this study, we demonstrated that Nmnat3 was localized in the cytoplasm of mature erythrocytes and critically regulated their NAD pool. Deficiency of Nmnat3 in mice caused splenomegaly and hemolytic anemia, which was associated with the findings that Nmnat3-deficient erythrocytes had markedly lower ATP levels and shortened lifespans. However, the NAD level in other tissues were not apparently affected by the deficiency of Nmnat3. LC-MS/MS-based metabolomics revealed that the glycolysis pathway in Nmnat3-deficient erythrocytes was blocked at a glyceraldehyde 3-phosphate dehydrogenase (GAPDH) step because of the shortage of the coenzyme NAD. Stable isotope tracer analysis further demonstrated that deficiency of Nmnat3 resulted in glycolysis stall and a shift to the pentose phosphate pathway. Our findings indicate the critical roles of Nmnat3 in maintenance of the NAD pool in mature erythrocytes and the physiological impacts at its absence in mice. PMID:24739386

  19. Narrative Review: Paroxysmal Nocturnal Hemoglobinuria: The Physiology of Complement-Related Hemolytic Anemia

    NSDL National Science Digital Library

    Robert A. Brodsky (Johns Hopkins Medicine)

    2008-04-15

    Physiology in Medicine review article. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem-cell disorder caused by a somatic mutation in a gene known as phosphatidylinositol glycan class A (PIGA). It may arise de novo or in the setting of acquired aplastic anemia.The absence of GPI-anchored proteins leads to complement-mediated intravascular hemolysis, because 2 important complement regulatory proteins (CD55 and CD59) are missing from PNH cells. Therapeutic options include supportive care, bone marrow transplantation, and monoclonal antibody therapy with the terminal complement inhibitor eculizumab.

  20. IgG red blood cell autoantibodies in autoimmune hemolytic anemia bind to epitopes on red blood cell membrane band 3 glycoprotein

    SciTech Connect

    Victoria, E.J.; Pierce, S.W.; Branks, M.J.; Masouredis, S.P. (Univ. of California, San Diego (USA))

    1990-01-01

    Red blood cell (RBC) autoantibodies from patients with IgG warm-type autoimmune hemolytic anemia were labeled with iodine 125 and their RBC binding behavior characterized. Epitope-bearing RBC membrane polypeptides were identified after autoantibody immunoprecipitation of labeled membranes and immunoblotting. Immunoaffinity isolation of labeled membrane proteins with 12 different IgG hemolytic autoantibodies with protein A-agarose revealed a major polypeptide at Mr 95 to 110 kd, which coelectrophoresed on sodium dodecylsulfate-polyacrylamide gel electrophoresis with a membrane component isolated with sheep IgG anti-band 3. Immunoprecipitation studies with chymotrypsinized RBCs resulted in the recovery of two labeled membrane polypeptides with molecular weights characteristically resulting from the chymotryptic fragmentation of band 3. Immunoblotting with sheep IgG anti-band 3 of the immunoprecipitated polypeptides confirmed that hemolytic autoantibody binding led to recovery of band 3 or its fragments. Two 125I-labeled IgG hemolytic autoantibodies showed binding behavior consistent with epitope localization on band 3. The labeled RBC autoantibodies bound immunospecifically to all types of human RBC tested, including those of rare Rh type (Rh-null, D--) at a site density of approximately 10(6) per RBC. The 125I-IgG in two labeled autoantibodies was 84% and 92% adsorbable by human and higher nonhuman primate RBCs. Antigen-negative animal RBC bound less than 10%, consistent with immunospecific RBC binding. IgG-1 was the major subclass in five autoantibodies tested; one of six fixed complement; and autoantibody IgG appeared polyclonal by isoelectric focusing. We conclude that IgG eluted from RBCs of patients with autoimmune hemolytic anemia consists predominantly of a single totally RBC-adsorbable antibody population that binds to antigenic determinants on band 3.

  1. Three episodes of delayed hemolytic transfusion reactions due to multiple red cell antibodies, anti-Di, anti-Jk and anti-E.

    PubMed

    Yasuda, H; Ohto, H; Yamaguchi, O; Sakuma, S; Suzuki, T; Mita, M; Tsuneyama, H; Uchikawa, M

    2000-10-01

    There is no report in which three episodes of delayed hemolytic transfusion reaction (DHTR) occurred from multiple antibodies to red cells (RBCs) in the course of treatment of a patient. This paper describes episodes of anemia and hyperbilirubinemia in concert with the development of three alloantibodies in a multiple transfused patient. The patient was a 71-year-old male suffering from valvular heart disease and hemophilia B with a history of transfusions. Although he received compatible RBCs from 14 donors as judged by a crossmatch test using the albumin-antiglobulin method, three episodes of DHTR occurred after surgery. The first hemolytic episode on day 7 after surgery was due to anti-Di(a) because of clinical and laboratory evidence which included jaundice, sudden increases in total bilirubin (T-Bil) and lactate dehydrogenase (LD) levels, and a decrease (2.2 g/dl) in hemoglobin (Hb) level. The second hemolytic episode on day 16 resulted from newly producted anti-Jk(b). The patient experienced fever, fatigue, nausea and anorexia, and laboratory data showed a second increase in T-Bil, a second decrease (3 g/dl) in Hb, and moderate elevations of blood urea nitrogen (BUN) and creatinine (CRE) levels. The third hemolytic episode on day 39 was due to anti-E. The patient complained of fever and fatigue and had a third unexplained drop (1.5 g/dl) in Hb despite no bleeding. This is the first reported case in which three episodes of DHTR occurred from different red cell antibodies. PMID:11035271

  2. Anemia (For Parents)

    MedlinePLUS

    ... of inherited hemolytic anemia include sickle cell anemia, thalassemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency, and hereditary ... children is born with this form of anemia. Thalassemia , which usually affects people of Mediterranean, African, and ...

  3. Assessment of the Red Cell Proteome of Young Patients with Unexplained Hemolytic Anemia by Two-Dimensional Differential In-Gel Electrophoresis (DIGE)

    PubMed Central

    von Löhneysen, Katharina; Scott, Thomas M.; Soldau, Katrin; Xu, Xiuling; Friedman, Jeffrey S.

    2012-01-01

    Erythrocyte cytosolic protein expression profiles of children with unexplained hemolytic anemia were compared with profiles of close relatives and controls by two-dimensional differential in-gel electrophoresis (2D-DIGE). The severity of anemia in the patients varied from compensated (i.e., no medical intervention required) to chronic transfusion dependence. Common characteristics of all patients included chronic elevation of reticulocyte count and a negative workup for anemia focusing on hemoglobinopathies, morphologic abnormalities that would suggest a membrane defect, immune-mediated red cell destruction, and evaluation of the most common red cell enzyme defects, glucose-6-phosphate dehydrogenase and pyruvate kinase deficiency. Based upon this initial workup and presentation during infancy or early childhood, four patients classified as hereditary nonspherocytic hemolytic anemia (HNSHA) of unknown etiology were selected for proteomic analysis. DIGE analysis of red cell cytosolic proteins clearly discriminated each anemic patient from both familial and unrelated controls, revealing both patient-specific and shared patterns of differential protein expression. Changes in expression pattern shared among the four patients were identified in several protein classes including chaperons, cytoskeletal and proteasome proteins. Elevated expression in patient samples of some proteins correlated with high reticulocyte count, likely identifying a subset of proteins that are normally lost during erythroid maturation, including proteins involved in mitochondrial metabolism and protein synthesis. Proteins identified with patient-specific decreased expression included components of the glutathione synthetic pathway, antioxidant pathways, and proteins involved in signal transduction and nucleotide metabolism. Among the more than 200 proteins identified in this study are 21 proteins not previously described as part of the erythrocyte proteome. These results demonstrate the feasibility of applying a global proteomic approach to aid characterization of red cells from patients with hereditary anemia of unknown cause, including the identification of differentially expressed proteins as potential candidates with a role in disease pathogenesis. PMID:22509282

  4. A molecular defect in two families with hemolytic poikilocytic anemia: reduction of high affinity membrane binding sites for ankyrin.

    PubMed Central

    Agre, P; Orringer, E P; Chui, D H; Bennett, V

    1981-01-01

    Patients from two families with chronic hemolytic anemia have been studied. The erythrocytes are very fragile and appear microcytic with a great variety of shapes. Clinical evaluation failed to identify traditionally recognized causes of hemolysis. Sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE) showed no significant abnormality of the major polypeptide bands. Erythrocytes spectrin-ankyrin and ankyrin-membrane interactions were analyzed with 125I-labeled spectrin, 125I-labeled ankyrin, and inside-out vesicles. Patients' vesicles bound 125I-spectrin normally. Likewise, patients' spectrin and ankyrin competed normally for the binding sites on control membranes. None of the individual components appeared to have abnormal thermal sensitivity. Ankyrin-stripped, inside-out vesicles prepared from the patients bound less 125I-ankyrin than did vesicles prepared from normals (P less than 0.05 for all corresponding points in the high-affinity region). Scatchard analysis showed the most significant abnormality to be a 50% reduction in the high affinity ankyrin binding sites. Similar experiments were performed with blood from patients with spherocytosis and splenectomized controls, but no abnormalities were detected. The water soluble 43,000-dalton fragments of band 3 (the high-affinity ankyrin binding sites) were prepared from one of the patients and competed normally for 125I-ankyrin binding in solution. This suggests that the primary structural defect is a reduction in the number of high affinity membrane binding sites for ankyrin, and is consistent with an abnormal organization of band 3 in the membrane. Images PMID:6459341

  5. Anemias.

    PubMed

    Broadway-Duren, Jacqueline B; Klaassen, Hillary

    2013-12-01

    Anemias continue to present a challenge to the health care profession. Anemia is defined as a reduction in one or more of the RBC indices. Patients presenting with a mild form of anemia may be asymptomatic; however, in more serious cases the anemia can become life threatening. In many cases the clinical presentation also reflects the underlying cause. Anemia may be attributed to various causes, whereas autoimmune RBC destruction may be attributed to intrinsic and extrinsic factors. Laboratory tests are essential in facilitating early detection and differentiation of anemia. PMID:24267278

  6. Anemia

    MedlinePLUS

    ... don't get enough iron in their diets. Iron Deficiency Anemia Iron deficiency anemia is the most ... your body's iron stores. Back Continue Getting Enough Iron Some people feel sick if they take an ...

  7. Anemia

    MedlinePLUS

    ... 3 million Americans. Jump To: The Role of Red Blood Cells in Anemia Red blood cells carry hemoglobin, an iron-rich protein ... Anemia occurs when you do not have enough red blood cells or when your red blood cells ...

  8. Anemia

    MedlinePLUS

    ... system problems) Long-term (chronic) diseases such as chronic kidney disease, cancer, ulcerative colitis, or rheumatoid arthritis Some forms of anemia, such as thalassemia or sickle cell anemia, which ...

  9. Types of Hemolytic Anemia

    MedlinePLUS

    ... is, they destroy red blood cells) at warm temperatures, such as body temperature. In other types of AIHA, the body makes ... reactive antibodies. These antibodies are active at cold temperatures. Cold-reactive antibodies can become active when parts ...

  10. Immune hemolytic anemia

    MedlinePLUS

    ... be caused by: Complication of another disease Past blood transfusions Pregnancy (if the baby's blood type is different ... cyclophosphamide (Cytoxan), and rituximab (Rituxan) have been used. Blood transfusions are given with caution, because the blood may ...

  11. HEMOLYTIC ANEMIA Erythrocytes premature

    E-print Network

    LABORATORY FINDINGS · M:E ratio decreased · Increased Reticulocytes · Nucleated RBC in peripheral blood HEMOLYISIS · Within the Macrophages of the Spleen, liver, or BM · Causes · Inherited RBC Defects · Acquired Nocturnal Hemoglobinurea · Enzyme Disorders · Embden-Meyerhof Pathway Enzymes Deficiencies · Hexose

  12. Genetics Home Reference: Anemia

    MedlinePLUS

    ... on Genetics Home Reference: acute promyelocytic leukemia alpha thalassemia atypical hemolytic-uremic syndrome beta thalassemia Coats plus syndrome congenital dyserythropoietic anemia Diamond-Blackfan ...

  13. Anemia

    MedlinePLUS

    If you have anemia, your blood does not carry enough oxygen to the rest of your body. The most common cause of anemia is not having enough ... rich protein that gives the red color to blood. It carries oxygen from the lungs to the ...

  14. Severe hemolytic transfusion reaction due to anti-d in a d+ patient with sickle cell disease.

    PubMed

    Ipe, Tina S; Wilkes, Jennifer J; Hartung, Helge D; Westhoff, Connie M; Chou, Stella T; Friedman, David F

    2015-03-01

    A 5-year-old male with sickle cell disease presented with pain, dark urine, and fatigue 10 days after a red blood cell (RBC) transfusion. Laboratory evaluation demonstrated severe anemia, blood type O+, and anti-D in the serum. Anti-D in a D+ patient led to RH genotyping, which revealed homozygosity for RHD*DAU4 that encodes partial D antigen. Anti-D in this patient whose RBCs exclusively express partial D caused a delayed hemolytic transfusion reaction after exposure to D+ RBCs. The finding of anti-D in a D+patient should be investigated by molecular methods to help distinguish an alloantibody from an autoantibody. PMID:25171447

  15. Graves' Disease Causing Pancytopenia and Autoimmune Hemolytic Anemia at Different Time Intervals: A Case Report and a Review of the Literature

    PubMed Central

    Kumar, Geetika; Dewani, Shabana; Diedrich, William A.; Gupta, Ankur

    2013-01-01

    Graves' disease (GD) is associated with various hematologic abnormalities but pancytopenia and autoimmune hemolytic anemia (AIHA) are reported very rarely. Herein, we report a patient with GD who had both of these rare complications at different time intervals, along with a review of the related literature. The patient was a 70-year-old man who, during a hospitalization, was also noted to have pancytopenia and elevated thyroid hormone levels. Complete hematologic workup was unremarkable and his pancytopenia was attributed to hyperthyroidism. He was started on methimazole but unfortunately did not return for followup and stopped methimazole after a few weeks. A year later, he presented with fatigue and weight loss. Labs showed hyperthyroidism and isolated anemia (hemoglobin 7?g/dL). He had positive direct Coombs test and elevated reticulocyte index. He was diagnosed with AIHA and started on glucocorticoids. GD was confirmed with elevated levels of thyroid stimulating immunoglobulins and thyroid uptake and scan. He was treated with methimazole and radioactive iodine ablation. His hemoglobin improved to 10.7?g/dL at discharge without blood transfusion. Graves' disease should be considered in the differential diagnosis of hematologic abnormalities. These abnormalities in the setting of GD generally respond well to antithyroid treatment. PMID:24319463

  16. [Paroxysmal nocturnal hemoglobinuria and aplastic anemia].

    PubMed

    Thollot, F; Bordigoni, P; Olive, D

    1984-03-01

    The case of a 14 year-old adolescent girl presenting with paroxysmal nocturnal hemoglobinuria (PNH) associated with aplastic anemia is reported. This disease, rare in children, is characterized by an acquired hemolytic anemia, with abnormal sensitivity to complement: PNH actually affects the bone marrow stem cell. This explains its possible association with any type of malignant blood disease and with aplastic anemia. When aplastic anemia is the first sign of the disease, diagnosis is delayed, due to the possible negative response of the specific Ham's test. Therefore, the proper complications of PNH, especially thromboses, may be misappreciated and poorly managed. PMID:6742973

  17. Anemia

    MedlinePLUS

    ... transfusions, they are rarely used to treat anemia. Blood transfusion used to be the only treatment for severe ... with erythropoietin, or, in rare cases, with a blood transfusion. Back to Fact Sheet Categories The AIDS InfoNet ...

  18. Identification, Molecular Characterization, and Experimental Transmission of a New Hemoplasma Isolate from a Cat with Hemolytic Anemia in Switzerland

    Microsoft Academic Search

    Barbara Willi; Felicitas S. Boretti; Valentino Cattori; Severine Tasker; Marina L. Meli; Claudia Reusch; Hans Lutz; Regina Hofmann-Lehmann

    2005-01-01

    Recently, there has been a growing interest in hemotropic mycoplasmal species (also known as the hemo- plasmas), the causative agents of infectious anemia in several mammalian species. In felids, two different hemoplasma species have been recognized: Mycoplasma haemofelis (formerly Haemobartonella felis) and \\

  19. Mechanisms of the anemia in trypanosomiasis: studies on the role of the hemolytic fatty acids derived from Trypanosoma congolense.

    PubMed

    Tizard, I R; Holmes, W L; Nielsen, K

    1978-03-01

    Trypanosoma congolense releases a number of hemolytic free fatty acids on autolysis of which the most potent is linoleic acid. These fatty acids can lyse washed rat and bovine erythrocytes in vitro. Autolysed T. congoleuse will cause increased fragility of erythrocytes in whole rat blood but not in whole bovine blood. The lack of effect of these fatty acids on bovine erythrocytes tends to be confirmed by the failure of this material to influence the survival of these cells in vivo. Calculations based on the amount of free fatty acids required to cause hemolysis in vivo also confirm that this mechanism of erythrocyte destruction is probably unimportant in bovine trypanosomiasis. PMID:644654

  20. Aplastic anemia and red cell aplasia due to pentachlorophenol

    SciTech Connect

    Roberts, H.J.

    1983-01-01

    Repeated exposure to commercial (technical grade) pentachlorophenol (PCP) preceded aplastic anemia in four patients and pure red cell aplasia in two. Two patients developed concomitant or subsequent Hodgkin's disease and acute leukemia. The hematologic, mutagenic, and carcinogenic effect of PCP and its chemical contaminants have been documented in other clinical and experimental reports. In view of the widespread contamination of our environment by PCP, clinicians and public health investigators must seek out such exposure in these and related disorders and initiate measures to reduce it.

  1. Refractory autoimmune hemolytic anemia in a patient with DiGeorge syndrome treated successfully with plasma exchange: a case report and review of the literature.

    PubMed

    Damlaj, Moussab; Séguin, Chantal

    2014-11-01

    Warm antibody autoimmune hemolytic anemia (AIHA) results from targeted antibodies towards the red blood cells (RBCs) and can be either idiopathic or secondary to certain diseases, such as autoimmune disorders or malignancy, drugs, or infection. Patients with DiGeorge syndrome are particularly vulnerable to autoimmune conditions secondary to thymic hypoplasia and dysfunction of the immune system. First-line therapy for AIHA consists of corticosteroids, with most patients showing signs of response. Relapses are not uncommon and are treated with splenectomy or rituximab. There is a paucity of reports in the literature regarding treatment options beyond this stage. Herein, we describe an unusual case of a 20-year-old female affected by DiGeorge syndrome with a history of immune thrombocytopenia (ITP), who presented with life-threatening AIHA. Standard first- and second-line therapeutic modalities were ineffective in controlling her disease and she ultimately underwent plasma exchange therapy with successful resolution of hemolysis. At her last follow-up, one year after her initial presentation, she remains clinically well without signs of hemolysis. We conclude that in refractory cases of warm AIHA, plasma exchange therapy can be a valuable tool in the therapeutic armamentarium. PMID:25079091

  2. Autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation: analysis of 533 adult patients who underwent transplantation at King's College Hospital.

    PubMed

    Wang, Meng; Wang, Wenjia; Abeywardane, Ayesha; Adikarama, Malinthi; McLornan, Donal; Raj, Kavita; de Lavallade, Hugues; Devereux, Stephen; Mufti, Ghulam J; Pagliuca, Antonio; Potter, Victoria T; Mijovic, Aleksandar

    2015-01-01

    Autoimmune hemolytic anemia (AIHA) is a recognized complication of hematopoietic stem cell transplantation (HSCT); it is often refractory to treatment and carries a high mortality. To improve understanding of the incidence, risk factors, and clinical outcome of post-transplantation AIHA, we analyzed 533 patients who received allogeneic HSCT, and we identified 19 cases of AIHA after HSCT (overall incidence, 3.6%). The median time to onset, from HSCT to AIHA, was 202 days. AIHA was associated with HSCT from unrelated donors (hazard ratio [HR], 5.28; 95% confidence interval [CI], 1.22 to 22.9; P = .026). In the majority (14 of 19; 74%) of AIHA patients, multiple agents for treatment were required, with only 9 of 19 (47%) patients achieving complete resolution of AIHA. Patients with post-transplantation AIHA had a higher overall mortality (HR, 2.48; 95% CI, 1.33 to 4.63; P = .004), with 36% (4 of 11 cases) of deaths attributable to AIHA. PMID:25262883

  3. Hemopoietic effect of extracts from constituent herbal medicines of Samul-tang on phenylhydrazine-induced hemolytic anemia in rats

    PubMed Central

    Lee, Hye Won; Kim, Hyojun; Ryuk, Jin Ah; Kil, Ki-Jung; Ko, Byoung Seob

    2014-01-01

    Samul-tang (Si-Wu-Tang, SMT), a kind of herbal medicines, has been used for the hemato-deficient disease for hundreds of years. In this work, investigate the anti-anemia activity of the H2O extracts from constituent herbal medicines of Samul-tang in an anemia model induced by intravenous infection of phenylhydrazine-HCL (PHZ) at 10 mg/kg for 4 days. After PHZ injection, female Sparague-Dawley rats were administrated extracts from constituent herbal medicines of SMT (300 mg/kg/day, p.o.) daily for 1 week. Results showed that sever hemolysis was induced by PHZ. For Paeonia lactiflora (PL2) H2O extract treated groups, the concentration of hemoglobin, hematocrit and red blood cells number increased much more significantly than PHZ-treated group. Moreover, Angelica gigas (AG), Angelica. acutiloba (AA), Paeonia lactiflora (PL2) and Rehmannia glutinosa (RG) extract administration significantly improved serum erythropoietin concentration. The activity of aminolevulinic acid dehydrates (ALDL) in liver homegenate was increased in Angelica gigas(AA), Paeonia lactiflora (PL2) and Rehmannia glutinosa (RG) treated group. PMID:25337267

  4. Iron-Deficiency Anemia Leading to Transient Ischemic Attacks due to Intraluminal Carotid Artery Thrombus.

    PubMed

    Batur Caglayan, H Z; Nazliel, B; Irkec, C; Dumlu, A; Filiz, A; Panpalli Ates, M

    2013-01-01

    Reactive thrombocytosis secondary to iron-deficiency anemia (IDA) is a rare but recognized cause of stroke. We report the case of a patient with iron-deficiency anemia presenting with multiple transient ischemic attacks (TIA) due to intraluminal thrombus of an internal carotid artery. The putative mechanisms underlying anemia and stroke syndromes are not completely understood, and it is believed that iron deficiency may cause ischemic stroke by several potential mechanisms. Thrombocytosis is often associated with iron deficiency, and microcytosis produces a reduction in the red cell deformability and could produce a hypercoagulable state. The platelet count and function observed in iron-deficiency anemia could act synergistically to promote thrombus formation, especially in the setting of an underlying atherosclerotic disease. The presence of floating thrombus in a patient with clinical and MRI evidence of stroke represents a significant therapeutic dilemma and requires immediate decision about treatment. PMID:24109530

  5. Iron-Deficiency Anemia Leading to Transient Ischemic Attacks due to Intraluminal Carotid Artery Thrombus

    PubMed Central

    Batur Caglayan, H. Z.; Nazliel, B.; Irkec, C.; Dumlu, A.; Filiz, A.; Panpalli Ates, M.

    2013-01-01

    Reactive thrombocytosis secondary to iron-deficiency anemia (IDA) is a rare but recognized cause of stroke. We report the case of a patient with iron-deficiency anemia presenting with multiple transient ischemic attacks (TIA) due to intraluminal thrombus of an internal carotid artery. The putative mechanisms underlying anemia and stroke syndromes are not completely understood, and it is believed that iron deficiency may cause ischemic stroke by several potential mechanisms. Thrombocytosis is often associated with iron deficiency, and microcytosis produces a reduction in the red cell deformability and could produce a hypercoagulable state. The platelet count and function observed in iron-deficiency anemia could act synergistically to promote thrombus formation, especially in the setting of an underlying atherosclerotic disease. The presence of floating thrombus in a patient with clinical and MRI evidence of stroke represents a significant therapeutic dilemma and requires immediate decision about treatment. PMID:24109530

  6. [Anemia induced by cadmium intoxication].

    PubMed

    Horiguchi, Hyogo

    2007-05-01

    Anemia is commonly induced by chronic cadmium (Cd) intoxication. Three main factors are involved in the development of Cd-induced anemia: hemolytic, iron-deficiency, and renal. Intravascular hemolysis can occur at the early stage of Cd exposure owing to the direct damaging effect on erythrocytes. In addition, Cd that accumulates in erythrocytes affects membrane cytoskeletons and decreases cell deformability, and these cells are then trapped and destroyed in the spleen. Iron deficiency can be detected in animals after an oral exposure to Cd, which competes with iron for absorption in the intestines, leading to anemia. However, an increase in body iron content along with anemia is often observed in cases of parenteral exposure or itai-itai disease. Therefore, it is estimated that Cd disrupts the efficient usage of iron in hemoglobin synthesis in the body. Renal anemia is observed during the very last phase of chronic, severe Cd intoxication, such as itai-itai disease, showing a decrease in the production of erythropoietin from renal tubular cells. Because the renal anemia is based on the same pathophysiology as Cd-induced osteomalacia, which is derived from the disturbance of mineral metabolism due to renal tubular dysfunction, it is reasonable to include renal anemia in the criteria for the diagnosis of itai-itai disease. Hemodilution could also contribute to the development of Cd-induced anemia. Bone marrow hypoplasia or the inhibition of heme synthesis might only be involved in Cd-induced anemia in severe cases of Cd intoxication. PMID:17575787

  7. Atypical Hemolytic Uremic Syndrome

    PubMed Central

    Kavanagh, David; Goodship, Tim H.; Richards, Anna

    2013-01-01

    Summary Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is reported, suggesting that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The underlying genetic defect predicts the prognosis both in native kidneys and after renal transplantation. The successful trials of the complement inhibitor eculizumab in the treatment of atypical HUS will revolutionize disease management. PMID:24161037

  8. Y O U R G U I D E T O Iron-Deficiency Anemia

    E-print Network

    Bandettini, Peter A.

    Y O U R G U I D E T O Anemia Iron-Deficiency Anemia Pernicious Anemia Aplastic Anemia Hemolytic Anema Anemia Healthy Lifestyle Changes Prevent Treat Control #12;#12;Y O U R G U I D E T O Anemia AnemiaHealthy Lifestyle Changes Prevent Treat Control Iron-Deficiency Anemia Pernicious Anemia Aplastic

  9. What Are the Signs and Symptoms of Sickle Cell Anemia?

    MedlinePLUS

    ... TRIALS LINKS Quiz Related Topics Anemia Blood Transfusion Hemolytic Anemia Pulmonary Hypertension Stroke Send a link to NHLBI ... a life-threatening condition linked to sickle cell anemia. This syndrome is similar ... and abnormal chest x ray results. Pulmonary Hypertension ...

  10. Atypical hemolytic uremic syndrome

    PubMed Central

    2011-01-01

    Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early. PMID:21902819

  11. Bendamustine and rituximab combination in the management of chronic lymphocytic leukemia-associated autoimmune hemolytic anemia: A multicentric retrospective study of the French CLL intergroup (GCFLLC/MW and GOELAMS).

    PubMed

    Quinquenel, Anne; Willekens, Christophe; Dupuis, Jehan; Royer, Bruno; Ysebaert, Loic; Guibert, S De; Michallet, Anne-Sophie; Feugier, Pierre; Guieze, Romain; Levy, Vincent; Delmer, Alain

    2015-03-01

    We report our experience on bendamustine and rituximab (BR) combination in 26 patients with chronic lymphocytic leukemia (CLL) complicated by autoimmune hemolytic anemia (AIHA). At the time of BR initiation, 88% of the patients had already been treated for AIHA and CLL was progressive regardless of AIHA in all patients but one. Overall response rates were 81% for AIHA and 77% for CLL. Median time to next treatment was 28.3 months and 26.2 months for AIHA and CLL, respectively. BR therapy may represent a good and safe therapeutic option in this setting where adequate control of CLL seems important for long-term AIHA response. Am. J. Hematol. 90:204-207, 2015. © 2014 Wiley Periodicals, Inc. PMID:25428829

  12. Hemolytic disease of fetus and newborn due to maternal red blood cell alloantibodies in the Malay population

    PubMed Central

    Hassan, Mohd Nazri; Mohd Noor, Noor Haslina; Johan Noor, Shah Reza; Sukri, Salamah Ahmad; Mustafa, Rapiaah; Luc Aster, Hans Van Rostenberghe

    2014-01-01

    Background: Maternal red blood cell (RBC) alloimmunization may lead to production of harmful antibodies that result in hemolytic disease of fetus and newborn (HDFN). There is insufficient data on the prevalence of HDFN due to RBC alloantibodies in the Malay neonatal population. Aim: The aim of this study was to determine the incidence of HDFN in the Malay neonatal population due to clinically significant RBC alloantibodies. Subjects and Methods: A cross sectional study was conducted in Transfusion Medicine Unit, Hospital Universitiy Sains Malaysia over one year period from January to December 2009. A total of 5163 Malay pregnant women who attended labor room for delivery were collected and analyzed prospectively. The blood samples were subjected to the standard immunohematological procedure for RBC antibody screening and identification using reagents of Diamed-ID Gel microtyping system. All the newborns with RBC alloantibody were investigated for the evidence of HDFN. Results: Thirty (0.58%) women were found to have clinically significant RBC alloantibodies. Most of the alloantibodies belonged to Rhesus (Rh) system (56.7%) where anti-E (33.3%) was the most common followed by anti-D (10.0%). Rh antibodies were the main cause of HDFN in fourteen (0.27%) neonates. Anti-D and anti-c were identified to cause moderate to very severe HDFN. Conclusions: With the low prevalence of clinically significant RBC alloantibodies and HDFN, routine antenatal antibody screening practice may not be advised as a routine practice at present, preferably reserved for those women of RhD negative or with history of HDFN, significantly of those attributed to anti-c. PMID:25161351

  13. Coexistence of symptomatic iron-deficiency anemia and duodenal nodular lymphoid hyperplasia due to giardiasis: case report.

    PubMed

    Kasirga, Erhun; Gülen, Hüseyin; Sim?ek, Ay?e; Ayhan, Semin; Yilmaz, Ozge; Ellidokuz, Ender

    2009-01-01

    Iron-deficiency anemia due to iron malabsorption and duodenal nodular lymphoid hyperplasia (NLH) has been described in children with Giardia intestinalis infection. Also, symptomatic iron-deficiency anemia is rarely encountered in male adolescents. A 14-year-old boy underwent esophagogastroduodenoscopy for investigation of symptomatic iron-deficiency anemia (hemoglobin 5.8 g/dL, mean corpuscular volume 65.3 fL, serum ferritin < 1.5 ng/mL). He had a sufficient diet for iron and recurrent bouts of diarrhea without melena. At upper endoscopy, duodenal mucosa was diffusely nodular. Histopathologic evaluation of biopsy samples from the duodenum revealed infection with Giardia intestinalis. His anemia improved with metronidazole and iron treatment. PMID:19206009

  14. Laboratory Evaluation of Anemia

    PubMed Central

    Wallerstein, Ralph O.

    1987-01-01

    The laboratory evaluation of anemia begins with a complete blood count and reticulocyte count. The anemia is then categorized as microcytic, macrocytic or normocytic, with or without reticulocytosis. Examination of the peripheral smear and a small number of specific tests confirm the diagnosis. The serum iron level, total iron-binding capacity, serum ferritin level and hemoglobin electrophoresis generally separate the microcytic anemias. The erythrocyte size-distribution width may be particularly helpful in distinguishing iron deficiency from thalassemia minor. Significant changes have occurred in the laboratory evaluation of macrocytic anemia, and a new syndrome of nitrous oxide-induced megaloblastosis and neurologic dysfunction has been recognized. A suggested approach to the hemolytic anemias includes using the micro-Coombs' test and ektacytometry. Finally, a number of causes have been identified for normocytic anemia without reticulocytosis, including normocytic megaloblastic anemia and the acquired immunodeficiency syndrome. PMID:3577135

  15. Genetics Home Reference: Atypical hemolytic-uremic syndrome

    MedlinePLUS

    ... is caused by infection with certain strains of Escherichia coli bacteria that produce toxic substances called Shiga-like ... clotting ; cell ; chronic ; clotting ; end-stage renal disease ; Escherichia coli ; ESRD ; familial ; gene ; hemolysis ; hemolytic anemia ; idiopathic ; immune ...

  16. Autoimmune hemolytic anemia and immune thrombocytopenia as unusual presentations of childhood Hodgkin lymphoma: a case report and review of the literature.

    PubMed

    Cecinati, Valerio; Brugnoletti, Fulvia; D'Angiò, Mariella; De Nicolò, Maria Chiara; De Vellis, Annalisa; Coluzzi, Serelina; Testi, Anna Maria

    2012-05-01

    We discuss an unusual clinical presentation of childhood Hodgkin lymphoma; a 16-year-old girl was referred for Coombs-positive severe anemia, thrombocytopenia, and asymptomatic anterior mediastinal mass. Bone marrow examination showed no evidence of neoplastic disease. Biopsy of the mass was possible only after administration of both intravenous immunoglobulins and steroids resulting in a prompt rise of the platelet count and partial hemoglobin level stabilization. The identification of this clinical picture as a possible complication of an underlying Hodgkin lymphoma presents difficulties in diagnosis and management of the primary condition. PMID:22258348

  17. Human parvovirus B19-induced aplastic crisis in iron deficiency anemia.

    PubMed

    Kudoh, T; Yoto, Y; Suzuki, N; Oda, T; Katoh, S; Chiba, S; Matsunaga, Y

    1994-08-01

    Human parvovirus B19 (HPVB19) infects and replicates in erythroid progenitor cells. Its specific cytotoxic effect on these cells results in aplastic crises in patients with congenital hemolytic anemias. Aplastic crisis due to HPVB19 infection in a healthy girl revealed occult iron deficiency anemia. The condition is characterized by a high serum iron level in the aplastic phase and rapid recovery after administration of iron. Temporary HPVB19-induced red blood cell aplasia could occur in patients with other anemias, particularly those with non-inherited form of hemolysis. PMID:7942015

  18. Evaluation of stem cell reserve using serial bone marrow transplantation and competitive repopulation in a murine model of chronic hemolytic anemia

    SciTech Connect

    Maggio-Price, L.; Wolf, N.S.; Priestley, G.V.; Pietrzyk, M.E.; Bernstein, S.E.

    1988-09-01

    Serial transplantation and competitive repopulation were used to evaluate any loss of self-replicative capacity of bone marrow stem cells in a mouse model with increased and persistent hemopoietic demands. Congenic marrows from old control and from young and old mice with hereditary spherocytic anemia (sphha/sphha) were serially transplanted at 35-day intervals into normal irradiated recipients. Old anemic marrow failed or reverted to recipient karyotype at a mean of 3.5 transplants, and young anemic marrow reverted at a mean of 4.0 transplants, whereas controls did so at a mean of 5.0 transplants. In a competitive assay in which a mixture of anemic and control marrow was transplanted, the anemic marrow persisted to 10 months following transplantation; anemic marrow repopulation was greater if anemic marrow sex matched with the host. It is possible that lifelong stress of severe anemia decreases stem cell reserve in the anemic sphha/sphha mouse marrow. However, marginal differences in serial transplantation number and the maintenance of anemic marrow in a competition assay would suggest that marrow stem cells, under prolonged stress, are capable of exhibiting good repopulating and self-replicating abilities.

  19. Gemcitabine induced hemolytic uremic syndrome

    PubMed Central

    Sadjadi, Seyed-Ali; Annamaraju, Pavan

    2012-01-01

    Summary Background: Gemcitabine is frequently used for the treatment of many cancers. Not infrequently it leads to development of hemolytic uremic syndrome, presenting with hemolytic anemia, acute kidney injury and occasionally peripheral edema, livedo reticularis and digital necrosis. Case Report: A 78 year old man with non-small cell lung cancer developed uremic syndrome following treatment with multiple chemotherapy agents including gemcitabine. He was treated aggressively with hemodialysis and plasmapheresis. Initially he responded but upon attempts at decreasing the frequency of plasmapheresis, lactate dehydrogenase increased and platelet count decreased, indicating continuing hemolysis. Hemolysis responded to splenectomy but he continued to require hemodialysis treatment. Conclusions: Although many cases of gemcitabine induced HUS have been reported, its cause and pathogenesis remain unclear and it should be used with caution. Frequent monitoring of renal function and close observation of the patient are essential. PMID:23569497

  20. Venous stroke and status epilepticus due to milk-induced anemia in a child.

    PubMed

    Finkel, Leslie; Piantino, Juan; Goldstein, Joshua; Wainwright, Mark S

    2015-02-01

    The risk factors for cerebral sinus venous thrombosis include dehydration, infection, and anemia. The clinical presentation in children of venous strokes associated with cerebral venous thrombosis is variable and may include seizures. Acute management should focus on the treatment of the primary cause and anticoagulation or antiplatelet therapy if needed. Early recognition and targeted treatment is important because survivors are at increased risk for long-term neurologic complications. We report a case of a 4-year-old girl who presented with status epilepticus and was subsequently found to have a cerebral venous sinus thrombosis in the transverse and sigmoid sinus, with venous infarction in the temporal lobe. Laboratory results were significant for a microcytic anemia caused by excessive milk intake. Although iron deficiency anemia is a common pediatric disorder, this uncommon presentation demonstrates the potential for neurologic complications secondary to anemia, as well as the need for a high index of suspicion in order to identify venous stroke as a cause in children who present to the emergency department with seizures. PMID:25513978

  1. Inborn anemias in mice

    SciTech Connect

    Bernstein, S.E.; Barker, J.E.; Russell, E.S.

    1981-06-01

    hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, five hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus our wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values, (b) determinations of radiosensitivity under a variety of conditions, (c) measurements of iron metabolism and heme synthesis, (d) histological and biochemical study of blood-forming tissue, (e) functional tests of the stem cell component, (f) examination of responses to erythroid stimuli, and (g) transplantation of tissue between individuals of differently affected genotypes.

  2. Inborn anemias in mice: (Annual report, 1981-1982)

    SciTech Connect

    Bernstein, S.E.

    1982-07-19

    Hereditary anemias of mice are the chief objects of investigation, specificially four macrocytic anemias, 3 types of hemolytic anemia, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, the autoimmune hemolytic anemia of NZB mice, an ..cap alpha..-thalassemia and a new hypochromic anemia with hemochromatosis. New types of anemia may be analyzed as new mutations appear. Three new mutations have been identified during the past 18 months. These anemias are studied through characterization of peripheral blood values, determinations of radiosensitivity under a variety of conditions, measurements of iron metabolism and heme synthesis, study of normal and abnormal erythrocyte membrane proteins, histological and biochemical characterization of blood-forming tissue, functional tests of the stem-cell component, examination of responses to erythroid stimuli, and transplantation of tissue and parabiosis between individuals of differently affected genotypes. 31 refs.

  3. Anemia and performance status as prognostic markers in acute hypercapnic respiratory failure due to chronic obstructive pulmonary disease

    PubMed Central

    Mydin, Helmy Haja; Murphy, Stephen; Clague, Howell; Sridharan, Kishore; Taylor, Ian K

    2013-01-01

    Background In patients with acute hypercapnic respiratory failure (AHRF) during exacerbations of COPD, mortality can be high despite noninvasive ventilation (NIV). For some, AHRF is terminal and NIV is inappropriate. However there is no definitive method of identifying patients who are unlikely to survive. The aim of this study was to identify factors associated with inpatient mortality from AHRF with respiratory acidosis due to COPD. Methods COPD patients presenting with AHRF and who were treated with NIV were studied prospectively. The forced expiratory volume in 1 second (FEV1), World Health Organization performance status (WHO-PS), clinical observations, a composite physiological score (Early Warning Score), routine hematology and biochemistry, and arterial blood gases prior to commencing NIV, were recorded. Results In total, 65 patients were included for study, 29 males and 36 females, with a mean age of 71 ± 10.5 years. Inpatient mortality in the group was 33.8%. Mortality at 30 days and 12 months after admission were 38.5% and 58.5%, respectively. On univariate analysis, the variables associated with inpatient death were: WHO-PS ? 3, long-term oxygen therapy, anemia, diastolic blood pressure < 70 mmHg, Early Warning Score ? 3, severe acidosis (pH < 7.20), and serum albumin < 35 g/L. On multivariate analysis, only anemia and WHO-PS ? 3 were significant. The presence of both predicted 68% of inpatient deaths, with a specificity of 98%. Conclusion WHO-PS ? 3 and anemia are prognostic factors in AHRF with respiratory acidosis due to COPD. A combination of the two provides a simple method of identifying patients unlikely to benefit from NIV. PMID:23658480

  4. Atypical hemolytic uremic syndrome in the Tunisian population

    Microsoft Academic Search

    Nadia LebanSabra; Sabra Aloui; Dalel Touati; Ramzy Lakhdhar; Habib Skhiri; Gerard Lefranc; Abdellatif Achour; Mezri Elmay; Margarita Lopez-Trascasa; Pilar Sanchez-Corral; Jemni Chibani; Amel Haj Khelil

    2011-01-01

    Background  Hemolytic uremic syndrome consists of a triad of acquired hemolytic anemia, thrombocytopenia and renal failure.\\u000a \\u000a \\u000a \\u000a Aim  Our objectives were to determine epidemiology, clinical and laboratory characteristics of patients with atypical hemolytic\\u000a uremic syndrome (aHUS) to determine the relationship between the complement protein deficit and aHUS in the Tunisian population.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  We studied retrospectively four cases of atypical HUS in adults admitted in

  5. Optimal management of iron deficiency anemia due to poor dietary intake

    PubMed Central

    Aspuru, Kattalin; Villa, Carlos; Bermejo, Fernando; Herrero, Pilar; López, Santiago García

    2011-01-01

    Iron is necessary for the normal development of multiple vital processes. Iron deficiency (ID) may be caused by several diseases, even by physiological situations that increase requirements for this mineral. One of its possible causes is a poor dietary iron intake, which is infrequent in developed countries, but quite common in developing areas. In these countries, dietary ID is highly prevalent and comprises a real public health problem and a challenge for health authorities. ID, with or without anemia, can cause important symptoms that are not only physical, but can also include a decreased intellectual performance. All this, together with a high prevalence, can even have negative implications for a community’s economic and social development. Treatment consists of iron supplements. Prevention of ID obviously lies in increasing the dietary intake of iron, which can be difficult in developing countries. In these regions, foods with greater iron content are scarce, and attempts are made to compensate this by fortifying staple foods with iron. The effectiveness of this strategy is endorsed by multiple studies. On the other hand, in developed countries, ID with or without anemia is nearly always associated with diseases that trigger a negative balance between iron absorption and loss. Its management will be based on the treatment of underlying diseases, as well as on oral iron supplements, although these latter are limited by their tolerance and low potency, which on occasions may compel a change to intravenous administration. Iron deficiency has a series of peculiarities in pediatric patients, in the elderly, in pregnant women, and in patients with dietary restrictions, such as celiac disease. PMID:22114518

  6. [Autoimmune hemolytic anemia and myelodysplastic syndromes].

    PubMed

    Martín Vega, C; Vallespí, T; Juliá, A; Zuazu, J; Torrabadella, M

    1989-10-01

    The association between autoimmune haemolytic anaemia (AHA) and myelodysplastic syndromes (MDS) was found in seven out of 156 patients with SMD who received several transfusions as supportive therapy. Three patients were diagnosed of refractory anaemia (RA), three more of refractory anaemia with excess of blasts (RAEB) and one of refractory anaemia with ring sideroblasts (RARS). All patients showed a positive direct antiglobulin test (DAT) and the presence of anti-red blood cell IgG type antibodies, both in serum and eluate. Clinically, three patients showed signs of low grade haemolysis. It is suggested that in the reported patients, who seem to be immunologically predisposed, red blood cell transfusions could trigger the autoantibodies and the AHA development. PMID:2617384

  7. Intravenous Imferon masquerading as an acute hemolytic transfusion reaction.

    PubMed

    Colburn, W J; Barnes, A

    1982-01-01

    The case of a 74-year-old woman with a history of chronic iron deficiency anemia requiring transfusion is reported. Shortly after receiving intravenous iron-dextran, the patient was transfused with two units of crossmatch compatible packed red blood cells and subsequently experienced severe racking chills associated with mild elevation of temperature. In the evaluation of this febrile reaction, her serum exhibited a distinct red-brown discoloration which was interpreted as free hemoglobin. Laboratory studies performed to evaluate the possibility of acute intravascular hemolysis were all within normal limits. Subsequent investigation revealed that the color of the recipients serum was due to iron-dextran. Caution is urged in the evaluation of patients for hemolytic transfusion reactions who have been administered intravenous iron-dextran, since the drug imparts a red-brown tinge to the plasma which may be misinterpreted as free hemoglobin. PMID:7071921

  8. Hemolytic Uremic Syndrome: New Developments in Pathogenesis and Treatment

    PubMed Central

    Boyer, Olivia; Niaudet, Patrick

    2011-01-01

    Hemolytic uremic syndrome is defined by the characteristic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In children, most cases of HUS are caused by Shiga-toxin-producing bacteria, especially Escherichia coli O157:H7. Common vehicles of transmission include ground beef, unpasteurized milk, and municipal or swimming water. Shiga-toxin-associated HUS is a main cause of acute renal failure in young children. Management remains supportive as there is at present no specific therapy to ameliorate the prognosis. Immediate outcome is most often favourable but long-term renal sequelae are frequent due to nephron loss. Atypical HUS represents 5% of cases. In the past 15 years, mutations in complement regulators of the alternative pathway have been identified in almost 60% of cases, leading to excessive complement activation. The disease has a relapsing course and more than half of the patients either die or progress to end-stage renal failure. Recurrence after renal transplantation is frequent. PMID:21876803

  9. Successful treatment with rituximab and mycophenolate mofetil of refractory autoimmune hemolytic anemia post-hematopoietic stem cell transplant for dyskeratosis congenita due to TINF2 mutation.

    PubMed

    O'Connell, Niall; Goodyer, Matthew; Gleeson, Mary; Storey, Lorna; Williams, Martina; Cotter, Melanie; O'Marcaigh, Aengus; Smith, Owen

    2014-02-01

    AIHA following allogeneic HSCT is appearing more frequently in the literature. It occurs as a result of donor cell-derived antibodies targeting donor red cell antigens. Little guidance exists on the management of such patients, particularly in the pediatric setting. First-line conventional treatment is corticosteroids and/or immunoglobulin therapy with monoclonal antibody therapy reserved for treatment failure. We report our experience of a child refractory to immunoglobulin and steroid therapy who required several infusions of rituximab and immunomodulatory therapy to obtain a clinically significant response. PMID:24168326

  10. Increased Serum CA15.3 Levels in Patients with Megaloblastic Anemia due to Vitamin B12 Deficiency

    Microsoft Academic Search

    Argiris Symeonidis; Alexandra Kouraklis-Symeonidis; Dimitris Apostolopoulos; Evangelia Arvanitopoulou; Nikolaos Giannakoulas; Pavlos Vassilakos; Nicholas Zoumbos

    2004-01-01

    Objectives: To estimate the usefulness of serum tumor markers’ monitoring, as predictors of gastric cancer in patients with pernicious anemia. Patients and Methods: We investigated serum levels of carcinoembryonic antigen (CEA), ?-fetal protein, cancer antigen (CA)-19.9, CA-125 and CA-15.3 in 50 patients with pernicious anemia and in 24 healthy controls, matched for age and sex. In 38 patients, the evaluation

  11. Reticulocytopenic, coombs' positive anemia induced by procainamide.

    PubMed

    Schifman, R B; Garewal, H; Shillington, D

    1983-07-01

    A case of Coombs' positive anemia in a man who had procainamide-induced lupus erythematosus syndrome is reported. The patient had a hemoglobin of 4.3 gm/dl and reticulocytopenia (3.1% corrected). Serum lactate dehydrogenase and haptoglobin levels were normal, and total bilirubin was only slightly elevated. Two other reported cases of procainamide-induced hemolytic anemia have demonstrated similar findings. Apparently, procainamide occasionally may induce a reversible, reticulocytopenic, Coombs' positive anemia that is not associated with laboratory evidence of acute hemolysis. PMID:6858967

  12. Atypical Hemolytic Uremic Syndrome: Update on the Complement System and What Is New

    Microsoft Academic Search

    Patricia Hirt-Minkowski; Michael Dickenmann; Jürg A. Schifferli

    2010-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disease of microangiopathic hemolytic anemia, thrombocytopenia, and predominant renal impairment. It is characterized by the absence of Shiga toxin-producing bacteria as a triggering factor. During the last decade, aHUS has been demonstrated to be a disorder of the complement alternative pathway dysregulation, as there is a growing list of mutations and polymorphisms

  13. Analysis of a Viridans Group Strain Reveals a Case of Bacteremia Due to Lancefield Group G Alpha-Hemolytic Streptococcus dysgalactiae subsp. equisimilis in a Patient with Pyomyositis and Reactive Arthritis

    Microsoft Academic Search

    Patrick C. Y. Woo; Jade L. L. Teng; Susanna K. P. Lau; Peggy N. L. Lum; Kit-Wah Leung; Kee-Lam Wong; Kin-Wah Li; Kui-Chun Lam; Kwok-Yung Yuen

    Streptococcus dysgalactiae is classified by a combination of phenotypic and genotypic characteristics into Lancefield group C alpha-hemolytic Streptococcus dysgalactiae subsp. dysgalactiae and Lancefield group C, group G, and group L beta-hemolytic Streptococcus dysgalactiae subsp. equisimilis. In this study, we report the isolation of a catalase-negative, alpha-hemolytic, optochin- and bacitracin-resistant viridans group strain, which does not grow in 10 or 40%

  14. Eculizumab Therapy Leads to Rapid Resolution of Thrombocytopenia in Atypical Hemolytic Uremic Syndrome

    PubMed Central

    Kuo, Elizabeth

    2014-01-01

    Eculizumab is highly effective in controlling complement activation in patients with the atypical hemolytic uremic syndrome (aHUS). However, the course of responses to the treatment is not well understood. We reviewed the responses to eculizumab therapy for aHUS. The results show that, in patients with aHUS, eculizumab therapy, when not accompanied with concurrent plasma exchange therapy, led to steady increase in the platelet count and improvement in extra-renal complications within 3 days. By day 7, the platelet count was normal in 15 of 17 cases. The resolution of hemolytic anemia and improvement in renal function were less predictable and were not apparent for weeks to months in two patients. The swift response in the platelet counts was only observed in one of five cases who received concurrent plasma exchange therapy and was not observed in a case of TMA due to gemcitabine/carboplatin. In summary, eculizumab leads to rapid increase in the platelet counts and resolution of extrarenal symptoms in patients with aHUS. Concurrent plasma exchange greatly impedes the response of aHUS to eculizumab therapy. Eculizumab is ineffective for gemcitabine/carboplatin associated TMA. PMID:25400666

  15. Gemcitabine-Induced Hemolytic Uremic Syndrome in Pancreatic Cancer: A Case Report and Review of the Literature

    PubMed Central

    Lee, Hye Won; Kang, Huapyong; Choi, Heun; Choi, Youn Jeong; Lee, Kyung Joo; Lee, Seung Woo; Han, Seung Hyuk; Kim, Jin Seok; Song, Si Young

    2014-01-01

    Hemolytic uremic syndrome (HUS) is a rare thrombotic complication characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. HUS may be caused by several different conditions, including infection, malignancy, and chemotherapeutic agents, such as mitomycin, cisplatin, and most recently, gemcitabine. The outcome of gemcitabine-induced HUS is poor, and the disease has a high mortality rate. This study reports a case of gemcitabine-induced HUS in a patient with pancreatic cancer in Korea. PMID:24516709

  16. Comparison of two recombinant erythropoietin formulations in patients with anemia due to end-stage renal disease on hemodialysis: A parallel, randomized, double blind study

    PubMed Central

    Pérez-Oliva, Jorge F; Casanova-González, Martha; García-García, Idrian; Porrero-Martín, Pedro J; Valenzuela-Silva, Carmen M; Hernández-Montero, Tairí; Lagarde-Ampudia, Marcia; Casanova-Kutsareva, Yuri; Ávila-Albuerne, Yisel; Vargas-Batista, Alicia; Bobillo-López, Hailen; Herrera-Valdés, Raúl; López-Saura, Pedro A

    2005-01-01

    Background Recombinant human erythropoietin (EPO) is used for the treatment of last stage renal anemia. A new EPO preparation was obtained in Cuba in order to make this treatment fully nationally available. The aim of this study was to compare the pharmacokinetic, pharmacodynamic and safety properties of two recombinant EPO formulations in patients with anemia due to end-stage renal disease on hemodialysis. Methods A parallel, randomized, double blind study was performed. A single 100 IU/Kg EPO dose was administered subcutaneously. Heberitro (Heber Biotec, Havana, formulation A), a newly developed product and Eprex (CILAG AG, Switzerland, formulation B), as reference treatment were compared. Thirty-four patients with anemia due to end-stage renal disease on hemodialysis were included. Patients had not received EPO previously. Serum EPO level was measured by enzyme immunoassay (EIA) during 120 hours after administration. Clinical and laboratory variables were determined as pharmacodynamic and safety criteria until 216 hours. Results Both groups of patients were similar regarding all demographic and baseline characteristics. EPO kinetics profiles were similar for both formulations; the pharmacokinetic parameters were very close (i.e., AUC: 4667 vs. 4918 mIU.h/mL; Cmax: 119.1 vs. 119.7 mIU/mL; Tmax: 13.9 vs. 18.1 h; half-life, 20.0 vs. 22.5 h for formulations A and B, respectively). The 90% confidence intervals for the ratio between both products regarding these metrics were close to the 0.8 – 1.25 range, considered necessary for bioequivalence. Differences did not reach 20% in any case and were not determined by a formulation effect, but probably by a patients' variability effect. Concerning pharmacodynamic features, a high similitude in reticulocyte counts increments until 216 hours and the percentage decrease in serum iron until 120 hours was observed. There were no differences between formulations regarding the adverse events and their intensity. The more frequent events were pain at injection site (35.3%) and hypertension (29%). Additionally, further treatment of the patients with the study product yielded satisfactory increases in hemoglobin and hematocrit values. Conclusion The formulations are comparable. The newly developed product should be acceptable for long-term application. PMID:15910687

  17. Method for analysis of nanoparticle hemolytic properties in vitro.

    PubMed

    Dobrovolskaia, Marina A; Clogston, Jeffrey D; Neun, Barry W; Hall, Jennifer B; Patri, Anil K; McNeil, Scott E

    2008-08-01

    Hemolysis (destruction of red blood cells) in vivo can lead to anemia, jaundice, and other pathological conditions; therefore the hemolytic potential of all intravenously administered pharmaceuticals must be evaluated. Nanotechnology-derived devices and drug carriers are emerging as alternatives to conventional small-molecule drugs, and in vitro evaluation of their biocompatibility with blood components is a necessary part of early preclinical development. The small size and unique physicochemical properties of nanoparticles may cause their interactions with erythrocytes to differ from those observed for conventional pharmaceuticals and may also cause interference with standardized in vitro tests. Separating true hemolytic responses from the false-positive or false-negative results caused by particle interference is important for correct interpretation of these tests. Here we describe validation of an in vitro assay for the analysis of nanoparticle hemolytic properties and discuss observed nanointerferences with the assay. We propose alternative methods to avoid misleading results from nanoparticles and discuss the potential relevance of nanoparticle in vitro hemolytic properties to in vivo systems. PMID:18605701

  18. Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome.

    PubMed

    Polito, Maria Goretti; Kirsztajn, Gianna Mastroianni

    2010-01-01

    Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic- uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Other disorders occasionally present with similar manifestations. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different disorders have been described: HUS and TTP. Injury to the endothelial cell is the central and likely inciting factor in the sequence of events leading to TMA. Loss of physiological thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, abnormal von Willebrand factor release and fragmentation, and increased vascular shear stress may then sustain and amplify the microangiopathic process. Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the disease that may play a paramount role in particular in familial and recurrent forms. In the case of diarrhea-associated HUS (D+HUS), renal endothelial damage is mediated (at least in large part) by the bacterial agent Shigatoxin (Stx), which is actually a family of toxins elaborated by certain strains of Escherichia coli and Shigella dysenteriae. Outcome is usually good in childhood, Shiga toxin-associated HUS, whereas renal and neurological sequelae are more frequently reported in adult, atypical, and familial forms of HUS and in TTP. Recent studies have demonstrated that deficiency in the von Willebrand factor cleaving protease ADAMTS13, due to deficiency of ADAMTS13 can be genetic or more common, acquired, resulting from autoimmune production of inhibitory anti-ADAMTS13 antibodies, that causes TTP. During the last decade, atypical HUS (aHUS) has been demonstrated to be a disorder of the complement alternative pathway dysregulation, as there is a growing list of mutations and polymorphisms in the genes encoding the complement regulatory proteins that alone or in combination may lead to aHUS. Approximately 60% of aHUS patients have so-called 'loss-of-function' mutations in the genes encoding the complement regulatory proteins, which normally protect host cells from complement activation: complement factor H (CFH), factor I (CFI) and membrane cofactor protein (MCP or CD46), or have 'gain-of-function' mutations in the genes encoding the complement factor B or C3. In addition, approximately 10% of aHUS patients have a functional CFH deficiency due to anti-CFH antibodies. Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13. Platelet transfusions are contraindicated. Plasma infusion or exchange (PE) is the only treatment of proven efficacy. PMID:21103695

  19. Diamond-Blackfan anemia and nutritional deficiency-induced anemia in children.

    PubMed

    Gelbart, David

    2014-04-01

    Diamond-Blackfan anemia is a rare, inherited disease that characteristically presents as a chronic, normochromic macrocytosis due to red cell lineage bone marrow failure. Although studies are elaborating on the genetic basis for its associated comorbidities, little has been published comparing this anemia to other chronic anemias that have similar laboratory results in children. This article offers a global perspective of the disease and compares it with anemia due to vitamin B12 and folate deficiency in children. PMID:24662257

  20. Management of hemolytic uremic syndrome

    PubMed Central

    Kavanagh, David; Raman, Shreya

    2014-01-01

    Hemolytic uremic syndrome (HUS) is a disease characterized by hemolysis, thrombocytopenia, and acute kidney injury, although other organs may be involved. Most cases are due to infection with Shiga toxin-producing Escherichia coli (STEC). Early identification and initiation of best supportive care, with microbiological input to identify the pathogen, result in a favorable outcome in most patients. The remaining 10% of HUS cases are classed together as atypical HUS and have a diverse etiology. The majority are due to inherited or acquired abnormalities that lead to a failure to control complement activation. Atypical HUS occurring in other situations (for example, related to pregnancy or kidney transplantation) may also involve excessive complement activation. Plasma therapies can reverse defective complement control, and it is now possible to specifically target complement activation. This has led to improved outcomes in patients with atypical forms of HUS. We will review our current understanding of the pathogenesis of HUS and how this has led to advances in patient care. PMID:25580273

  1. Inborn anemias in mice. Progress report, 1 August 1979-15 July 1980

    SciTech Connect

    Bernstein, S.E.; Russell, E.S.

    1980-08-01

    Four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia are under investigation in mice. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values; (b) determinations of radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme synthesis; (d) histological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli; and (g) transplantation of tissue between individuals of differently affected genotypes.

  2. Hemolytic Anemia in Wild Seaducks Caused by Marine Oil Pollution

    Microsoft Academic Search

    Osamu Yamato; Yoshimitsu Macdc

    Clinico-pathological examinations were conducted on wild white-winged scoters (Melanitta fusca) contamiminated with fuel oil (Bunker G oil) from a capsized cargo ship in February 1993 in Japan. The emythmocyte count, hemoglobin concentration and hematocrit val- ue in the oiled seaducks all were decreased and numerous immature erythrocytes were oh- served in blood smears. In addition, hemosi- derosis was observed in

  3. [Mycoplasma pneumoniae: a cause of febrile hemolytic anemia in travelers].

    PubMed

    Ficko, C; Andriamanantena, D; Flateau, F; Mangouka, L; Soler, C; Carmoi, T; Rapp, C

    2012-01-01

    Mycoplasma pneumoniae can cause varied hematologic manifestations that are frequently associated with lower respiratory tract infections. Acute febrile hemolysis without respiratory symptoms is quite rare. We describe the case of a 25-year-old man, admitted for acute fever with hemolysis, after returning from Djibouti. M. pneumoniae infection was proved by serological testing. A favorable outcome followed macrolide treatment. PMID:23352983

  4. Arthrographis kalrae soluble antigens present hemolytic and cytotoxic activities.

    PubMed

    Nagashima, Luciene Airy; Akagi, Claudia Yuri; Sano, Ayako; Álvares e Silva, Paula Leonello; Murata, Yoshiteru; Itano, Eiko Nakagawa

    2014-12-01

    Arthrographis kalrae is a dimorphic, cosmopolitan and neurotropic fungus that has been described as a rare human pathogen. This study investigated the hemolytic and cytotoxic activities of A. kalrae cell-free antigens (CFAs). Total CFAs and their Sephadex chromatography fractions were tested on mouse erythrocytes for hemolysis and on the P3U1 cell line for cytotoxicity. Hemolytic and cytotoxic activities were detected in distinct molecular mass (MM) fractions. Additionally, antibodies against isogenic erythrocytes sensitized with CFAs (anti-E-CFAs) inhibited hemolysis but not cytotoxicity. Hemolysis was not affected by heating, and a higher reactivity was detected in the carbohydrate-rich fractions, which decreased after reduction by periodate treatment. The pioneering nature of this work is due to the demonstration of the cytotoxic activity in A. kalrae and the suggestion that this activity may be due to molecules distinct from the hemolytic factor, with the latter potentially being a component with a high MM. PMID:25449999

  5. About Anemia

    MedlinePLUS

    ... as citrus fruits, green vegetables, and fortified cereals. Anemia also can develop if the bone marrow is not working properly. This may be because of an infection or a chronic illness, such as arthritis or kidney disease. In rare cases, someone might be born without ...

  6. Hemolytic uremic syndrome: toxins, vessels, and inflammation.

    PubMed

    Cheung, Victoria; Trachtman, Howard

    2014-01-01

    Hemolytic uremic syndrome (HUS) is characterized by thrombotic microangiopathy of the glomerular microcirculation and other vascular beds. Its defining clinical phenotype is acute kidney injury (AKI), microangiopathic anemia, and thrombocytopenia. There are many etiologies of HUS including infection by Shiga toxin-producing bacterial strains, medications, viral infections, malignancy, and mutations of genes coding for proteins involved in the alternative pathway of complement. In the aggregate, although HUS is a rare disease, it is one of the most common causes of AKI in previously healthy children and accounts for a sizable number of pediatric and adult patients who progress to end stage kidney disease. There has been great progress over the past 20?years in understanding the pathophysiology of HUS and its related disorders. There has been intense focus on vascular injury in HUS as the major mechanism of disease and target for effective therapies for this acute illness. In all forms of HUS, there is evidence of both systemic and intra-glomerular inflammation and perturbations in the immune system. Renewed investigation into these aspects of HUS may prove helpful in developing new interventions that can attenuate glomerular and tubular injury and improve clinical outcomes in patients with HUS. PMID:25593915

  7. Hemolytic Uremic Syndrome: Toxins, Vessels, and Inflammation

    PubMed Central

    Cheung, Victoria; Trachtman, Howard

    2014-01-01

    Hemolytic uremic syndrome (HUS) is characterized by thrombotic microangiopathy of the glomerular microcirculation and other vascular beds. Its defining clinical phenotype is acute kidney injury (AKI), microangiopathic anemia, and thrombocytopenia. There are many etiologies of HUS including infection by Shiga toxin-producing bacterial strains, medications, viral infections, malignancy, and mutations of genes coding for proteins involved in the alternative pathway of complement. In the aggregate, although HUS is a rare disease, it is one of the most common causes of AKI in previously healthy children and accounts for a sizable number of pediatric and adult patients who progress to end stage kidney disease. There has been great progress over the past 20?years in understanding the pathophysiology of HUS and its related disorders. There has been intense focus on vascular injury in HUS as the major mechanism of disease and target for effective therapies for this acute illness. In all forms of HUS, there is evidence of both systemic and intra-glomerular inflammation and perturbations in the immune system. Renewed investigation into these aspects of HUS may prove helpful in developing new interventions that can attenuate glomerular and tubular injury and improve clinical outcomes in patients with HUS. PMID:25593915

  8. [The importance of antenatal immunoprophylaxis for prevention of hemolytic disease of the fetus and newborn].

    PubMed

    Starcevi?, Mirta; Mataija, Marina; Sovi?, Dragica; Dodig, Javorka; Matijevi?, Ratko; Kukuruzovi?, Monika

    2011-03-01

    Hemolytic disease of the fetus and newborn (HDFN) is a consequence of maternal alloimmunization against fetal red blood cell antigens. Alloimmunization against D antigen from Rhesus (Rh) blood group system is particularly important because of its strong immunogenicity. During the last few decades, the introduction of RhD prophylaxis by postpartum administration of anti-D immunoglobulin to RhD negative women, now improved with antenatal prophylaxis, has led to a dramatic decrease in perinatal mortality and morbidity from HDFN. However, severe cases have not disappeared, mostly due to prophylaxis failure. In our case, inappropriate prenatal care during the first pregnancy in an RhD negative mother resulted in primary immunization. In the next pregnancy with an RhD positive child, the mother's secondary immune response was extremely strong and led to early development of severe fetal anemia. The fetus survived thanks to the treatment with intrauterine transfusions (IUT), but they caused suppression of erythropoiesis, which lasted for months after birth. The long lasting, late anemia was treated with repeated postnatal red cell transfusions and recombinant human erythropoietin (rHuEPO). Despite the severity of HDFN in our case, the short-term outcome is good. The boy has normal growth until now, but due to the possibility of an adverse long-term neurodevelopmental outcome, this case requires continuous follow up. It also reminds of the fact that RhD alloimmunization remains an actual problem in daily routine. Antenatal prophylaxis is a crucial step in quality care of those who are at a risk of HDFN. PMID:21568074

  9. [Acute erythroblastopenia due to Parvovirus B19 revealing hereditary spherocytosis].

    PubMed

    Kamoun, T; Chabchoub, I; Aissa, K; Ben Mansour, L; Hachicha, M

    2011-09-01

    Acute Parvovirus B19 infection is responsible for blocking the erythroblastic line, usually with no consequences on hematopoiesis except in patients with chronic hemolytic anemia in whom it can evolve to potentially serious acute anemia. We report 2 observations of acute erythroblastopenia revealing hereditary spherocytosis in 2 children (1 boy and 1 girl) of non-consanguineous parents. PMID:21820287

  10. Sickle cell anemia - resources

    MedlinePLUS

    Resources - sickle cell anemia ... The following organizations are good resources for information on sickle cell anemia : American Sickle Cell Anemia Association - www.ascaa.org/ National Heart, Blood, and Lung Institute - www. ...

  11. Iron deficiency anemia

    MedlinePLUS

    Anemia - iron deficiency ... Iron deficiency anemia is the most common form of anemia. Red blood cells bring oxygen to the ... such as your spleen, remove old blood cells. Iron is a key part of red blood cells. ...

  12. What Causes Aplastic Anemia?

    MedlinePLUS

    ... to aplastic anemia. Examples include Fanconi anemia , Shwachman-Diamond syndrome, dyskeratosis (DIS-ker-ah-TO-sis) congenita, and Diamond-Blackfan anemia. Rate This Content: Next >> Featured Video ...

  13. Iron refractory iron deficiency anemia

    PubMed Central

    De Falco, Luigia; Sanchez, Mayka; Silvestri, Laura; Kannengiesser, Caroline; Muckenthaler, Martina U.; Iolascon, Achille; Gouya, Laurent; Camaschella, Clara; Beaumont, Carole

    2013-01-01

    Iron refractory iron deficiency anemia is a hereditary recessive anemia due to a defect in the TMPRSS6 gene encoding Matriptase-2. This protein is a transmembrane serine protease that plays an essential role in down-regulating hepcidin, the key regulator of iron homeostasis. Hallmarks of this disease are microcytic hypochromic anemia, low transferrin saturation and normal/high serum hepcidin values. The anemia appears in the post-natal period, although in some cases it is only diagnosed in adulthood. The disease is refractory to oral iron treatment but shows a slow response to intravenous iron injections and partial correction of the anemia. To date, 40 different Matriptase-2 mutations have been reported, affecting all the functional domains of the large ectodomain of the protein. In vitro experiments on transfected cells suggest that Matriptase-2 cleaves Hemojuvelin, a major regulator of hepcidin expression and that this function is altered in this genetic form of anemia. In contrast to the low/undetectable hepcidin levels observed in acquired iron deficiency, in patients with Matriptase-2 deficiency, serum hepcidin is inappropriately high for the low iron status and accounts for the absent/delayed response to oral iron treatment. A challenge for the clinicians and pediatricians is the recognition of the disorder among iron deficiency and other microcytic anemias commonly found in pediatric patients. The current treatment of iron refractory iron deficiency anemia is based on parenteral iron administration; in the future, manipulation of the hepcidin pathway with the aim of suppressing it might become an alternative therapeutic approach. PMID:23729726

  14. Beta-Hemolytic Streptococcal Erythroderma Syndrome: A Clinical and Pathogenic Analysis

    PubMed Central

    Tyner, Harmony L; Schlievert, M; Baddour

    2011-01-01

    The syndrome of erythroderma due to beta-hemolytic streptococci is rarely seen and should be distinguished from cellulitis and toxic shock-like syndrome. We describe a novel syndrome of non-group A, beta-hemolytic streptococcal infection truncal erythroderma. The characteristics of this syndrome suggest that local factors were likely operative in the cutaneous manifestations of an exotoxin-associated erythroderma. PMID:21841465

  15. Inborn anemias in mice: (Annual report, 1982-1983)

    SciTech Connect

    Bernstein, S.E.

    1983-09-09

    The nature of the defects that shorten the effective lifespan of red blood cells in the circulation and which gave rise to anemia, jaundice and to spleen, liver and heart enlargement are studied because they so closely parallel inherited hemolytic anemias in man. In mice, ''hemolytic disease'' initiated by the ja, sph, sph/sup ha/, or the nb genes has been traced to abnormalities in the protein components of their red cell membranes. Polyacrylamide gel electrophoresis of detergent solubilized membranes reveal that in the different genetic types one or more of the major high molecular weight proteins called spectrins is decreased or totally missing. It is one thing to observe a correlation between missing or defective components in selected analytical procedures, and another to establish a causal relationship between the two. To investigate the possible interrelationships, we examined the associations between spectrin or ankyrin content, the severity of the resulting anemia, red cell osmotic fragilities, and the capacity of cells from each genotype to be deformed in a continuous osmotic gradient at constant sheer stress. Our findings indicate that sensitivity to osmotic stress, cell rigidity (inadequate deformability), deficiency of spectrin or ankyrin, and the severity of the anemia, are statistically highly correlated. 11 refs., 3 tabs.

  16. Diffuse large B-cell lymphoma with hemolytic crisis developed twenty years after the onset of Evans syndrome.

    PubMed

    Yoshimura, Takuro; Nakane, Takahiko; Kamesaki, Toyomi; Inaba, Akiko; Nishimoto, Mitsutaka; Mukai, Satoru; Sakabe, Manami; Ohsawa, Masahiko; Fujino, Keizo; Koh, Hideo; Nakao, Yoshitaka; Nakamae, Hirohisa; Hino, Masayuki

    2014-05-01

    A 65-year-old woman was diagnosed with Coombs-positive autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) in May 1992. One month later, her PRCA went into remission following treatment but she developed idiopathic thrombocytopenic purpura and was diagnosed with Evans syndrome. Although her condition resolved with administration of prednisolone and azathioprine, it was necessary to continue treatment with gradual tapering over the following two decades. In October 2012, her hemolytic anemia again worsened, and lymph node swelling, splenomegaly and B symptoms developed. She was diagnosed as having diffuse large B-cell lymphoma (DLBCL) based on lymph node biopsy. However, AIHA was not considered to be the cause of her hemolytic anemia, but rather to be related to DLBCL. This was because a Coombs test and other extensive investigations for Coombs negative-AIHA yielded negative results. The patient underwent CHOP therapy, and all of her symptoms improved. Herein, we report this rare case in which DLBCL developed after the onset of Evans syndrome. PMID:24881920

  17. What Causes Anemia?

    MedlinePLUS

    ... red blood cells to cause anemia. Lack of Red Blood Cell Production Both acquired and inherited conditions ... also can cause aplastic anemia. High Rates of Red Blood Cell Destruction Both acquired and inherited conditions ...

  18. Sickle Cell Anemia

    MedlinePLUS

    ... do not have the disease itself. What Is Sickle Cell Anemia? Sickle cell anemia is a blood disorder that ... NIH) recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in ...

  19. Nitrite-induced anemia in channel catfish, Ictalurus punctatus Rafinesque

    SciTech Connect

    Tucker, C.S. (Mississippi Agricultural and Forestry Experiment Station, Stoneville (USA)); Francis-Floyd, R.; Beleau, M.H. (College of Veterinary Medicine, Stoneville, MS (USA))

    1989-08-01

    Since 1983 numerous cases of anemia have been reported in populations of channel catfish Ictalurus punctatus Rafinesque cultured in the southeastern United States. Environmental nitrite-nitrogen concentrations of 4 mg/L or more occur sporadically in channel catfish culture ponds, and the frequency of occurrence is greatest in the fall and spring. The authors have observed that some cases of anemia in populations of pond-raised channel catfish follow prolonged exposure to high concentrations of environmental nitrite. However, there was no evidence that exposure of channel catfish to environmental nitrite was the cause of the observed anemia. Hemolytic anemia following nitrite exposure has been described for sea bass Dicentrarchus labrax (L.) and rainbow trout Salmo gairdneri, but not for channel catfish. In the present study the authors show that a variable, but generally mild, anemia develops in channel catfish exposed to nitrite. They also offer a management procedure for preventing the development of anemia during periods of elevated environmental nitrite concentrations.

  20. Age, anemia, and fatigue

    Microsoft Academic Search

    Matti S. Aapro; David Cella; Martin Zagari

    2002-01-01

    Many conditions that would not be considered normal in a younger population are routinely accepted in older people as a part of so-called “normal” aging. Among these conditions are many chronic and debilitating conditions such as chronic pain, insomnia, weakness, fatigue, and anemia. This article reviews current evidence regarding the relationships among age, fatigue, weakness, anemia, and erythropoiesis. Anemia in

  1. Effects of immunosuppressive therapy in a patient with aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome during ongoing eculizumab treatment.

    PubMed

    Asano, Jin; Ueda, Ryosuke; Tanaka, Yasuhiro; Shinzato, Isaku; Takafuta, Toshiro

    2014-01-01

    A 65-year-old woman experienced a hemolytic attack triggered by sepsis. She presented with markedly increased CD55(-) CD59(-) erythrocytes and the signs of bone marrow failure, which led to a diagnosis of aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome. There was a possibility of increasing hemolysis, as large PNH clones remained after immunosuppressive therapy (IST). Accordingly, eculizumab was first used to control the hemolytic attack followed by IST with antithymocyte globulin and cyclosporine A. The patient was successfully weaned from blood transfusions and has been followed up without any recurrence of hemolytic attacks. PMID:24429452

  2. Posttransplant anemia in solid organ recipients.

    PubMed

    Blosser, Christopher D; Bloom, Roy D

    2010-04-01

    Posttransplantation anemia (PTA) is a prevalent sequela of solid organ transplantation and a potential independent risk factor for cardiovascular morbidity and mortality in kidney transplant recipients. There are multiple causes of PTA, some of which are associated with early phase anemia (<6 months), whereas others more often induce anemia in the late posttransplant phase (>6 months). Although impaired kidney function contributes to PTA, it is only one of many factors that result in anemia in transplant recipients. Other causes include iron deficiency, medications, infections, acute rejection, inflammation, and erythropoietin deficiency. Unlike in the predialysis chronic kidney disease population, the impact of anemia after kidney transplantation outcomes is unknown. This is in large part due to the absence of controlled trials that address whether correction of anemia improves allograft function or patient morbidity and mortality. Current guidelines recommend evaluation for hemoglobin level of less than 12 g/dL and treatment when the value falls less than 11 g/dL and a target of 11 to 12 g/dL. Additional treatments may entail removing the cause of the anemia, nutritional supplementation, and/or an erythrocyte stimulating agent. PMID:20303457

  3. Inborn anemias in mice: (Annual report, 1980-1981)

    SciTech Connect

    Bernstein, S.E.

    1981-07-02

    The basic purpose of this study is the delineation and exploitation of inborn anemias of the laboratory mouse, carried out by utilization of genetically homogeneous stocks segregating only for anemia-producing genes; by physiological and histological descriptions of each condition at all stages in the life history; by determination of tissue sites of primary gene action through tissue culture studies, tissue transplantation and parabiosis experiments; by analysis of reactions of normal and anemic mice to a variety of stressful stimuli, including x-irradiation, hypoxia, and toxic chemicals, and by biochemical comparisons between tissues, especially erythrocytes and hemopoietic cells of normal vs each type of anemic mouse. At present 16 single-locus anemias are known in the mouse, plus one with multifactorial inheritance (the autoimmune hemolytic anemia of NZB inbred mice). Of these, six are maintained only by the Jackson Laboratory, and two others have but one additional source. Effects of anemia-producing mutant alleles of these loci (an; f; ja; ha; Hba/sup th/; mk; nb; Sl and Sl/sup d/; sla; sph; and W, W/sup v/, W/sup J/ and 10 other putative W-alleles) are currently under investigation at the Jackson Laboratory. 15 refs.

  4. Iron deficiency anemia.

    PubMed

    Naigamwalla, Dinaz Z; Webb, Jinelle A; Giger, Urs

    2012-03-01

    Iron is essential to virtually all living organisms and is integral to multiple metabolic functions. The most important function is oxygen transport in hemoglobin. Iron deficiency anemia in dogs and cats is usually caused by chronic blood loss and can be discovered incidentally as animals may have adapted to the anemia. Severe iron deficiency is characterized by a microcytic, hypochromic, potentially severe anemia with a variable regenerative response. Iron metabolism and homeostasis will be reviewed, followed by a discussion of diagnostic testing and therapeutic recommendations for dogs and cats with iron deficiency anemia. PMID:22942439

  5. Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

    PubMed Central

    Mijatovic, Dino; Blagaic, Ana; Zupan, Zeljko

    2014-01-01

    Introduction: Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF). Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions. PMID:25075296

  6. Fifth Cooley's anemia symposium

    SciTech Connect

    Bank, A.; Anderson, W.F.; Zaino, E.C.

    1985-01-01

    This book discusses the topics presented at the symposium on the subject of 'Thalassemia'. Sickle cell anemia is also briefly discussed. The aspects discussed are chromosomal defects of anemias particularly globin synthesis, and the role of messenger RNA and other chromosomes.

  7. Sickle Cell Anemia

    MedlinePLUS

    Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They ... last as long as normal, round red blood cells. This leads to anemia. The sickle cells also ...

  8. Anemia: determining the cause.

    PubMed

    Fleischman, Wendy

    2012-06-01

    Anemia is a common finding in small animal practice; however, the multitude of potential causes can make determining the underlying diagnosis a challenging and frustrating endeavor. With a basic understanding of red blood cell production and a systematic diagnostic approach, clinicians should be able to clearly define the cause of anemia in most cases. PMID:22692674

  9. Anemia of renal disease

    Microsoft Academic Search

    Cathy E. Langston; Adam Eatroff

    2011-01-01

    Patient group It is estimated that 15–30% of geriatric cats will develop chronic kidney disease (CKD), and that 30–65% of these cats will develop anemia as their renal disease worsens. Anemia of renal disease is multifactorial in its pathogenesis, but the main cause is reduced production of erythropoietin, a renal hormone that controls the bone marrow's production of red blood

  10. Fatal hemolysis induced by ceftriaxone in a child with sickle cell anemia

    Microsoft Academic Search

    Juan C. Bernini; Mahmoud M. Mustafa; Laurie J. Sutor; George R. Buchanan

    1995-01-01

    A 2-year-old boy with sickle cell anemia had a massive, fatal hemolytic reaction after administration of an intravenous dose of ceftriaxone. Laboratory studies demonstrated the presence of an IgM antibody against ceftriaxone, binding to and destroying the patient's erythrocytes by an immune complex mechanism. This rare complication should be considered in the differential diagnosis when hemoglobinuria develops in a child

  11. Hemolytic uremic syndrome in solid-organ transplant recipients

    Microsoft Academic Search

    Nina Singh; Timothy Gayowski; Ignazio R. Marino

    1996-01-01

    Post-transplant hemolytic uremic syndrome characterized by microangiopathic hemolysis, thrombocytopenia, and renal failure is an infrequent but potentially serious complication in organ transplant recipients. Hemolytic uremic syndrome developed in 2% (2\\/100) of our consecutive liver transplants. We report our patients and review a total of 91 cases of hemolytic uremic syndrome in adult solid organ transplant recipients reported in the literature.

  12. Heme-Regulated eIF2? Kinase Plays a Crucial Role in Protecting Erythroid Cells against Pb-Induced Hemolytic Stress.

    PubMed

    Wang, Xiaoyan; Wang, Lixin; Liu, Sijin

    2015-03-16

    Lead (Pb) is a heavy metal with considerable environmental contamination. It is toxic to diverse cells and has been reported to cause a wide array of detrimental health problems including neurological disorders and anemia. In light of the mechanisms underlying Pb-induced anemia, the current understanding is still limited, in spite of efforts for years. Our previous studies recognized a protective role for the heme-regulated eIF2? kinase (Hri) in erythroid cells against oxidative stress exerted by arsenic and cadmium. Whether Hri is involved in Pb-induced hemolytic stress has not been scrutinized. In the current study, to more stringently address this question, we looked into erythropoiesis upon Pb(NO3)2 exposure by using an in vivo mouse model and ex vivo cultured E14.5 fetal liver cells. Diagnoses of hemolytic anemia, decreased red cell count, reduced hemoglobin concentration, and elevated bilirubin level were observed in Hri knockout (Ko) mice only, upon low-dose Pb administration. Significantly different from Ko mice, wild type (Wt) mice did not develop hemolytic anemia. Enforced extramedullary and medullary erythropoieses were found in Ko mice with Pb exposure. However, anemia was not compensated in Hri-deficient mice, as in vivo and ex vivo results manifested that expanded Hri-null erythroid precursors experienced blocked differentiation and enhanced apoptosis, leading to ineffective erythropoiesis under Pb exposure. Additionally, Pb treatment also promoted hepcidin expression and consequentially increased splenic iron storage, resulting in restrained iron availability for erythropoiesis. All considered, Hri-null erythroid precursors were prone to Pb-induced hemolytic stress. Hri deficiency gave rise to ineffective erythropoiesis and reduced iron availability for erythropoiesis under Pb stimulation, and these events together exacerbated Pb-induced hemolytic anemia. It is thus conceivable that this study delineated an indispensable function of Hri in maintaining red cell membrane integrity and guiding erythroid cell differentiation under Pb exposure. Our findings therefore deciphered a crucial role for Hri in protecting erythroid cells against Pb-induced toxicity. PMID:25411909

  13. Reassessment of the microcytic anemia of lead poisoning

    SciTech Connect

    Cohen, A.R.; Trotzky, M.S.; Pincus, D.

    1981-06-01

    Hematologic abnormalities in childhood lead poisoning may be due, in part, to the presence of other disorders, such as iron deficiency or thalassemia minor. In order to reassess increased lead burden as a cause of microcytic anemia, we studied 58 children with class III or IV lead poisoning, normal iron stores, and no inherited hemoglobinopathy. Anemia occurred in 12% and microcytosis in 21% of these children. The combination of anemia and microcytosis was found in only one of 58 patients (2%). When only children with class IV lead poisoning were studied, the occurrence of microcytosis increased to 46%. However, the combination of microcytosis and anemia was found in only one of these 13 more severely affected patients. Microcytic anemia was similarly uncommon in children with either blood lead concentration greater than or equal to 50 microgram/100 ml. These data indicate that microcytosis and anemia occur much less commonly than previously reported in childhood lead poisoning uncomplicated by other hematologic disorders.

  14. Depressed eruption rate of the rat maxillary incisor in a drug-induced uncompensated hemolytic state model

    SciTech Connect

    Giglio, M.J.; Sanz, A.M.; Bozzini, C.E. (Univ. of Buenos Aires (Argentina))

    1990-03-01

    Female rats weighing about 180 g were separated into two groups. One group (A) received phenylhydrazine (PHZ) every other day during three weeks (for induction of an uncompensated hemolytic state), while the control group (C) received saline. The evidence for the establishment of the uncompensated hemolytic state was obtained by hematocrit value, reticulocyte count, and red-cell-volume-59Fe uptake. Body-weight gain (which is a measure of overall body growth rate), body-length gain (which is a measure of longitudinal skeletal growth rate), food intake, and maxillary incisor eruption rate (ER) were significantly depressed in rats of group A during the PHZ-injection period, in relation to rats of group C. These results indicate that anemia and/or associated factors depress ER, along with body growth and skeletal growth.

  15. MEGALOBLASTIC AND OTHER MACROCYTIC ANEMIA

    E-print Network

    9/16/2013 1 MEGALOBLASTIC AND OTHER MACROCYTIC ANEMIA MACROCYTOSIS MCV > 100 fL MCHC ­ Normal False) Absorption Transport VITAMIN B 12 DEFICIENCY Pernicious Anemia Shilling Test Other Causes of Malabsorption Oral Parenteral ­ Pernicious Anemia OTHER MEGALOBLASTIC ANEMIAS Drugs Enzyme Deficiencies Congenital

  16. Anemia in Chronic Kidney Disease

    MedlinePLUS

    ... Topics and Titles : Anemia in Chronic Kidney Disease Anemia in Chronic Kidney Disease On this page: What is anemia? How is ... carry oxygen throughout the body. [ Top ] What causes anemia in chronic kidney disease? When kidneys are diseased or damaged, they do ...

  17. Anemia in Frailty

    PubMed Central

    Roy, Cindy N.

    2010-01-01

    Synopsis While anemia is regarded as a relatively common occurrence in older adults, the vigor with which the medical community should intervene to correct this common problem is disputed. Epidemiologic data clearly correlate anemia with functional decline, disability and mortality. Anemia may contribute to functional decline by restricting oxygen delivery to muscle, or to cognitive decline by restricting oxygen delivery to the brain. On the other hand, the erythron may be a separate target of the same biological mediators that influence deterioration of physiologic systems that contribute to weakness, functional and cognitive decline and mortality. Clinical trials aimed to treat anemia in older adults could assess whether physical performance is improved or whether mortality risk declines with improved hemoglobin, but sufficient evidence from such trials is currently lacking. With few guidelines regarding treatment for older adults and significant risk for adverse events associated with transfusion and erythroid stimulating agents (ESA), anemia often goes untreated or ignored in geriatric clinics. This article reviews the problem of anemia in older adults, with a particular emphasis on the frail elderly. We will review the gaps in our evidence base for the treatment of anemia in older adults and assess options for advancing the field. PMID:21093723

  18. Anemia in the Elderly

    PubMed Central

    Berliner, Nancy

    2013-01-01

    As the population ages, increasing attention has become focused on the prevalence of anemia in elderly individuals. Anemia occurs in more than 10% of individuals who are older than the age of 65 years, and it increases to more than 50% in individuals who are older than the age of 80 years. Although the anemia is typically mild and unlikely to result in symptoms, it is uniformly associated with increased morbidity and mortality as assessed in large cohort studies. Anemia is an independent predictor of these adverse outcomes both in healthy community-dwelling subjects and in patients with significant co-morbidities. Efforts to understand the pathophysiology of anemia in this population, especially the one third of patients with “unexplained” anemia, have focused on the potential contributions of inflammatory pathways, erythropoietin resistance, and changes in hematopoietic stem cells to the age-dependent decrease in red cell mass. We would argue that these pathways are closely interrelated and combine to lead to anemia in aging individuals. This brief review summarizes the current understanding of this entity and our studies aimed at further delineating its pathophysiology. PMID:23874029

  19. Hemolytic Streptococci in Raw Market Milk

    Microsoft Academic Search

    J. B. Gunnison; M. P. Luxen; M. S. Marshall; B. Q. Engle

    1940-01-01

    The Laneefield technic (1) for the serologic grouping of hemolytic strep- tococci has furnished, for the first time, a method of detecting significant differences within this group of organisms. This procedure has been used to study streptococci of bovine origin by Plastridge and Hartsell (2), Stable- forth (3), Stewart (4) and Edwards (5) among others. Sherman and Riven (6) and

  20. Typical and Atypical Hemolytic Uremic Syndrome

    Microsoft Academic Search

    Willem Proesmans

    1996-01-01

    The hemolytic uremic syndrome is the most frequent cause of acute renal failure in childhood. In the vast majority of patients, the syndrome of acute hemolysis, thrombopenia and renal dysfunction is preceded by an episode of diarrhea with or without bloody stools. This colitis is caused by different strains of Escherichia coli which produce shiga like toxins. These toxins are

  1. Atypical hemolytic uremic syndrome with membranoproliferative glomerulonephritis.

    PubMed

    Mehta, Kumud; More, Vaishali; Chitale, Arun; Khubchandani, Shaila

    2013-08-01

    Atypical hemolytic uremic syndrome (aHUS) associated with membranoproliferative glomerulonephritis (MPGN) is an uncommon clinical presentation, especially in children. We report a 8-year-old-boy who presented like aHUS but the kidney biopsy showed MPGN type 1. PMID:24036644

  2. Etiopathogenesis of hemolytic reactions in total artificial heart recipients.

    PubMed

    Vask?, J; Urbánek, P

    1997-12-01

    Hemolysis in total artificial heart (TAH) recipients was analyzed. From a total of 66 long-term experiments lasting from 30-314 days performed in the Brno Research Center, in 53 animals, the total red blood cell (RBC) count, hematocrit, total hemoglobin, and free plasma hemoglobin were investigated. We could essentially divide the whole group of calves in 2 subgroups. The first subgroup was calves with hemolytic reactions, and the second subgroup was calves without any hemolytic reaction at all. In the first subgroup, hemolysis occurred in 47% of the overall number of animals during extracorporeal circulation (ECC), in 15% during ECC and later periodically during the experiment, in 8% during ECC and then continuously during the experiment, and finally in 10% not during ECC but repeatedly during the experiment. In 20% of the animals from the overall number, hemolysis did not occur at all (second subgroup). These results testify to the great individual differences within 1 breed (Bohemian with a substantial component of Holstein). These differences are further modified by exogenous and endogenous factors. First, the inborn resistance of the RBC membrane and also thrombi formation and the mineralization of the driving diaphragm are very important. The extreme situation of decreased RBC membrane resistance was proved using a calf from another breed, the slow growing Scottish Highland breed, which did not survive 22 days of pumping due to intractable lethal hemolysis. These factors are also indicated by the hemolytic action of some drugs (e.g., Dopegyt) used during the experiment for another reason. Also important are the mechanical forces of pumping, surface moieties of the biomaterial, mineralization of the driving diaphragms, thrombi formation, infection, etc. Essentially, the hemolytic reaction in the TAH recipient has a multifactorial character. Hemolysis is undoubtedly an important factor, which can have a profound impact on the length of survival. The experimental and clinical experiences must be continuously integrated, and conclusions valid for human TAH application must be considered as very important for further TAH experimental and clinical research. PMID:9423978

  3. Iron-Deficiency Anemia

    MedlinePLUS Videos and Cool Tools

    ... a lower than normal number of red blood cells. Red blood cells carry oxygen and remove carbon dioxide (a waste ... Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE-muh-glow- ...

  4. Cooley's Anemia Foundation

    MedlinePLUS

    Cooley's Anemia Foundation Leading the Fight against Thalassemia About Us Mission/Purpose History Medical Research Board/Staff Contact the Foundation U.S. Patients: Register for Information Learn about Thalassemia About Thalassemia Clinical ...

  5. Anemia in the Newborn

    MedlinePLUS

    ... mother's abdomen when the umbilical cord is clamped. Twin-to-twin transfusions, in which blood flows from one fetus to the other, can cause anemia in one twin and too much blood (polycythemia) in the other ...

  6. [Heart failure and anemia].

    PubMed

    Reda, S; Motloch, L J; Hoppe, U C

    2013-09-01

    Chronic heart failure has an age-dependent prevalence of 2% and is therefore one of the most frequent diseases in western societies. A reduced hemoglobin concentration according to the definition of the World Health Organization is a common comorbidity affecting more than half of all heart failure patients. Elderly patients, patients suffering from renal impairment and women are more likely to develop anemia but a definitive etiology of anemia is only identified in the minority of cases. Anemia is associated with a poor clinical status and a greater risk of hospitalization and is a predictive factor for increased mortality. The incidence of anemia appears to increase with a poorer functional class. Intravenous iron therapy improves the exercise capacity in patients with systolic heart failure and iron deficiency and is currently being recommended for patients with persistent symptoms despite optimal medical and device therapy. However, erythropoietin-stimulating agents as a treatment for anemia in chronic heart failure have failed to improve clinical outcome in a large randomized trial. In patients with heart failure but with maintained ejection fraction, anemia is also associated with a poor prognosis. Specific therapeutic recommendations for these patients are still not available. PMID:23900390

  7. FURTHER STUDIES WITH TOXIC SERUM EXTRACTS OF HEMOLYTIC STREPTOCOCCI.

    PubMed

    Weld, J T

    1935-03-31

    1. The same Streptococcus hemolyticus organisms may be subjected to extraction six times in 2 days with untreated inactivated serum with no loss in potency of the later extracts when the organisms are kept frozen solid during the night between the extractions. 2. The serum extract toxins of hemolytic streptococci can be preserved without deterioration for at least 6 months if kept frozen solid. 3. No toxins stronger than those containing 10 units per cc. for mice have been prepared. Reasons for thinking that this is due to the saturation of the serum with the toxin at this point are given. 4. Half saturation with (NH(4))(2)SO(4) precipitates out practically all of the hemotoxin in a preparation. 5. Serum extracts were made from strains of hemolytic streptococci other than the Gay strain and attempts were made to correlate the virulence and toxin production from each strain. No such correlation could be established. 6. The principal pathologic finding in mice inoculated with the streptococcus serum extract toxin is a marked degeneration of the tubular epithelium of the kidney. PMID:19870372

  8. Anemia, tumor hypoxemia, and the cancer patient

    SciTech Connect

    Varlotto, John [Department of Radiation Oncology, Boston VA Medical Center, Boston, MA (United States) and Department of Radiation Oncology, Beth (Israel) and Deaconess Medical Center, Harvard Medical School, Boston, MA (United States)]. E-mail: jvarlott@bidmc.harvard.edu; Stevenson, Mary Ann [Department of Radiation Oncology, Boston VA Medical Center, Boston, MA (United States); Department of Radiation Oncology, Beth Israel/Deaconess Medical Center, Harvard Medical School, Boston, MA (United States)

    2005-09-01

    Purpose: To review the impact of anemia/tumor hypoxemia on the quality of life and survival in cancer patients, and to assess the problems associated with the correction of this difficulty. Methods: MEDLINE searches were performed to find relevant literature regarding anemia and/or tumor hypoxia in cancer patients. Articles were evaluated in order to assess the epidemiology, adverse patient effects, anemia correction guidelines, and mechanisms of hypoxia-induced cancer cell growth and/or therapeutic resistance. Past and current clinical studies of radiosensitization via tumor oxygenation/hypoxic cell sensitization were reviewed. All clinical studies using multi-variate analysis were analyzed to show whether or not anemia and/or tumor hypoxemia affected tumor control and patient survival. Articles dealing with the correction of anemia via transfusion and/or erythropoietin were reviewed in order to show the impact of the rectification on the quality of life and survival of cancer patients. Results: Approximately 40-64% of patients presenting for cancer therapy are anemic. The rate of anemia rises with the use of chemotherapy, radiotherapy, and hormonal therapy for prostate cancer. Anemia is associated with reductions both in quality of life and survival. Tumor hypoxemia has been hypothesized to lead to tumor growth and resistance to therapy because it leads to angiogenesis, genetic mutations, resistance to apoptosis, and a resistance to free radicals from chemotherapy and radiotherapy. Nineteen clinical studies of anemia and eight clinical studies of tumor hypoxemia were found that used multi-variate analysis to determine the effect of these conditions on the local control and/or survival of cancer patients. Despite differing definitions of anemia and hypoxemia, all studies have shown a correlation between low hemoglobin levels and/or higher amounts of tumor hypoxia with poorer prognosis. Radiosensitization through improvements in tumor oxygenation/hypoxic cell sensitization has met with limited success via the use of hyperbaric oxygen, electron-affinic radiosensitizers, and mitomycin. Improvements in tumor oxygenation via the use of carbogen and nicotinamide, RSR13, and tirapazamine have shown promising clinical results and are all currently being tested in Phase III trials. The National Comprehensive Cancer Network (NCCN) guidelines recommend transfusion or erythropoietin for symptomatic patients with a hemoglobin of 10-11 g/dl and state that erythropoietin should strongly be considered if hemoglobin falls to less than 10 g/dl. These recommendations were based on studies that revealed an improvement in the quality of life of cancer patients, but not patient survival with anemia correction. Phase III studies evaluating the correction of anemia via erythropoietin have shown mixed results with some studies reporting a decrease in patient survival despite an improvement in hemoglobin levels. Diverse functions of erythropoietin are reviewed, including its potential to inhibit apoptosis via the JAK2/STAT5/BCL-X pathway. Correction of anemia by the use of blood transfusions has also shown a decrement in patient survival, possibly through inflammatory and/or immunosuppressive pathways. Conclusions: Anemia is a prevalent condition associated with cancer and its therapies. Proper Phase III trials are necessary to find the best way to correct anemia for specific patients. Future studies of erythropoietin must evaluate the possible anti-apoptotic effects by directly assessing the tumor for erythropoietin receptors or the presence of the JAK2/STAT5/BCL-X pathway. Due to the ability of transfusions to cause immunosuppression, most probably through inflammatory pathways, it may be best to study the effects of transfusion with the prolonged use of anti-inflammatory medications.

  9. Megaloblastic anemia presenting with massive reversible splenomegaly.

    PubMed

    Behera, Vineet; Randive, Makarand; Sharma, Praveen; Nair, Velu

    2015-06-01

    Megaloblastic anemia (MA) is a common disorder with varied manifestations. It generally results in mild to moderate splenomegaly which is due to sequestration of macrocytic erythrocytes in spleen. Massive splenomegaly is generally seen in infections, myeloproliferative diseases, neoplasms, storage disorders or hematological conditions; but is not heard of and has rarely been reported in MA. We discuss a case of massive splenomegaly who presented with symptomatic anemia and was found to have MA. He was extensive evaluated for all other causes of massive splenomegaly which was normal. Further, after a therapeutic trial of MA he showed a regression in spleen size confirming that the massive splenomegaly was attributable to MA. PMID:25825577

  10. What Causes Sickle Cell Anemia?

    MedlinePLUS

    ... from the NHLBI on Twitter. What Causes Sickle Cell Anemia? Sickle cell anemia is an inherited disease. ... can also raise the risk for infection. Sickle Cell Trait People who inherit a sickle hemoglobin gene ...

  11. Facts about Diamond Blackfan Anemia

    MedlinePLUS

    ... Form Controls NCBDDD Cancel Submit Search The CDC Diamond Blackfan Anemia (DBA) Note: Javascript is disabled or ... Español (Spanish) Recommend on Facebook Tweet Share Compartir Diamond Blackfan anemia (DBA) is a rare blood disorder ...

  12. Initiation and Regulation of Complement during Hemolytic Transfusion Reactions

    PubMed Central

    Stowell, Sean R.; Winkler, Anne M.; Maier, Cheryl L.; Arthur, C. Maridith; Smith, Nicole H.; Girard-Pierce, Kathryn R.; Cummings, Richard D.; Zimring, James C.; Hendrickson, Jeanne E.

    2012-01-01

    Hemolytic transfusion reactions represent one of the most common causes of transfusion-related mortality. Although many factors influence hemolytic transfusion reactions, complement activation represents one of the most common features associated with fatality. In this paper we will focus on the role of complement in initiating and regulating hemolytic transfusion reactions and will discuss potential strategies aimed at mitigating or favorably modulating complement during incompatible red blood cell transfusions. PMID:23118779

  13. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation

    PubMed Central

    Matsukuma, Eiji; Imamura, Atsushi; Iwata, Yusuke; Takeuchi, Takamasa; Yoshida, Yoko; Fujimura, Yoshihiro; Fan, Xinping; Miyata, Toshiyuki; Kuwahara, Takashi

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underwent surgical repair of tetralogy of Fallot. Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed. Further evaluation revealed that his complement factor H (CFH) level was normal and that anti-FH antibodies were not detected in his plasma. Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal. His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13) activity was also normal. However, he had a potentially causative mutation (R425C) in complement component C3. Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS. We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation. PMID:25431709

  14. Serum transferrin receptor in the megaloblastic anemia of cobalamin deficiency.

    PubMed

    Carmel, R; Skikne, B S

    1992-11-01

    In order to further study the relation between transferrin receptor and erythropoiesis we examined serum receptor levels in megaloblastic anemia, which is the classic example of ineffective erythropoiesis. We studied 33 patients with unequivocal cobalamin deficiency, only 22 of whom were anemic. High serum transferrin receptor levels were found in 12 patients, all of whom were anemic and had high lactate dehydrogenase (LDH) levels; in contrast, only 10 of the 21 patients with normal receptor levels were anemic. Receptor correlated most strongly with LDH (r = 0.573, p < 0.001) and, inversely, with hemoglobin values (r = -0.560, p < 0.001); it also correlated with ferritin and total bilirubin levels, but not with cobalamin, MCV or erythropoietin. No association was found with the hemolytic component of megaloblastic anemia, represented indirectly by haptoglobin levels. Changes induced by cobalamin therapy were also examined in 13 patients. Transferrin receptors rose in all 6 patients who initially had high levels and in 2 of 3 patients who had borderline levels, but not in the 4 patients with initially normal levels. The receptor levels began to rise within 1-3 days, peaked at about 2 weeks and returned to normal at about the 5th wk. The findings indicate that serum transferrin receptor levels reflect the severity of the megaloblastic anemia. The elevated receptor levels rise further with cobalamin therapy, however, as effective erythropoiesis replaces ineffective erythropoiesis, and these persist until the increased erythropoiesis returns to normal. PMID:1473586

  15. Anemia and inflammatory bowel diseases

    Microsoft Academic Search

    Fernando Gomollón; Javier P Gisbert

    2009-01-01

    Abstract Too often anemia,is considered,a rare or unimportant manifestation,in inflammatory,bowel,disease,(IBD). However, over the last 10 years a number of studies have been conducted,and the most relevant conclusions obtained are: (1) anemia,is quite common,in IBD; (2) although,in many,cases anemia,parallels the clinical activity of the disease, many patients in remission have anemia, and iron, vitamin B12 and\\/or folic acid deficiency; (3) anemia,

  16. Living with Anemia

    MedlinePLUS

    ... and young children have a greater need for iron because of their rapid growth. Not enough iron can lead to anemia. Premature and low-birth- ... only or formula that isn't fortified with iron, especially after the child is 6 months old. ...

  17. Aplastic Anemia, Pediatric Aspects

    Microsoft Academic Search

    BLANCHE P. A LTER

    Inherited bone marrow failure syndromes (BMFs) comprise at least one-fourth of children with aplastic ane- mia, and perhaps up to 10% of adults. The most common syndrome is Fanconi's anemia (FA), with more than 1,000 reported cases. FA is autosomal recessive, with birth defects in ~75% of patients. It is a DNA repair syn- drome, diagnosed by finding chromosomal aberrations

  18. Sickle Cell Anemia Bibliography.

    ERIC Educational Resources Information Center

    Christy, Steven C.

    Presents sources for the acquisition of medical, social, psychological, educational, and practical knowledge of sickle cell anemia. The materials listed are designed to help parents, educators, and public service workers. Materials include journal articles, films, brochures, slides, and fact sheets. The usual bibliographic information is given.…

  19. Anemia and inflammatory bowel diseases

    PubMed Central

    Gomollón, Fernando; Gisbert, Javier P

    2009-01-01

    Too often anemia is considered a rare or unimportant manifestation in inflammatory bowel disease (IBD). However, over the last 10 years a number of studies have been conducted and the most relevant conclusions obtained are: (1) anemia is quite common in IBD; (2) although in many cases anemia parallels the clinical activity of the disease, many patients in remission have anemia, and iron, vitamin B12 and/or folic acid deficiency; (3) anemia, and also iron deficiency without anemia, have important consequences in the clinical status and quality of life of the patient; (4) oral iron can lead to gastrointestinal intolerance and failure of treatment; (5) intravenous iron is an effective and safe way to treat iron deficiency; (6) erythropoietin is needed in a significant number of cases to achieve normal hemoglobin levels. Thus, the clinician caring for IBD patients should have a comprehensive knowledge of anemia, and apply recently published guidelines in clinical practice. PMID:19787829

  20. Letters to the Editor Autoimmunity due to RAG deficiency and esti-

    E-print Network

    Yandell, Mark

    autoimmune hemolytic anemia, recurrent viral and bacterial infections, and nephrotic syndrome. Patient 2 (P2 the phenotype beyond T2 /B2 /natural killer1 severe combined immunodeficiency (SCID) and Omenn syndrome (OS).1 (rituximab). Both siblings had significantly reduced naive CD41 T-cell counts. Notable family history

  1. Inborn anemias in mice. Comprehensive progress report, 1 August 1979-1 June 1982, to accompany twenty-seventh renewal proposal

    SciTech Connect

    Bernstein, S.E.; Russell, E.S.; Barker, J.E.

    1982-07-01

    Hereditary anemias of mice have been investigated including four macrocytic anemias, three hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules controlling a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse and by extension to man from an understanding of mammalian mechanisms utilized in the control of erythropoiesis. Each of the different anemias is studied through: (a) biochemical and biophysical characterization of peripheral blood cells; (b) determinations of cellular and organismic radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme biosynthesis; (d) morphological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli and inhibitors; and (g) physiological complementation analysis via transplantation of tissue between individuals of differently affected genotypes.

  2. Generation of hemolytic activity in ozone-treated phosphatidylcholine

    SciTech Connect

    Butterman, J.; Chan, P.C.; Kesner, L.

    1987-04-01

    When liposomes prepared from purified soybean phosphatidylcholine were treated with ozone, at least two types of hemolytic agents were formed. One type was stable at 0 degree C but was destroyed rapidly at 37 degrees C. A second type was evolved during storage of ozone-treated phosphatidylcholine at 37 degrees C in the absence of EDTA. This study is concerned mainly with the heat-labile type. The hemolytic activity was not associated with lipid hydroperoxides. A number of substances were shown to inhibit the hemolytic activity and these may be divided into two classes. The first included cysteine, polyamines, n-heptylamine, semicarbazide, and tryptophan. Preincubation of the ozone-treated phosphatidylcholine was necessary with a Class 1 inhibitor, presumably for the interaction of the inhibitor with a functional group of the hemolytic agents. The Class II inhibitors, including BHT and vitamin C, required no preincubation. These possibly abolished the hemolytic activity by scavenging free radicals in the process.

  3. Inborn anemias in mice: (Annual report, 1983-1984)

    SciTech Connect

    Bernstein, S.E.

    1984-09-01

    The hypotranserrinemic-hemochromatosis mutation in mice discovered in our laboratory is an almost exact duplicate of human atransferrinemia. Just as in man, the condition is inherited as a recessive lethal. The disease appears to stem from a congenital deficiency in transferrin. The new mutation arose spontaneously in BALB/c mice and results in death before 12 days of age. It is characterized by stunted growth, low numbers of erythrocytes, hypochromia, and in the absence of jaundice. Treatments with Imferon or other iron preparations were uniformly unsuccessful, but the use of normal mouse serum proved successful as a therapeutic measure. We find that we are able to keep these afflicted mice alive for more than a year with small amounts of normal serum, and transferrin bands are missing on cellulose acetate electrophoresis of serum proteins from affected individuals receiving no treatment. Genetic tests indicated that the new mutation was not an allele of any of the other known iron deficiency anemias in the mouse: sex linked anemia (sla), microcytic anemia (mk), or flexed anemia (f) or any of the members of the hemolytic disease group (sph, sph/sup ha/, nb, or ja). Biochemical and genetic analyses carried out during the past year indicate that the new mutation, tentatively designated hpx is not likely to be a mutation at the transferrin (Trf) locus on Chromosome 9. We observed no unusual serum proteins on cellulose acetate electrophoresis, such as might be expected if the Trf gene had mutated. Moreover, radial immunodiffusion examination and Ouchterlony analysis did not show the presence of smaller molecules (or fragments) with transferrin antigenic specificities. Instead they showed a total loss in serum transferrin. 14 refs., 5 tabs.

  4. Acute methemoglobinemia with hemolytic anemia following bio-organic plant nutrient compound exposure: Two case reports

    PubMed Central

    Malkarnekar, Santoshi Balkrishna; Anjanappa, Raveesha; Naveen, L.; Kiran, B. G.

    2014-01-01

    Two young women, were reffered to our hospital on two different occasions with history of breathlessness and mental confusion, following consumption of two different bio-organic plant nutrient compounds with a suicidal intent. On examination, they had cyanotic mucous membranes, and their blood samples showed the classic ‘dark chocolate brown’ appearance. Work up revealed cyanosis unresponsive to oxygen supplementation and absence of cardiopulmonary abnormality. Pulse oximetry revealed saturation of 75% in case 1 and 80% in case 2, on 8 liters oxygen supplementation via face masks, although their arterial blood gas analysis was normal, suggestive of “saturation gap”. Methemoglobinemia was suspected based on these findings and was confirmed by Carbon monoxide-oximetry (CO-oximetry). Methylene blue was administered and the patients showed dramatic improvement. Both the patients developed evidence of hemolysis approximately 72 hours following admission which improved with blood transfusion and supportive treatment. The patients were eventually discharged without any neurological sequalae. PMID:24678158

  5. A genome-wide association study of total bilirubin and cholelithiasis risk in sickle cell anemia.

    PubMed

    Milton, Jacqueline N; Sebastiani, Paola; Solovieff, Nadia; Hartley, Stephen W; Bhatnagar, Pallav; Arking, Dan E; Dworkis, Daniel A; Casella, James F; Barron-Casella, Emily; Bean, Christopher J; Hooper, W Craig; DeBaun, Michael R; Garrett, Melanie E; Soldano, Karen; Telen, Marilyn J; Ashley-Koch, Allison; Gladwin, Mark T; Baldwin, Clinton T; Steinberg, Martin H; Klings, Elizabeth S

    2012-01-01

    Serum bilirubin levels have been associated with polymorphisms in the UGT1A1 promoter in normal populations and in patients with hemolytic anemias, including sickle cell anemia. When hemolysis occurs circulating heme increases, leading to elevated bilirubin levels and an increased incidence of cholelithiasis. We performed the first genome-wide association study (GWAS) of bilirubin levels and cholelithiasis risk in a discovery cohort of 1,117 sickle cell anemia patients. We found 15 single nucleotide polymorphisms (SNPs) associated with total bilirubin levels at the genome-wide significance level (p value <5 × 10(-8)). SNPs in UGT1A1, UGT1A3, UGT1A6, UGT1A8 and UGT1A10, different isoforms within the UGT1A locus, were identified (most significant rs887829, p = 9.08 × 10(-25)). All of these associations were validated in 4 independent sets of sickle cell anemia patients. We tested the association of the 15 SNPs with cholelithiasis in the discovery cohort and found a significant association (most significant p value 1.15 × 10(-4)). These results confirm that the UGT1A region is the major regulator of bilirubin metabolism in African Americans with sickle cell anemia, similar to what is observed in other ethnicities. PMID:22558097

  6. A Genome-Wide Association Study of Total Bilirubin and Cholelithiasis Risk in Sickle Cell Anemia

    PubMed Central

    Milton, Jacqueline N.; Sebastiani, Paola; Solovieff, Nadia; Hartley, Stephen W.; Bhatnagar, Pallav; Arking, Dan E.; Dworkis, Daniel A.; Casella, James F.; Barron-Casella, Emily; Bean, Christopher J.; Hooper, W. Craig; DeBaun, Michael R.; Garrett, Melanie E.; Soldano, Karen; Telen, Marilyn J.; Ashley-Koch, Allison; Gladwin, Mark T.; Baldwin, Clinton T.; Steinberg, Martin H.; Klings, Elizabeth S.

    2012-01-01

    Serum bilirubin levels have been associated with polymorphisms in the UGT1A1 promoter in normal populations and in patients with hemolytic anemias, including sickle cell anemia. When hemolysis occurs circulating heme increases, leading to elevated bilirubin levels and an increased incidence of cholelithiasis. We performed the first genome-wide association study (GWAS) of bilirubin levels and cholelithiasis risk in a discovery cohort of 1,117 sickle cell anemia patients. We found 15 single nucleotide polymorphisms (SNPs) associated with total bilirubin levels at the genome-wide significance level (p value <5×10?8). SNPs in UGT1A1, UGT1A3, UGT1A6, UGT1A8 and UGT1A10, different isoforms within the UGT1A locus, were identified (most significant rs887829, p?=?9.08×10?25). All of these associations were validated in 4 independent sets of sickle cell anemia patients. We tested the association of the 15 SNPs with cholelithiasis in the discovery cohort and found a significant association (most significant p value 1.15×10?4). These results confirm that the UGT1A region is the major regulator of bilirubin metabolism in African Americans with sickle cell anemia, similar to what is observed in other ethnicities. PMID:22558097

  7. Change in Serum Ferritin Concentration in Experimentally Induced Anemia of Chronic Inflammation in Dogs

    PubMed Central

    CHIKAZAWA, Seishiro; NAKAZAWA, Takafumi; HORI, Yasutomo; HOSHI, Fumio; KANAI, Kazutaka; ITO, Naoyuki; ORINO, Koichi; WATANABE, Kiyotaka; HIGUCHI, Seiichi

    2013-01-01

    ABSTRACT In veterinary medicine, hyperferritinemia is often observed in dogs with various diseases (e.g., histiocytic sarcoma and immune-mediated hemolytic anemia) without evidence of iron overload. The mechanism underlying hyperferritinemia development is not well understood. Anemia caused by inflammation is termed as anemia of chronic disease (ACD), and experimentally induced ACD is known to cause slight hyperferritinemia. However, almost all these studies were based on short-term acute inflammation. Hepcidin, a protein mainly produced by hepatocytes, is thought to be a key regulator in iron release from reticuloendothelial cells (RECs), and its expression is related to ACD. We hypothesized that in the case of long-term ACD, iron deposition in RECs increases through hepcidin, causing a diachronic increase in serum ferritin levels. In the present study, we used a canine model with repeated subcutaneous administration of turpentine oil every 3 days over a period of 42 days (15 injections) and induced long-term inflammatory conditions; furthermore, we evaluated the change in serum ferritin concentration. Hypoproliferative anemia, bone marrow iron deposition and hypoferremia, which are characteristic of ACD, were observed on administering the turpentine injections. Hepatic iron content, hepatic hepcidin mRNA expression and serum ferritin concentration increased during the early period after turpentine injection, but returned to normal levels later. These results show that experimentally induced long-term ACD caused hypoproliferative anemia without sustained increase in hepcidin expression and did not cause systemic iron overload. Thus, chronic inflammation may not contribute greatly to increase in hyperferritinemia. PMID:23803460

  8. Change in serum ferritin concentration in experimentally induced anemia of chronic inflammation in dogs.

    PubMed

    Chikazawa, Seishiro; Nakazawa, Takafumi; Hori, Yasutomo; Hoshi, Fumio; Kanai, Kazutaka; Ito, Naoyuki; Orino, Koichi; Watanabe, Kiyotaka; Higuchi, Seiichi

    2013-11-01

    In veterinary medicine, hyperferritinemia is often observed in dogs with various diseases (e.g., histiocytic sarcoma and immune-mediated hemolytic anemia) without evidence of iron overload. The mechanism underlying hyperferritinemia development is not well understood. Anemia caused by inflammation is termed as anemia of chronic disease (ACD), and experimentally induced ACD is known to cause slight hyperferritinemia. However, almost all these studies were based on short-term acute inflammation. Hepcidin, a protein mainly produced by hepatocytes, is thought to be a key regulator in iron release from reticuloendothelial cells (RECs), and its expression is related to ACD. We hypothesized that in the case of long-term ACD, iron deposition in RECs increases through hepcidin, causing a diachronic increase in serum ferritin levels. In the present study, we used a canine model with repeated subcutaneous administration of turpentine oil every 3 days over a period of 42 days (15 injections) and induced long-term inflammatory conditions; furthermore, we evaluated the change in serum ferritin concentration. Hypoproliferative anemia, bone marrow iron deposition and hypoferremia, which are characteristic of ACD, were observed on administering the turpentine injections. Hepatic iron content, hepatic hepcidin mRNA expression and serum ferritin concentration increased during the early period after turpentine injection, but returned to normal levels later. These results show that experimentally induced long-term ACD caused hypoproliferative anemia without sustained increase in hepcidin expression and did not cause systemic iron overload. Thus, chronic inflammation may not contribute greatly to increase in hyperferritinemia. PMID:23803460

  9. Fanconi anemia and the development of leukemia.

    PubMed

    Alter, Blanche P

    2014-01-01

    Fanconi anemia (FA) is a rare autosomal recessive cancer-prone inherited bone marrow failure syndrome, due to mutations in 16 genes, whose protein products collaborate in a DNA repair pathway. The major complications are aplastic anemia, acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), and specific solid tumors. A severe subset, due to mutations in FANCD1/BRCA2, has a cumulative incidence of cancer of 97% by age 7 years; the cancers are AML, brain tumors, and Wilms tumor; several patients have multiple events. Patients with the other genotypes (FANCA through FANCQ) have cumulative risks of more than 50% of marrow failure, 20% of AML, and 30% of solid tumors (usually head and neck or gynecologic squamous cell carcinoma), by age 40, and they too are at risk of multiple adverse events. Hematopoietic stem cell transplant may cure AML and MDS, and preemptive transplant may be appropriate, but its use is a complicated decision. PMID:25455269

  10. Iron-Deficiency Anemia (For Parents)

    MedlinePLUS

    ... high in iron. Kids or teens on a vegetarian diet also might not get enough iron, because iron ... Multivitamins with iron and changes to a child's diet can help, but usually are not enough on ... Anemia Word! Anemia About Anemia ...

  11. Who Is at Risk for Anemia?

    MedlinePLUS

    ... from the NHLBI on Twitter. Who Is at Risk for Anemia? Anemia is a common condition. It ... people have other medical conditions as well. Major Risk Factors Factors that raise your risk for anemia ...

  12. Anemia associated with chronic heart failure: current concepts

    PubMed Central

    Shah, Ravish; Agarwal, Anil K

    2013-01-01

    Anemia is a frequent comorbidity of heart failure and is associated with poor outcomes. Anemia in heart failure is considered to develop due to a complex interaction of iron deficiency, kidney disease, and cytokine production, although micronutrient insufficiency and blood loss may contribute. Currently, treatment of anemia of heart failure lacks clear targets and specific therapy is not defined. Intravenous iron use has been shown to benefit anemic as well as nonanemic patients with heart failure. Treatment with erythropoietin-stimulating agents has been considered alone or in combination with iron, but robust evidence to dictate clear guidelines is not currently available. Available and emerging new agents in the treatment of anemia of heart failure will need to be tested in randomized, controlled studies. PMID:23403618

  13. Hyperemic peripheral red marrow in a patient with sickle cell anemia demonstrated on Tc-99m labeled red blood cell venography

    SciTech Connect

    Heiden, R.A.; Locko, R.C.; Stent, T.R. (Columbia Univ. College of Physicians and Surgeons, New York, NY (USA))

    1991-03-01

    A 25-year-old gravid woman, homozygous for sickle cell anemia, with a history of recent deep venous thrombosis, was examined using Tc-99m labeled red blood cell venography for recurrent thrombosis. Although negative for thrombus, the study presented an unusual incidental finding: the patient's peripheral bone marrow was hyperemic in a distribution consistent with peripheral red bone marrow expansion. Such a pattern has not been documented before using this technique. This report supports other literature that has demonstrated hyperemia of peripheral red bone marrow in other hemolytic anemias. This finding may ultimately define an additional role of scintigraphy in assessing the pathophysiologic status of the sickle cell patient.

  14. Sickle Cell Anemia

    NSDL National Science Digital Library

    Debra L. Stamper

    2000-01-01

    In this case study on sickle cell anemia, students are introduced to some of the key researchers responsible for determining the molecular basis of the disease and learn about the functioning of erythrocytes as well as the notion that changes in the environment can influence the functioning of cells.  Students also become familiar with the process of osmosis and how it can influence the sickling of the erythrocytes.  Throughout the case, students must address experimental design questions. The case was designed for use in the first semester of an introductory majors biology course.

  15. FA (Fanconi Anemia) Family Newsletter

    MedlinePLUS

    Family Newsletter The Fanconi Anemia Research Fund publishes the FA Family Newsletter twice a year and mails it to all FA patients and their ... of note Dave Frohnmayer, Co-founder of the Fanconi Anemia Research Fund, has sadly passed away... learn more... ...

  16. Aplastic crisis as a complication of congenital dyserythropoietic anemia type II.

    PubMed

    Heimpel, H; Wilts, H; Hirschmann, W D; Hofmann, W K; Siciliano, R D; Steinke, B; Wechsler, J G

    2007-01-01

    A transient aplastic crisis (TAC) is a well-known complication in all types of chronic hemolytic anemia but only 2 cases of such an event were described in congenital dyserythropoietic anemias (CDAs). Here, we report a third case, and by retrospective chart review of 78 cases we found evidence of TAC in 8 further patients with CDA II, with serological evidence of previous human parvovirus B19 (B19V) infection in all but one. Although B19V infection results in TAC in only a minority of patients with CDA, physicians responsible for these patients should be aware of such a potentially life-threatening complication. Testing for B19V-specific IgG is recommended in patients with CDA to estimate the risk of a possible future aplastic crisis. PMID:17127819

  17. Cardio-renal anemia syndrome

    PubMed Central

    Efstratiadis, G; Konstantinou, D; Chytas, I; Vergoulas, G

    2008-01-01

    The interaction between cronic heart failure, cronic kidney insufficiency and anemia, form a vicious cycle, termed as the cardio-renal anemia syndrome. The interaction between these three conditions causes deterioration of the cardiac and renal function and increases anemia. Each of the three can cause or be caused by the others. We herein analyze and speculate the mechanisms involved in the pathophysiology of this new syndrome highlighting the main points of interest that seem to expand upon more than one specialty. The cardio-renal anemia syndrome is emerging in the area of clinical investigation with progressively elevated significance. Additionaly we report the data related to anemia treatment as part of therapeutic perspective concerning the management of patients manifesting the profile of this syndrome. PMID:18923761

  18. Hemolytic effects of water-soluble fullerene derivatives.

    PubMed

    Bosi, Susanna; Feruglio, Luigi; Da Ros, Tatiana; Spalluto, Giampiero; Gregoretti, Barbara; Terdoslavich, Michela; Decorti, Giuliana; Passamonti, Sabina; Moro, Stefano; Prato, Maurizio

    2004-12-30

    A series of water-soluble fullerene C(60) derivatives has been investigated for their cytotoxic and hemolytic properties, with the aim to correlate structure with toxicity. We observed that cationic chains induce significant toxicity while the presence of neutral or anionic moieties did not produce any response in our model. A validation of these experimental observations has been performed by theoretical studies in which hydrophilic and hydrophobic surface areas were correlated quantitatively with hemolytic properties. PMID:15615520

  19. A multicomponent hemolytic system in the pathogenic amoeba Naegleria fowleri.

    PubMed Central

    Lowrey, D M; McLaughlin, J

    1984-01-01

    A hemolytic activity associated with postnuclear supernatant fractions of Naegleria fowleri has been partially characterized in an attempt to isolate cytolytic molecules that may participate in naeglerial cytopathogenicity. Hemolysis by naeglerial postnuclear supernatant fractions was sensitive to heat and trypsin hydrolysis, and was inhibited by divalent cations. The majority of the hemolytic activity was nonlatent and associated with a particle fraction sedimenting at 48,000 X g (maximum) for 1 h. This particle-associated hemolytic activity appears to be membrane associated, as high salt concentration, chelating agents, and pH extremes were ineffective in solubilizing the hemolytic activity, whereas treatment with 0.15% Zwittergent 3-12, a dipolar ionic detergent, results in 98% release of the sedimentable hemolysin. The sigmoidal nature of the progress curve of postnuclear supernatant hemolysis, as well as synergistic interactions between fractions of amoebal whole cell extracts, suggests that the hemolytic activity has a multicomponent nature, with at least two and possibly three components participating in the hemolytic event. The significance of these findings in the context of naeglerial cytopathogenicity is discussed. PMID:6469359

  20. Managing Anemia of Chronic Kidney Disease

    Microsoft Academic Search

    Susan A. Krikorian

    2009-01-01

    Anemia begins early in the course of declining kidney function and is a frequent complication of chronic kidney disease. Both anemia and chronic kidney disease are underdiagnosed and undertreated. Anemia is associated with significantly increased risk of morbidity and mortality, including increased risks of left ventricular hypertrophy and heart failure. Although the detrimental effects of anemia are more common in

  1. Do You Know about Sickle Cell Anemia?

    MedlinePLUS

    Do You Know About Sickle Cell Anemia? KidsHealth > Kids > Health Problems > Blood > Do You Know About Sickle Cell Anemia? Print A A A Text Size What's in ... to stay in the hospital. What Causes Sickle Cell Anemia? Sickle cell anemia is an inherited (say: ...

  2. The Molecular Connection Between Aluminum Toxicity, Anemia,

    E-print Network

    Appanna, Vasu

    25 The Molecular Connection Between Aluminum Toxicity, Anemia, Inflammation and Obesity D. Appanna Laurentian University Canada 1. Introduction Anemia is reported to be the most common of the types of anemia has different underlying causes. Iron (Fe) deficiency, a potent instigator of anemia

  3. Mitochondrial Iron Metabolism and Sideroblastic Anemia

    Microsoft Academic Search

    Alex D. Sheftel; Des R. Richardson; Josef Prchal; Prem Ponka

    2009-01-01

    Sideroblastic anemias are a heterogeneous group of disorders, characterized by mitochondrial iron overload in developing red blood cells. The unifying characteristic of all sideroblastic anemias is the ring sideroblast, which is a pathological erythroid precursor containing excessive deposits of non-heme iron in mitochondria with perinuclear distribution creating a ring appearance. Sideroblastic anemias may be hereditary or acquired. Hereditary sideroblastic anemias

  4. Anemia of Inflammation and Chronic Disease

    MedlinePLUS

    ... Besarab A, Coyne DW. Iron supplementation to treat anemia in patients with chronic kidney disease. Nature Reviews Nephrology . 2010;6(12):699–710. ... Anemia in Kidney Disease and Dialysis at www.kidney.niddk.nih.gov. 4 Anemia of Inflammation ... Eating, Diet, and Nutrition People with anemia caused ...

  5. Prenatal Genotyping of the RhD Locus to Identify Fetuses at Risk for Hemolytic Disease of the Newborn.

    PubMed

    Hessner, M J; Bellissimo, D B

    2001-01-01

    Hemolytic disease of the newborn (HDN) can occur when there are fetomaternal incompatibilities within any number of different erythrocyte antigen systems, including the RhD, Cc, Ee, Kidd and Duffy, and Kell antigen systems. In these disorders, maternal antibodies are developed by alloimmunization of the mother to fetal red blood cells during pregnancy when the fetal cells carry an alloantigen inherited from the father. The maternal antibodies result in the destruction of fetal erythrocytes leading to severe hemolytic anemia and hyperbilirubinemia. Permanent neurologic damage can result from HDN, and in extreme cases loss of the fetus or death of the neonate may occur. In subsequent pregnancies, it is important to determine the status of the incompatible allele in the fetus. If the father is heterozygous or homozygous for the allele, the chance of the fetus inheriting the paternal alloallele to which the mother is immunologically sensitized is 50 or 100%, respectively. Fetuses that do not inherit the alloallele will not be at risk for HDN. PMID:21370158

  6. Quiescent complement in nonhuman primates during E coli Shiga toxin-induced hemolytic uremic syndrome and thrombotic microangiopathy

    PubMed Central

    Lee, Benjamin C.; Mayer, Chad L.; Leibowitz, Caitlin S.

    2013-01-01

    Enterohemorrhagic Escherichia coli (EHEC) produce ribosome-inactivating Shiga toxins (Stx1, Stx2) responsible for development of hemolytic uremic syndrome (HUS) and acute kidney injury (AKI). Some patients show complement activation during EHEC infection, raising the possibility of therapeutic targeting of complement for relief. Our juvenile nonhuman primate (Papio baboons) models of endotoxin-free Stx challenge exhibit full spectrum HUS, including thrombocytopenia, hemolytic anemia, and AKI with glomerular thrombotic microangiopathy. There were no significant increases in soluble terminal complement complex (C5b-9) levels after challenge with lethal Stx1 (n = 6) or Stx2 (n = 5) in plasma samples from T0 to euthanasia at 49.5 to 128 hours post-challenge. d-dimer and cell injury markers (HMGB1, histones) confirmed coagulopathy and cell injury. Thus, complement activation is not required for the development of thrombotic microangiopathy and HUS induced by EHEC Shiga toxins in these preclinical models, and benefits or risks of complement inhibition should be studied further for this infection. PMID:23733336

  7. Iron-deficiency anemia in Castleman disease: implication of the interleukin 6/hepcidin pathway.

    PubMed

    Arlet, Jean-Benoît; Hermine, Olivier; Darnige, Luc; Ostland, Vaughn; Westerman, Mark; Badoual, Cécile; Pouchot, Jacques; Capron, Loïc

    2010-12-01

    In addition to occasional autoimmune hemolytic anemia, unexplained iron-deficiency anemia has been reported in childhood Castleman disease (CD). The recent discovery of hepcidin has regenerated the research on iron metabolism. This hormone is a key regulator of iron homeostasis, mainly by inhibiting intestinal iron absorption. Liver expression of hepcidin increases in response to interleukin 6 (IL-6). With chronic overproduction of IL-6 as a hallmark, CD could be an interesting human model for studying the contribution of the IL-6/hepcidin pathway in the pathogenesis of anemia of chronic disease. We report here the case of a 16-year-old boy with chronic iron-deficiency anemia (plasma ferritin: 19 ?g/L; plasma iron: 2.2 ?mol/L; negative bone marrow Perls' Prussian blue stain), inflammatory syndrome (C-reactive protein: 108 mg/L), and growth retardation for the previous 2 years. Diagnostic workup revealed a large mesenteric mass corresponding to localized CD of mixed histologic type. Resection of the tumor resulted in complete resolution of iron-deficiency anemia and inflammatory syndrome. Parallel variations of plasma IL-6, C-reactive protein, and hepcidin concentrations, together with tumor immunohistochemistry, strongly suggested that IL-6 synthesized by the tumor caused both the inflammation and iron deficiency through enhancement of hepcidin production by the liver. The results of this unique case study (1) explain the mechanism of iron deficiency observed in some children with CD, (2) confirm in vivo the regulatory effect of IL-6 in human hepcidin production, and (3) suggest that iron deficiency is a causal link between IL-6 and anemia of chronic disease. PMID:21041280

  8. How Is Aplastic Anemia Treated?

    MedlinePLUS

    ... need for blood transfusions. Medicines To Suppress the Immune System Research suggests that aplastic anemia may sometimes occur because the body's immune system attacks its own cells by mistake. For this ...

  9. Current treatment of atypical hemolytic uremic syndrome

    PubMed Central

    Kaplan, Bernard S.; Ruebner, Rebecca L.; Spinale, Joann M.; Copelovitch, Lawrence

    2014-01-01

    Summary Tremendous advances have been made in understanding the pathogenesis of atypical Hemolytic Uremic Syndrome (aHUS), an extremely rare disease. Insights into the molecular biology of aHUS resulted in rapid advances in treatment with eculizumab (Soliris®, Alexion Pharmaceuticals Inc.). Historically, aHUS was associated with very high rates of mortality and morbidity. Prior therapies included plasma therapy and/or liver transplantation. Although often life saving, these were imperfect and had many complications. We review the conditions included under the rubric of aHUS: S. pneumoniae HUS (SpHUS), inborn errors of metabolism, and disorders of complement regulation, emphasizing their differences and similarities. We focus on the clinical features, diagnosis, and pathogenesis, and treatment of aHUS that results from mutations in genes encoding alternative complement regulators, SpHUS and HUS associated with inborn errors of metabolism. Mutations in complement genes, or antibodies to their protein products, result in unregulated activity of the alternate complement pathway, endothelial injury, and thrombotic microangiopathy (TMA). Eculizumab is a humanized monoclonal antibody that inhibits the production of the terminal complement components C5a and the membrane attack complex (C5b-9) by binding to complement protein C5a. This blocks the proinflammatory and cytolytic effects of terminal complement activation. Eculizumab use has been reported in many case reports, and retrospective and prospective clinical trials in aHUS. There have been few serious side effects and no reports of tachphylaxis or drug resistance. The results are very encouraging and eculizumab is now recognized as the treatment of choice for aHUS. PMID:25343125

  10. X-linked Sideroblastic Anemia Due to Carboxyl-terminal ALAS2 Mutations That Cause Loss of Binding to the ?-Subunit of Succinyl-CoA Synthetase (SUCLA2)*

    PubMed Central

    Bishop, David F.; Tchaikovskii, Vassili; Hoffbrand, A. Victor; Fraser, Marie E.; Margolis, Steven

    2012-01-01

    Mutations in the erythroid-specific aminolevulinic acid synthase gene (ALAS2) cause X-linked sideroblastic anemia (XLSA) by reducing mitochondrial enzymatic activity. Surprisingly, a patient with the classic XLSA phenotype had a novel exon 11 mutation encoding a recombinant enzyme (p.Met567Val) with normal activity, kinetics, and stability. Similarly, both an expressed adjacent XLSA mutation, p.Ser568Gly, and a mutation (p.Phe557Ter) lacking the 31 carboxyl-terminal residues also had normal or enhanced activity, kinetics, and stability. Because ALAS2 binds to the ? subunit of succinyl-CoA synthetase (SUCLA2), the mutant proteins were tested for their ability to bind to this protein. Wild type ALAS2 bound strongly to a SUCLA2 affinity column, but the adjacent XLSA mutant enzymes and the truncated mutant did not bind. In contrast, vitamin B6-responsive XLSA mutations p.Arg452Cys and p.Arg452His, with normal in vitro enzyme activity and stability, did not interfere with binding to SUCLA2 but instead had loss of positive cooperativity for succinyl-CoA binding, an increased Km for succinyl-CoA, and reduced vitamin B6 affinity. Consistent with the association of SUCLA2 binding with in vivo ALAS2 activity, the p.Met567GlufsX2 mutant protein that causes X-linked protoporphyria bound strongly to SUCLA2, highlighting the probable role of an ALAS2-succinyl-CoA synthetase complex in the regulation of erythroid heme biosynthesis. PMID:22740690

  11. Malignant nephrosclerosis during pregnancy and in the postpartum period (the uremic hemolytic syndrome).

    PubMed

    Beller, F K; Intorp, H W; Losse, H; Loew, H; Moenninghoff, W; Schmidt, E H; Grundmann, E

    1976-07-01

    Histologic, immunohistologic, and ultrastructural features are presented of two cases with malignant nephrosclerosis during pregnancy. Primary malignant nephrosclerosis emerges as a clinical entity which can be distinguished from toxemia of pregnancy in the midtrimester and post partum. The first description of malignant nephrosclerosis dates from 40 years ago, but only a few cases were reported associated with pregnancy. Although disseminated intravascular coagulation seems involved, the morphology is different from that of toxemia. Malignant nephrosclerosis reveals a close similarity to the hemolytic uremic syndrome. Early diagnosis by renal biopsy and proper treatment may prevent a lethal outcome due to progressive failure. PMID:779474

  12. 78 FR 79469 - Strategies To Address Hemolytic Complications of Immune Globulin Infusions; Public Workshop

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-30

    ...Hemolytic Complications of Immune Globulin Infusions; Public Workshop AGENCY: Food and Drug...Hemolytic Complications of Immune Globulin Infusions.'' The purpose of the public workshop...Globulin Intravenous (IGIV) (Human) infusion. Complications of hemolysis...

  13. Hydroxyurea and erythropoietin therapy in sickle cell anemia.

    PubMed

    Goldberg, M A; Brugnara, C; Dover, G J; Schapira, L; Lacroix, L; Bunn, H F

    1992-06-01

    Hydroxyurea has been shown to increase fetal hemoglobin (Hb F) production in patients with sickle cell disease and therefore has the potential to alleviate both the hemolytic and vaso-occlusive manifestations of the disease. Preliminary evidence indicates that recombinant human erythropoietin (rhEpo) may also induce Hb F. Three sickle cell anemia patients were treated with escalating doses of intravenous rhEpo and, subsequently, with daily oral hydroxyurea. After the optimal hydroxyurea dose was attained, rhEpo was added again. Two additional patients were treated with hydroxyurea alone. Treatment with rhEp, either alone or in combination with hydroxyurea, had no significant effect on the percentage of F reticulocytes or F cells. In contrast, hydroxyurea treatment was associated with a 1.5-fold to sevenfold increase in F cells and a 2.3- to 27-fold increase in the percentage of Hb F. In the three patients whose response reached a plateau, hydroxyurea treatment was associated with lessened hemolysis, decreased serum bilirubin and lactate dehydrogenase levels, and prolonged 51chromium-labeled RBC survival. Hydroxyurea treatment also resulted in decreased numbers of irreversibly sickled cells and in decreased sickling at partial oxygen saturation, increased oxygen affinity, increased total RBC cation content, and diminished potassium:chloride co-transport. All five patients treated with hydroxyurea experienced a decrease in severity and frequency of painful sickle crises. This study confirms that hydroxyurea therapy increases Hb F production and provides objective evidence of a significant reduction in hemolytic rate and intracellular polymerization. In contrast, rhEpo, either alone or in combination with hydroxyurea, offered no measurable benefit. Based on these encouraging preliminary data, large-scale, controlled clinical trials are warranted to study the safety and efficacy of hydroxyurea in the treatment of sickle cell disease. PMID:1379376

  14. Necrotizing tonsillitis caused by group C beta-hemolytic streptococci.

    PubMed

    Bastaki, Jassem M

    2015-03-01

    Tonsillitis and pharyngitis are among the most common infections in the head and neck. Viral tonsillitis is usually caused by enterovirus, influenza, parainfluenza, adenovirus, rhinovirus and Epstein-Barr virus (causing infectious mononucleosis). Acute bacterial tonsillitis is most commonly caused by group A beta-hemolytic streptococci. On the other hand, pseudomembranous and necrotizing tonsillitis are usually caused by fusiform bacilli and spirochetes. Here we report what is, to our knowledge, the first case of necrotizing tonsillitis caused by group C beta-hemolytic streptococci. PMID:25738719

  15. Issues in prevention of iron deficiency anemia in India.

    PubMed

    Anand, Tanu; Rahi, Manju; Sharma, Pragya; Ingle, Gopal K

    2014-01-01

    Iron deficiency anemia (IDA) continues to be major public health problem in India. It is estimated that about 20% of maternal deaths are directly related to anemia and another 50% of maternal deaths are associated with it. The question, therefore, is why, despite being the first country to launch the National Nutritional Anemia Prophylaxis Programme in 1970, the problem of IDA remains so widespread. As is to be expected, the economic implications of IDA are also massive. The issues of control of IDA in India are multiple. Inadequate dietary intake of iron, defective iron absorption, increased iron requirements due to repeated pregnancies and lactation, poor iron reserves at birth, timing of umbilical cord clamping, timing and type of complementary food introduction, frequency of infections in children, and excessive physiological blood loss during adolescence and pregnancy are some of the causes responsible for the high prevalence of anemia in India. In addition, there are other multiple programmatic and organizational issues. This review, therefore, is an attempt to examine the current burden of anemia in India, its epidemiology, and the various issues regarding its prevention and control, as well as to offer some innovative approaches to deal with this major health problem. PMID:24984990

  16. Anemia - Multiple Languages: MedlinePlus

    MedlinePLUS

    ... enable JavaScript. Anemia - Multiple Languages Arabic (???????) Bosnian (Bosanski) Chinese - Simplified (????) French (français) Hindi (??????) ... Arabic) ??????? Bilingual PDF Health Information Translations Bosnian (Bosanski) Anemia Anemija - Bosanski (Bosnian) Bilingual PDF Health Information ...

  17. How Is Iron-Deficiency Anemia Treated?

    MedlinePLUS

    ... page from the NHLBI on Twitter. How Is Iron-Deficiency Anemia Treated? Treatment for iron-deficiency anemia ... cells, hemoglobin, and iron. Dietary Changes and Supplements Iron You may need iron supplements to build up ...

  18. Anemia of Inflammation and Chronic Disease

    MedlinePLUS

    ... Besarab A, Coyne DW. Iron supplementation to treat anemia in patients with chronic kidney disease. Nature Reviews Nephrology. 2010;6(12):699–710. [ Top ] How is AI/ACD treated? Anemia of inflammation and chronic disease often is not ...

  19. A hemolytic factor from Haemonchus contortus alters erythrocyte morphology.

    PubMed

    Fetterer, R H; Rhoads, M L

    1998-12-15

    A hemolytic factor from adult Haemonchus contortus caused distinct morphological changes in the surface of sheep red blood cells (RBCs). After a 15 min exposure to the hemolytic factor, hemolysis was not detected in incubation media, but RBCs were spherical in shape with numerous surface projections compared to control cells that were smooth-surfaced biconcave disks. After 30 min, a time at which significant hemolysis occurred, echinocytes were formed, and after 90 min, cells were severely disrupted with many visible holes in membranes. No RBC ghosts were observed. RBCs from four other mammalian species were lysed by the H. contortus hemolytic factor. However, the rate of hemolysis varied with a relative order of sheep approximately rabbit>goat>pig>calf. The morphology of RBCs from all four species was significantly altered after 30 min incubation with the degree of morphological changes related to the degree of hemolysis. These results support the hypothesis that the hemolytic factor acts as a pore-forming agent, although a phospholipase or other enzyme might play a role in solubilization of cell membranes. PMID:9877069

  20. A hemolytic pigment of Group B Streptococcus allows bacterial penetration of human placenta

    PubMed Central

    Whidbey, Christopher; Harrell, Maria Isabel; Burnside, Kellie; Ngo, Lisa; Becraft, Alexis K.; Iyer, Lakshminarayan M.; Aravind, L.; Hitti, Jane

    2013-01-01

    Microbial infection of the amniotic fluid is a significant cause of fetal injury, preterm birth, and newborn infections. Group B Streptococcus (GBS) is an important human bacterial pathogen associated with preterm birth, fetal injury, and neonatal mortality. Although GBS has been isolated from amniotic fluid of women in preterm labor, mechanisms of in utero infection remain unknown. Previous studies indicated that GBS are unable to invade human amniotic epithelial cells (hAECs), which represent the last barrier to the amniotic cavity and fetus. We show that GBS invades hAECs and strains lacking the hemolysin repressor CovR/S accelerate amniotic barrier failure and penetrate chorioamniotic membranes in a hemolysin-dependent manner. Clinical GBS isolates obtained from women in preterm labor are hyperhemolytic and some are associated with covR/S mutations. We demonstrate for the first time that hemolytic and cytolytic activity of GBS is due to the ornithine rhamnolipid pigment and not due to a pore-forming protein toxin. Our studies emphasize the importance of the hemolytic GBS pigment in ascending infection and fetal injury. PMID:23712433

  1. A hemolytic pigment of Group B Streptococcus allows bacterial penetration of human placenta.

    PubMed

    Whidbey, Christopher; Harrell, Maria Isabel; Burnside, Kellie; Ngo, Lisa; Becraft, Alexis K; Iyer, Lakshminarayan M; Aravind, L; Hitti, Jane; Waldorf, Kristina M Adams; Rajagopal, Lakshmi

    2013-06-01

    Microbial infection of the amniotic fluid is a significant cause of fetal injury, preterm birth, and newborn infections. Group B Streptococcus (GBS) is an important human bacterial pathogen associated with preterm birth, fetal injury, and neonatal mortality. Although GBS has been isolated from amniotic fluid of women in preterm labor, mechanisms of in utero infection remain unknown. Previous studies indicated that GBS are unable to invade human amniotic epithelial cells (hAECs), which represent the last barrier to the amniotic cavity and fetus. We show that GBS invades hAECs and strains lacking the hemolysin repressor CovR/S accelerate amniotic barrier failure and penetrate chorioamniotic membranes in a hemolysin-dependent manner. Clinical GBS isolates obtained from women in preterm labor are hyperhemolytic and some are associated with covR/S mutations. We demonstrate for the first time that hemolytic and cytolytic activity of GBS is due to the ornithine rhamnolipid pigment and not due to a pore-forming protein toxin. Our studies emphasize the importance of the hemolytic GBS pigment in ascending infection and fetal injury. PMID:23712433

  2. Erythropoietic protoporphyria in the house mouse. A recessive inherited ferrochelatase deficiency with anemia, photosensitivity, and liver disease.

    PubMed Central

    Tutois, S; Montagutelli, X; Da Silva, V; Jouault, H; Rouyer-Fessard, P; Leroy-Viard, K; Guénet, J L; Nordmann, Y; Beuzard, Y; Deybach, J C

    1991-01-01

    A viable autosomal recessive mutation (named fch, or ferrochelatase deficiency) causing jaundice and anemia in mice arose in a mutagenesis experiment using ethylnitrosourea. Homozygotes (fch/fch) display a hemolytic anemia, photosensitivity, cholestasis, and severe hepatic dysfunction. Protoporphyrin is found at high concentration in erythrocytes, serum, and liver. Ferrochelatase activity in various tissues is 2.7-6.3% of normal. Heterozygotes (+/fch) are not anemic and have normal liver function; they are not sensitive to light exposure; ferrochelatase activity is 45-65% of normal. Southern blot analysis using a ferrochelatase cDNA probe reveals no gross deletion of the ferrochelatase gene. This is the first spontaneous form of erythropoietic protoporphyria in the house mouse. Despite the presence in the mouse of clinical and biochemical features infrequent in the human, this mutation may represent a model for the human disease, especially in its severe form. Images PMID:1939658

  3. Hemolytic activity of dermatophytes species isolated from clinical specimens.

    PubMed

    Aktas, E; Y?g?t, N

    2015-03-01

    Hemolytic activity was recently reported for several pathogenic fungal species, such as Aspergillus, Candida, Trichophyton, Penicillium and Fusarium. Based on a number of mechanistic and characterization studies, several fungal hemolysins have been proposed as virulence factors. Hemolysins lyse red blood cells resulting in the release of iron, an important growth factor for microbes especially during infection. The requirement of iron in fungal growth is necessary for metabolic processes and as a catalyst for various biochemical processes. Expression of a hemolytic protein with capabilities to lyse red blood cells has also been suggested to provide a survival strategy for fungi during opportunistic infections. The aims of this study were to investigate the hemolytic activities of dermatophytes species isolated from patients with dermatophytosis. Hair, skin and nail samples of patients were examined with direct microscopy using potassium hydroxide and cultivated on Mycobiotic agar and Sabouraud's dextrose agar. To determine hemolytic activities of dermatophytes species, they were subcultured on Columbia Agar with 5% sheep blood and incubated for 7-14 days at 25°C in aerobic conditions. Media which displayed hemolysis were further incubated for 1-5 days at 37°C to increase hemolytic activity. In this study, 66 dermatophytes strains were isolated from clinical specimens and were identified by six different species: 43 (65.1%) Trichophyton rubrum, 7 (10.7%) Trichophyton mentagrophytes, 5 (7.6%) Microsporum canis, 5 (7.6%) Trichophyton tonsurans, 4 (6.0%) Epidermophyton floccosum and 2 (3.0%) Trichophyton violaceum. Twenty-one T. rubrum strains showed incomplete (alpha) hemolysis and nine T. rubrum strains showed complete (beta) hemolysis, whereas hemolysis was absent in 13 T. rubrum strains. Four T. mentagrophytes strains showed complete hemolysis and three T. tonsurans strains showed incomplete hemolysis. However, M. canis, E. floccosum and T. violaceum species had no hemolytic activity. Hemolytic activity is pronounced in dermatophytes and may play an important role as a virulence factor. Hemolysins produced may play an important role in the balance between the host's cellular immunity and the ability of the fungus to diminish the immune response. PMID:25467819

  4. Recurrent Syncope Due to Refractory Cerebral Venous Sinus Thrombosis and Transient Elevations of Intracranial Pressure

    PubMed Central

    Larimer, P.; McDermott, M.W.; Scott, B.J.; Shih, T.T.; Poisson, S.N.

    2014-01-01

    Chronic paroxysmal intracranial hypertension leading to syncope is a phenomenon not reported previously in patients with refractory cerebral venous sinus thrombosis. We report a case of paroxysmal intracranial hypertension leading to syncopal episodes in a patient with idiopathic autoimmune hemolytic anemia and venous sinus thrombosis. This case demonstrates that intermittent elevations in intracranial pressure can lead to syncope in patients with venous sinus thrombosis and emphasizes the importance of considering this potentially treatable etiology of syncopal episodes. PMID:24381706

  5. Anemia in inflammatory bowel disease: A neglected issue with relevant effects

    PubMed Central

    Guagnozzi, Danila; Lucendo, Alfredo J

    2014-01-01

    Anemia, a common complication associated with inflammatory bowel disease (IBD), is frequently overlooked in the management of IBD patients. Unfortunately, it represents one of the major causes of both decreased quality of life and increased hospital admissions among this population. Anemia in IBD is pathogenically complex, with several factors contributing to its development. While iron deficiency is the most common cause, vitamin B12 and folic acid deficiencies, along with the effects of pro-inflammatory cytokines, hemolysis, drug therapies, and myelosuppression, have also been identified as the underlying etiology in a number of patients. Each of these etiological factors thus needs to be identified and corrected in order to effectively manage anemia in IBD. Because the diagnosis of anemia in IBD often presents a challenge, combinations of several hematimetric and biochemical parameters should be used. Recent studies underscore the importance of determining the ferritin index and hepcidin levels in order to distinguish between iron deficiency anemia, anemia due to chronic disease, or mixed anemia in IBD patients. With regard to treatment, the newly introduced intravenous iron formulations have several advantages over orally-administered iron compounds in treating iron deficiency in IBD. In special situations, erythropoietin supplementation and biological therapies should be considered. In conclusion, the management of anemia is a complex aspect of treating IBD patients, one that significantly influences the prognosis of the disease. As a consequence, its correction should be considered a specific, first-line therapeutic goal in the management of these patients. PMID:24707137

  6. Feline Nonregenerative Anemia: Diagnosis and Treatment

    Microsoft Academic Search

    Carrie White; Nyssa Reine

    Anemia in cats is not a diagnosis but rather a sign of an underlying disease. The diagnos- tic work-up for an anemic patient is often extensive, starting with classification of the anemia as re- generative or nonregenerative. Once nonregenerative anemia is diagnosed, a number of tests may be required to determine its cause, including a close examination of the patient's

  7. Marzo de 2012 Lucha contra la anemia

    E-print Network

    N° 399 Marzo de 2012 Lucha contra la anemia: una estrategia más eficaz Scientific news Actualidad cientifica Actualité scientifique La carencia de hierro y la anemia1 que ésta puede provocar constituyen un. África y la India registran los mayores índices de anemia, con casi un 50% de las mujeres afectadas. En

  8. (Inborn anemias of mice): Terminal progress report

    SciTech Connect

    Bernstein, S.E.

    1987-01-01

    Mutations located at 11 different chromosomal locations in the mouse all affecting hemopoiesis have been studied. These include: Hertwig's anemia (an), W-anemias (W, W/sup v/, W/sup 17J/ to W/sup 41J/), Steel anemias (Sl, Sl/sup d/, etc.), Normoblastic anemia (nb), Jaundiced (ja), Spherocytic anemias (sph, sph/sup ha/), sph/sup 2J/, sph/sup 2BC/, Flexed-tail anemia (f), Microcytic anemia (mk), Sex-linked anemia (Sla), Alpha thallasemia (Hba/sup th/), and a hypochromic anemia associated with low transferrin levels (hpx). Our findings indicate that the erythroid defect in W-anemias stem from an intrinsic defect in the erythroid progenitor cells, and that all other erythroid hemostatic mechanisms are fully functional. Hertwig's anemia (an) is affected in a similar fashion. However, in the case of Steel anemias, the erythroid progenitors are repressed, but when transplanted to appropriate recipients were found to be fully functional. 70 refs., 4 tabs.

  9. Erythroblast transferrin receptors and transferrin kinetics in iron deficiency and various anemias

    SciTech Connect

    Muta, K.; Nishimura, J.; Ideguchi, H.; Umemura, T.; Ibayashi, H.

    1987-06-01

    To clarify the role of transferrin receptors in cases of altered iron metabolism in clinical pathological conditions, we studied: number of binding sites; affinity; and recycling kinetics of transferrin receptors on human erythroblasts. Since transferrin receptors are mainly present on erythroblasts, the number of surface transferrin receptors was determined by assay of binding of /sup 125/I-transferrin and the percentage of erythroblasts in bone marrow mononuclear cells. The number of binding sites on erythroblasts from patients with an iron deficiency anemia was significantly greater than in normal subjects. Among those with an aplastic anemia, hemolytic anemia, myelodysplastic syndrome, and polycythemia vera compared to normal subjects, there were no considerable differences in the numbers of binding sites. The dissociation constants (Kd) were measured using Scatchard analysis. The apparent Kd was unchanged (about 10 nmol/L) in patients and normal subjects. The kinetics of endocytosis and exocytosis of /sup 125/I-transferrin, examined by acid treatment, revealed no variations in recycling kinetics among the patients and normal subjects. These data suggest that iron uptake is regulated by modulation of the number of surface transferrin receptors, thereby reflecting the iron demand of the erythroblast.

  10. Perioperative anemia management in colorectal cancer patients: a pragmatic approach.

    PubMed

    Muñoz, Manuel; Gómez-Ramírez, Susana; Martín-Montañez, Elisa; Auerbach, Michael

    2014-02-28

    Anemia, usually due to iron deficiency, is highly prevalent among patients with colorectal cancer. Inflammatory cytokines lead to iron restricted erythropoiesis further decreasing iron availability and impairing iron utilization. Preoperative anemia predicts for decreased survival. Allogeneic blood transfusion is widely used to correct anemia and is associated with poorer surgical outcomes, increased post-operative nosocomial infections, longer hospital stays, increased rates of cancer recurrence and perioperative venous thromboembolism. Infections are more likely to occur in those with low preoperative serum ferritin level compared to those with normal levels. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management, minimizes or eliminates allogeneic blood transfusion. This includes restrictive transfusion policy, thromboprophylaxis and anemia management to improve outcomes. Normalization of preoperative hemoglobin levels is a World Health Organization recommendation. Iron repletion should be routinely ordered when indicated. Oral iron is poorly tolerated with low adherence based on published evidence. Intravenous iron is safe and effective but is frequently avoided due to misinformation and misinterpretation concerning the incidence and clinical nature of minor infusion reactions. Serious adverse events with intravenous iron are extremely rare. Newer formulations allow complete replacement dosing in 15-60 min markedly facilitating care. Erythropoiesis stimulating agents may improve response rates. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management used to minimize or eliminate allogeneic blood transfusion is indicated to improve outcomes. PMID:24587673

  11. Perioperative anemia management in colorectal cancer patients: A pragmatic approach

    PubMed Central

    Muñoz, Manuel; Gómez-Ramírez, Susana; Martín-Montañez, Elisa; Auerbach, Michael

    2014-01-01

    Anemia, usually due to iron deficiency, is highly prevalent among patients with colorectal cancer. Inflammatory cytokines lead to iron restricted erythropoiesis further decreasing iron availability and impairing iron utilization. Preoperative anemia predicts for decreased survival. Allogeneic blood transfusion is widely used to correct anemia and is associated with poorer surgical outcomes, increased post-operative nosocomial infections, longer hospital stays, increased rates of cancer recurrence and perioperative venous thromboembolism. Infections are more likely to occur in those with low preoperative serum ferritin level compared to those with normal levels. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management, minimizes or eliminates allogeneic blood transfusion. This includes restrictive transfusion policy, thromboprophylaxis and anemia management to improve outcomes. Normalization of preoperative hemoglobin levels is a World Health Organization recommendation. Iron repletion should be routinely ordered when indicated. Oral iron is poorly tolerated with low adherence based on published evidence. Intravenous iron is safe and effective but is frequently avoided due to misinformation and misinterpretation concerning the incidence and clinical nature of minor infusion reactions. Serious adverse events with intravenous iron are extremely rare. Newer formulations allow complete replacement dosing in 15-60 min markedly facilitating care. Erythropoiesis stimulating agents may improve response rates. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management used to minimize or eliminate allogeneic blood transfusion is indicated to improve outcomes. PMID:24587673

  12. Human fetal bilirubin levels and fetal hemolytic disease.

    PubMed

    Weiner, C P

    1992-05-01

    The development of secondary fetal anemia in association with maternal red blood cell alloimmunization requires hemolysis. In specimens obtained at the time of a clinically indicated cordocentesis, total and direct umbilical venous bilirubin was measured and the indirect umbilical venous bilirubin calculated in 43 antigen-positive and 30 control fetuses. Twenty-two (51%) of the antigen-positive fetuses had or subsequently developed severe anemia (hematocrit less than 30%). Umbilical venous total bilirubin (r = 0.47, p = 0.0008) and direct bilirubin (r = 0.520, p = 0.04) levels each rose with gestation. Indirect bilirubin did not vary significantly with gestation. Bilirubin was unrelated to hemoglobin. In contrast to the control fetuses, umbilical venous total bilirubin for antigen-positive fetuses was inversely related to hemoglobin (r = -0.57, p less than 0.0001) independent of gestational age (r = 0.53, p less than 0.0001) (multiple R of hemoglobin and gestational age for umbilical venous total bilirubin = 0.76, p less than 0.0001). Eighteen of 22 (82%) fetuses in whom anemia developed had an umbilical venous total bilirubin greater than or equal to 97.5 percentile compared with only eight of 21 (38%) fetuses in whom anemia did not develop (p = 0.009). In longitudinal study the umbilical venous total bilirubin frequently rose above normal weeks before the development of anemia. An umbilical venous total bilirubin greater than 3 mg/dl represented the warning line. Fifteen of 16 (94%) fetuses in whom either severe antenatal anemia or significant postnatal hyperbilirubinemia developed had an umbilical venous total bilirubin greater than 3 mg/dl. We conclude that the normal placental capacity for the transport of fetal bilirubin is exceeded in the face of enhanced fetal hemolysis. An elevated fetal bilirubin often precedes the development of antenatal anemia. The antigen-positive fetus with an elevated bilirubin is at high risk to develop anemia antenatally. PMID:1595799

  13. Aplastic Anemia and Myelodysplastic Syndromes

    MedlinePLUS

    ... and sending the sample to a lab for analysis. A complete blood count is usually the first test a health care provider uses to detect aplastic anemia or MDS. The test includes measurement of a person’s hematocrit, the percentage of the blood that consists of red blood ...

  14. Erythema nodosum and pernicious anemia.

    PubMed

    Milman, Perry J; Goldenberg, Steven P; Scheinfeld, Noah; Pereira, Frederick A

    2013-07-01

    Erythema nodosum (EN) often presents as a sudden onset of tender, erythematous, subcutaneous nodules on the legs and ankles. Although rare, pernicious anemia may be related to vitamin B12 deficiency. Discussion of this association in the context of a particular patient is presented. PMID:24010520

  15. Deafferentation anemia in splenectomized animals

    Microsoft Academic Search

    M. I. Pekarskii

    1970-01-01

    Experiments on cats have shown that the reaction of the blood and bone marrow to division of the brachial plexus is the same in intact and splenectomized animals. This reaction consists of the development of anemia and neutrophilic leukocytosis, as the result of myeloid metaplasia of the bone marrow, which regularly develops in response to a focus of deafferentation in

  16. Impacts of mesoporous silica nanoparticle size, pore ordering, and pore integrity on hemolytic activity.

    PubMed

    Lin, Yu-Shen; Haynes, Christy L

    2010-04-01

    This paper uses the measure of hemolysis to evaluate the toxicity of nonporous and porous silica nanoparticles with varied sizes and investigates the effects of porous structure and integrity on the nanoparticle-cell interaction. The results show that both nonporous and porous silica cause red blood cell membrane damage in a concentration- and size-dependent manner. In the case of mesoporous silica nanoparticles, the size-dependent hemolysis effect is only present when the nanoparticles have long-range ordered porous structure, revealing that pore structure is critical in cell-nanoparticle interactions. Mesoporous silica nanoparticles show lower hemolytic activity than their nonporous counterparts of similar size, likely due to fewer silanol groups on the cell-contactable surface of the porous silica nanoparticles. The extent of hemolysis by mesoporous silica nanoparticles increases as the pore structure is compromised by mild aging in phosphate-buffered solutions, initiating mesopore collapse. The pore integrity of mesoporous silica nanoparticles is examined by TEM, XRD, N(2) adsorption-desorption isotherms, and quantification of dissolved silica. In these nanoparticles, pore stability is clearly an important factor in determining the hemolytic activity; further work demonstrates that nanoparticle-induced hemolysis can be eliminated by modifying the silanol surface with a poly(ethylene glycol) coating. PMID:20230032

  17. Anemia prevalence and treatment practice in patients with non-myeloid tumors receiving chemotherapy

    PubMed Central

    Merlini, Laura; Cartenì, Giacomo; Iacobelli, Stefano; Stelitano, Caterina; Airoldi, Mario; Balcke, Peter; Keil, Felix; Haslbauer, Ferdinand; Belton, Laura; Pujol, Beatriz

    2013-01-01

    Purpose To describe the prevalence and management of anemia in cancer patients. Methods This cross-sectional, observational survey was conducted in Italy and Austria. Centers prespecified one day, during a 4-month enrollment window, to report specific data collected during normal clinical practice for patients with non-myeloid tumors attending for chemotherapy (±radiotherapy) treatment. The primary endpoint was the prevalence of anemia as determined using a prespecified algorithm: hemoglobin (Hb) ?10 g/dL on/within 3 days prior to visit; ongoing anemia treatment; physician diagnosis of anemia, together with ?1 anemia symptom. Results Between November 18, 2010 and March 18, 2011, data for 1412 patients were collected (Italy n = 1130; Austria n = 282). Most patients (n = 1136; 80%) had solid tumors; 809 (57%) had received ?3 chemotherapy cycles. The prevalence of anemia was 32% (95% confidence interval: 29.4%–34.2%); 196 patients (14%) were deemed anemic based on Hb ?10 g/dL, 131 (9%) on ongoing anemia treatment, and 121 (9%) on physician diagnosis/anemia symptom. Overall, 1153 patients (82%) had Hb data; mean (standard deviation [SD]) Hb levels were 11.7 (1.7) g/dL. In total, 456 patients (32%) had anemia symptoms: fatigue (n = 392; 28%), depression (n = 122; 9%), and dyspnea (n = 107; 8%) were most common. Fifty-one patients (4%) had had their current chemotherapy cycle delayed due to anemia. On visit day, or ?28 days prior, 91 (6%), 188 (13%), and 81 patients (6%) had evidence of whole blood/red blood cell transfusion, erythropoiesis-stimulating agent use, or iron use, respectively. Conclusion On the prespecified study day, one-third of patients with non-myeloid tumors undergoing chemotherapy were found to be anemic and 13% had evidence of erythropoiesis-stimulating agent use then or in the 28 days prior. PMID:23946669

  18. Cerebral sinovenous thrombosis associated with iron deficiency anemia secondary to severe menorrhagia: a case report.

    PubMed

    Corrales-Medina, Fernando F; Grant, Leon; Egas-Bejar, Daniela; Valdivia-Ascuna, Zoila; Rodriguez, Nidra; Mancias, Pedro

    2014-09-01

    Cerebral sinovenous thrombosis is a rare condition presenting with a wide spectrum of nonspecific symptoms that can make early diagnosis difficult. Cerebral sinovenous thrombosis has been associated with various etiologies. Iron deficiency anemia associated with cerebral sinovenous thrombosis in teenagers is rare. We present a teenage patient with complete thrombosis of the vein of Galen, straight sinus, and left internal cerebral vein associated with iron deficiency anemia due to severe menorrhagia. Mechanisms that can explain the association between iron deficiency anemia and thrombosis are discussed. PMID:24056151

  19. Tc-99m red blood cells for the study of rapid hemolytic processes associated with heterologous blood transfusions

    SciTech Connect

    Benedetto, A.R.; Harrison, C.R.; Blumhardt, R.; Trow, L.L.

    1984-10-01

    Chromium-51 labeled erythrocytes (Cr-51 RBC) are suitable for the study of hematologic disorders which involve relatively slow destruction of circulating erythrocytes, taking several days to several weeks. However, Cr-51 RBC are not suitable for investigating rapid hemolytic processes which occur within a matter of a few hours due to the variable and unpredictable elution of Cr-51 from the erythrocytes during the first 24 hours or so. Imaging, which could be useful in identifying organ systems involved in the hemolytic process, cannot be performed with Cr-51 RBC because of the high dose commitment caused by the low yield of gamma rays from Cr-51 (2). A method of labeling RBC with Tc-99m, which results in a radiopharmaceutical that combines the excellent dosimetric and imaging qualities of Tc-99m with an extremely stable bond between the Tc-99m and the RBC, is reported. The successful application of this technique in providing red cell support for a cancer patient with an unusual history of intravascular hemolytic transfusion reactions is also reported.

  20. Autoimmune Polyglandular Syndrome Type 3c with Ectodermal Dysplasia, Immune Deficiency and Hemolytic-Uremic Syndrome

    PubMed Central

    Büyükçelik, Mithat; Keskin, Mehmet; Keskin, Özlem; Bay, Ali; Demircio?lu K?l?ç, Beltinge; Kor, Y?lmaz; K?l?nç, M. Arda; Balat, Ay?e

    2014-01-01

    Autoimmune polyglandular syndrome (APS) is a disorder which is associated with multiple endocrine gland insufficiency and also with non-endocrine manifestations. The pathophysiology of APS is poorly understood, but the hallmark evidence of APS is development of autoantibodies against multiple endocrine and non-endocrine organs. These autoantibodies are responsible for the dysfunction of the affected organs and sometimes may also cause non-endocrine organ dysfunction. The hemolytic-uremic syndrome (HUS) is a serious and life-threatening disease which develops due to many etiological factors including autoimmune disorders. Here, we present an unusual case of APS. Ectodermal dysplasia with immune deficiency and HUS occurred concomitantly in the same patient with APS type 3c. Once the autoantibody generation was initiated in the human body, development of multiple disorders due to organ dysfunction and also autoantibody-related diseases may have occurred. PMID:24637310

  1. [Occurrence and drug-resistance of beta-hemolytic streptococci].

    PubMed

    Miko?ajczyk, Dorota; Budzy?ska, Anna; Kaczmarek, Agnieszka; Gospodarek, Eugenia

    2007-01-01

    The aim of this study was the analysis of drug-resistance and frequency appearance of beta-hemolytic streptococci strains which were isolated in 2003-2005 in the University Hospital at the L. Rydygier Collegium Medicum in Bydgoszcz University of Nicolaus Copernicus in Toru?. Among investigeted beta-hemolytic streptococci the most frequency isolated species was S. agalactiae. All isolates examined in our study were susceptible to penicillin, the higest rate of resistance was found for tetracycline. The rates of resistence to macrolide-lincosamide-streptogramin B (phenotyp MLS(B)) were as follows: S. agalactiae (18.7%), S. pyogenes (10.1%), group G streptococci (10.6%) and group C streptococci (8.0%). In our study we presented also a special case patient from which in investigeted period S. agalactiae was isolated twenty eight times. For ten chromosomal DNA isolated from this patient three different PFGE profiles were obtained. PMID:18416122

  2. A simple microassay for computing the hemolytic potency of drugs.

    PubMed

    Raghava, G P; Goel, A; Singh, A M; Varshney, G C

    1994-12-01

    A simple microassay and computer program are described for determining the erythrocyte hemolytic potency of drugs in vitro. This microassay is sensitive for both micro as well as macro ranges of hemoglobin concentration. An ELISA reader has been adapted to read erythrocyte lysis (hemolysis), which reduces the number and culture of replicates. A computer program was developed that calculates parameters such as C50 (concentration of drug causing 50% hemolysis), C100 (concentration of drug causing 100% hemolysis) and beta (slope of the curve) and graphically expresses the hemolytic patterns of various drugs simultaneously. The program can obtain optical densities directly from a 96-well plate ELISA reader by interfacing the microplate reader to the computer or by using a keyboard. This method is useful for screening a large number of hemolytic drugs and requires lower amounts of test compounds. It may also be applicable to quantitative functional assays, such as complement-mediated hemolysis and enumeration of antibody-secreting cells. The program can be obtained from the authors on request. PMID:7873185

  3. Hemolytic venoms from marine cnidarian jellyfish – an overview

    PubMed Central

    Mariottini, Gian Luigi

    2014-01-01

    Cnidarian jellyfish are viewed as an emergent problem in several coastal zones throughout the world. Recurrent outbreaks pose a serious threat to tourists and bathers, as well as to sea-workers, involving health and economical aspects. As a rule, cnidarian stinging as a consequence of nematocyst firing induces merely local symptoms but cardiovascular or neurological complications can also occur. Hemolysis is a frequent effect of cnidarian stinging; this dangerous condition is known to be caused by several venoms and can sometimes be lethal. At present, the bulk of data concerning hemolytic cnidarian venoms comes from the study of benthic species, such as sea anemones and soft corals, but hemolytic factors were found in venoms of several siphonophore, cubozoan and scyphozoan jellyfish, which are mainly involved in the envenomation of bathers and sea-workers. Therefore, the aim of this paper is to review the scientific literature concerning the hemolytic venoms from cnidarian jellyfish taking into consideration their importance in human pathology as well as health implications and possible therapeutic measures. PMID:25386336

  4. Human spleen heme oxygenase in normal, hemolytic and other pathological states.

    PubMed

    Schacter, B A; Yoda, B; Israels, L G

    1976-01-01

    Heme oxygenase (HO), the primary enzyme responsible for heme catabolism, was measured in spleens from 10 normal subjects, 14 patients with chronic hemolytic anemia (HA), 12 with idiopathic thrombocytopenic purpura (ITP), and 10 with various lymphoproliferative disorders (LD) to determine the splenic enzymatic capacity for heme catabolism and the response of splenic HO to hemolysis. Splenic NADPH-cytochrome c reductase activity and cytochrome P-450 levels were also measured. Splenic HO specific activity in normal spleens was 0.235 +/- 0.106 (SE) nmoles bilirubin/mg protein/min; in HA, 0.276 +/- 0.050; in ITP, 0.228 +/- 0.036; and in LD, 0.420 +/- 0.105. However, the total HO activity per spleen was significantly greater in HA (742.9 +/- 137.4 (SE) nmoles/min, p less than 0.001) and LD (681.9 +/- 180.3, p less than 0.005) than in normal spleens (137.1 +/- 55.0), but was not significantly increased in ITP (269.5 +/- 121.5). In normal spleens cytochrome P-450 was 0.052 +/- 0.006 (SE) nmoles/mg and NADPH-cytochrome c reductase activity was 8.3 +/- 2.2 (SE) nmoles/mg/min; neither of these specific activities was significantly altered in HA, ITP, or LD. Again, total activity was significantly increased in HA and LD associated with increased splenic size. Although total HO activity is greater in the larger spleens, HO activity does not increase per unit weight of tissue. In normal spleen the calculated capacity for bilirubin production by HO was 115 mg per day. This accounts for only 50% of normal daily production from erythroid sources and suggests that other sites are of major importance for hemoglobin degradation. PMID:827232

  5. Atypical Hemolytic Uremic Syndrome Post-Kidney Transplantation: Two Case Reports and Review of the Literature

    PubMed Central

    Alasfar, Sami; Alachkar, Nada

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1–2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (infection-induced) HUS and is frequently characterized by relapses that leads to end stage renal disease. For a long time, kidney transplantation for these patients was contraindicated because of high rate of recurrence and subsequent renal graft loss. The post-kidney transplantation recurrence rate largely depends on the pathogenetic mechanisms involved. However, over the past several years, advancements in the understanding and therapeutics of aHUS have allowed successful kidney transplantation in these patients. Eculizumab, which is a complement C5 antibody that inhibits complement factor 5a and subsequent formation of the membrane-attack complex, has been used in prevention and treatment of post-transplant aHUS recurrence. In this paper, we present two new cases of aHUS patients who underwent successful kidney transplantation in our center with the use of prophylactic and maintenance eculizumab therapy that have not been published before. The purpose of reporting these two cases is to emphasize the importance of using eculizumab as a prophylactic therapy to prevent aHUS recurrence post-transplant in high-risk patients. We will also review the current understanding of the genetics of aHUS, the pathogenesis of its recurrence after kidney transplantation, and strategies for prevention and treatment of post-transplant aHUS recurrence. PMID:25593925

  6. Multiparameter FLAER-based flow cytometry for screening of paroxysmal nocturnal hemoglobinuria enhances detection rates in patients with aplastic anemia.

    PubMed

    Sachdeva, Man Updesh Singh; Varma, Neelam; Chandra, Dinesh; Bose, Parveen; Malhotra, Pankaj; Varma, Subhash

    2015-05-01

    Flow cytometry is the gold standard methodology for screening of paroxysmal nocturnal hemoglobinuria. In the last few years, proaerolysin conjugated with fluorescein (FLAER) has become an important component of antibody panel used for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study aimed to compare PNH clone detection by flow cytometry in the pre-FLAER era versus the FLAER era. This was a retrospective analysis of 4 years and included 1004 individuals screened for PNH clone, either presenting as hemolytic anemia or as aplastic anemia. In the pre-FLAER time period, the RBCs and neutrophils were screened with antibodies against CD55 and CD59. With the introduction of FLAER, neutrophils were screened with FLAER/CD24/CD15 and monocytes with FLAER/CD14/CD33 combination. A comparative analysis was done for detection of PNH clone in aplastic anemia patients versus non-aplastic anemia patients, as well as between pre-FLAER and FLAER era. Out of a total of 1004 individuals, 59 (5.8 %) were detected to have PNH clone positivity. The frequency of PNH clone detected in aplastic anemia and non-aplastic anemia groups was 12.02 and 3.36 %, respectively. The detection rate of PNH clone increased from 4.5 % (32/711) in the pre-FLAER era to 9.2 % (27/293) with the introduction of FLAER. However, this increase could be attributed to increased detection of PNH clone in the aplastic anemia group, which showed a significant increase from 8.3 to 18.2 % after use of FLAER. In the non-aplastic group, PNH clone was detected with similar frequencies before and after use of FLAER (3.2 versus 3.8 %, respectively). Mean PNH clone size was lower in the aplastic anemia group when compared with the non-aplastic group. RBCs always showed a lower clone size than neutrophils. PNH clone on neutrophils and monocytes was however similar. Inclusion of FLAER increases the sensitivity of the test which is especially useful in picking up small PNH clones in patients of aplastic anemia. PMID:25465235

  7. Antibacterial and Hemolytic Activities of Quaternary Pyridinium

    E-print Network

    bacteria but not mammalian cells.[2­4] Polymers have been used as antimicrobial agents due commonly used as biocidal agents.[6­15] A number of polymeric disinfectants based on quaternary pyridinium bacteria. Recently, Gao and coworkers synthesized random copolymers of acrylamide and vinyl pyridine

  8. [Erythropoietin is a novel therapeutic option in the treatment of anemia caused by small cell lung cancer].

    PubMed

    Tamási, Lilla; Bohács, Anikó; Wollák, András; Magyar, Pál

    2006-01-01

    Anemia is very common among patients with malignant tumors, due to the disease and chemotherapy. Anemia decreases the patient's quality of life. Erythropoietin therapy is accessible in Hungary for the treatment of chemotherapy-induced anemia in patients suffering from small cell lung cancer. In our case report we present the case of a 62-year-old female small cell lung cancer patient with severe anemia, treated by erythropoietin-beta. The erythropoietin treatment provided the possibility of effective chemo- and radiotherapy. The patient's quality of life greatly improved due to the lack of the symptoms of anemia. The adequate use of erythropoietin is of great help to the physician in the management of small cell lung cancer patients, by improving the quality of life. PMID:17099785

  9. Megaloblastic anemia: back in focus.

    PubMed

    Chandra, Jagdish

    2010-07-01

    Megaloblastic anemia (MA), in most instances in developing countries, results from deficiency of vitamin B(12) or folic acid. Over the last two to three decades, incidence of MA seems to be increasing. Of the two micronutrients, folic acid deficiency contributed to MA in a large majority of cases. Now deficiency of B(12) is far more common. In addition to anemia, occurrence of neutropenia and/or thrombocytopenia is increasingly being reported. Among cases presenting with pancytopenia, MA stands out as an important (commonest cause in some series) cause. This article focuses on these and certain other aspects of MA. Possible causes of increasing incidence of MA are discussed. Observations on other clinical features like neurocognitive dysfunction, associated hyperhomocysteinemeia and occurrence of tremors and thrombocytosis during treatment are highlighted. PMID:20589460

  10. Acquired Aplastic Anemia in Children

    PubMed Central

    Hartung, Helge D.; Olson, Timothy S.; Bessler, Monica

    2013-01-01

    SYNOPSIS This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder. PMID:24237973

  11. Aplastic anemia following varicella vaccine.

    PubMed

    Angelini, Paola; Kavadas, Fotini; Sharma, Navneet; Richardson, Susan E; Tipples, Graham; Roifman, Chaim; Dror, Yigal; Nofech-Mozes, Yehuda

    2009-08-01

    Varicella zoster vaccine is a safe vaccine that is rarely associated with life-threatening complications. We describe an immunocompetent child who developed transient severe aplastic anemia concomitant with a typical clinical and laboratory-proven chickenpox syndrome 3 weeks after immunization. A causative association between the vaccine and the hematologic disease is possible, and pediatricians should be aware of this severe although rare adverse event. PMID:19633522

  12. Group G Beta-Hemolytic Streptococcal Bacteremia Characterized by 16S Ribosomal RNA Gene Sequencing

    Microsoft Academic Search

    PATRICK C. Y. WOO; AMI M. Y. FUNG; SUSANNA K. P. LAU; SAMSON S. Y. WONG; KWOK-YUNG YUEN

    2001-01-01

    Little is known about the relative importance of the four species of Lancefield group G beta-hemolytic streptococci in causing bacteremia and the factors that determine the outcome for patients with group G beta-hemolytic streptococcal bacteremia. From 1997 to 2000, 75 group G beta-hemolytic streptococcal strains were isolated from the blood cultures of 66 patients. Sequencing of the 16S rRNA genes

  13. [Anemia in chronic kidney disease].

    PubMed

    Amador-Medina, Lauro Fabián

    2014-01-01

    Anemia is almost unavoidable in the last stages of chronic kidney disease. It is defined as a condition where hemoglobin concentration is below 2 standard deviations from the mean hemoglobin level of the general population, corrected for age and sex (typically, hemoglobin < 13 g/dL in adults and 12 g/dL in women). Although the cause is multi-factorial, the most known is inadequate erythropoietin production. Anemia has been associated with poor prognosis in patients with several conditions such as cancer, chronic kidney disease and congestive heart failure. Treatment with erythropoiesis-stimulating agents, such as erythropoietin, is a logical strategy that has enabled clinical improvement and reduced transfusion requirements for the patients; however, total correction of anemia with erythropoiesis-stimulating agents has demonstrated an increase in the risk of mortality or cardiovascular complications associated with these agents. In randomized trials, the achievement of normal or nearly normal hemoglobin levels is not associated with improved survival and reduced cardiovascular risk; however the ideal hemoglobin level with the use of erythropoiesis-stimulating agents seems to be problematic. More information is needed in order to obtain definite conclusions; in the meantime, using the lowest possible dose of erythropoietin seems to be the most prudent approach. PMID:25354060

  14. Osteonecrosis in a chemically induced rat model of human hemolytic disorders associated with thrombosis--a new model for avascular necrosis of bone.

    PubMed

    Shabat, S; Nyska, A; Long, P H; Goelman, G; Abramovitch, R; Ezov, N; Levin-Harrus, T; Peddada, S; Redlich, M; Yedgar, S; Nyska, M

    2004-03-01

    Bone injury occurs in human hemolytic disorders associated with thrombosis, such as beta-thalassemia and sickle cell disease. Exposure of rats to 2-butoxyethanol (BE) has been associated with hemolytic anemia, disseminated thrombosis, and infarction in multiple organs including bone. This rat model apparently mimics acute hemolysis and thrombosis in humans. To elucidate the extent of bone injury, male and female Fischer F344 rats were given 4 daily doses of 250 mg BE/5 ml water/kg of body weight. Tail vertebrae were studied by histopathology and magnetic resonance imaging (MRI). Thrombosis and infarction were seen in both sexes, but females were more severely affected. Lesions were characterized by extensive medullary fat necrosis, granulomatous inflammation, fibroplasia, growth plate degeneration, and new woven bone formation adjacent to necrotic bone trabeculae. MRI mean and standard deviation tissue-density data for both sexes indicated a significant (P < or = 0.05) decrease following 4-days treatment and a significant increase (P < or = 0.05) following an additional 24 days without treatment. Thus, MRI was useful in revealing BE-induced bone injury, which was predominantly necrotic initially and subsequently regenerative with proliferation of connective tissue and bone following postischemia recovery. PMID:14517720

  15. Distinct Renal Pathology and a Chemotactic Phenotype after Enterohemorrhagic Escherichia coli Shiga Toxins in Non-Human Primate Models of Hemolytic Uremic Syndrome

    PubMed Central

    Stearns-Kurosawa, Deborah J.; Oh, Sun-Young; Cherla, Rama P.; Lee, Moo-Seung; Tesh, Vernon L.; Papin, James; Henderson, Joel; Kurosawa, Shinichiro

    2014-01-01

    Enterohemorrhagic Escherichia coli cause approximately 1.5 million infections globally with 176,000 cases occurring in the United States annually from ingesting contaminated food, most frequently E. coli O157:H7 in ground beef or fresh produce. In severe cases, the painful prodromal hemorrhagic colitis is complicated by potentially lethal hemolytic uremic syndrome (HUS), particularly in children. Bacterial Shiga-like toxins (Stx1, Stx2) are primarily responsible for HUS and the kidney and neurologic damage that ensue. Small animal models are hampered by the inability to reproduce HUS with thrombotic microangiopathy, hemolytic anemia, and acute kidney injury. Earlier, we showed that nonhuman primates (Papio) recapitulated clinical HUS after Stx challenge and that novel therapeutic intervention rescued the animals. Here, we present detailed light and electron microscopic pathology examination of the kidneys from these Stx studies. Stx1 challenge resulted in more severe glomerular endothelial injury, whereas the glomerular injury after Stx2 also included prominent mesangiolysis and an eosinophilic inflammatory infiltration. Both toxins induced glomerular platelet-rich thrombi, interstitial hemorrhage, and tubular injury. Analysis of kidney and other organs for inflammation biomarkers showed a striking chemotactic profile, with extremely high mRNA levels for IL-8, monocyte chemoattractant protein 1, and macrophage inflammatory protein 1? and elevated urine chemokines at 48 hours after challenge. These observations give unique insight into the pathologic consequences of each toxin in a near human setting and present potential pathways for therapeutic intervention. PMID:23402998

  16. [Anemia in pregnancy].

    PubMed

    Huch, R

    1999-01-28

    Pregnancy and postpartum anaemia occurs worldwide, particularly in developing countries where it accounts for substantial maternal and infant morbidity and mortality. The main cause is iron deficiency, primarily of dietary origin: 20% of the world population are estimated to have some degree of trace element deficiency. Even in industrialized countries iron deficiency anaemia is common in pregnancy due to the negative iron balance created by the high fetal demand for iron. It is compounded by blood loss during and after delivery, particularly in the absence of adequate prevention and treatment. The main effects of pregnancy and postpartum anaemia (defined by the WHO as hemoglobin values < 110 g/l and < 100 g/l, respectively) present for the mother an increased susceptibility to infection and premature delivery and for the baby intrauterine growth retardation and the consequences of prematurity. Diagnosis and differential diagnosis are thus a major obstetric concern. Iron deficiency can be particularly difficult to diagnose in postpartum anaemia because ferrritin is often falsely elevated due to concurrent infection. Prevention with oral iron + folic acid supplementation has proven effective, as has intravenous iron in more severe cases, while the addition of recombinant erythropoietin augments the effect of iron alone. PMID:10067372

  17. Therapy Insight: congestive heart failure, chronic kidney disease and anemia, the cardio–renal–anemia syndrome

    Microsoft Academic Search

    Donald S Silverberg; Dov Wexler; Adrian Iaina

    2005-01-01

    Congestive heart failure (CHF) and chronic kidney disease (CKD) often progress to end stage even with optimum medical therapy. One factor that is common to both conditions is anemia, which is present in about a third of CHF patients. CHF can cause or worsen both anemia and CKD, and CKD can cause or worsen both anemia and CHF. Thus, a

  18. Cost-effectiveness of continuous erythropoietin receptor activator in anemia

    PubMed Central

    Schmid, Holger

    2014-01-01

    Background Erythropoiesis-stimulating agents (ESAs) are the mainstay of anemia therapy. Continuous erythropoietin receptor activator (CERA) is a highly effective, long-acting ESA developed for once-monthly dosing. A multitude of clinical studies has evaluated the safety and efficiency of this treatment option for patients with renal anemia. In times of permanent financial pressure on health care systems, the cost-effectiveness of CERA should be of particular importance for payers and clinicians. Objective To critically analyze, from the nephrologists’ point of view, the published literature focusing on the cost-effectiveness of CERA for anemia treatment. Methods The detailed literature search covered electronic databases including MEDLINE, PubMed, and Embase, as well as international conference abstract databases. Results Peer-reviewed literature analyzing the definite cost-effectiveness of CERA is scarce, and most of the available data originate from conference abstracts. Identified data are restricted to the treatment of anemia due to chronic kidney disease. Although the majority of studies suggest a considerable cost advantage for CERA, the published literature cannot easily be compared. While time and motion studies clearly indicate that a switch to CERA could minimize health care staff time in dialysis units, the results of studies comparing direct costs are more ambivalent, potentially reflecting the differences between health care systems and variability between centers. Conclusion Analyzed data are predominantly insufficient; they miss clear evidence and have to thus be interpreted with great caution. In this day and age of financial restraints, results from well-designed, head-to-head studies with clearly defined endpoints have to prove whether CERA therapy can achieve cost savings without compromising anemia management. PMID:25050070

  19. Gua breve sobre la La anemia es un trastorno de la sangre. La sangre es

    E-print Network

    Bandettini, Peter A.

    Anemia Guía breve sobre la La anemia es un trastorno de la sangre. La sangre es un líquido esencial de anemia, como la anemia por deficien- cia de hierro, la anemia perniciosa, la anemia aplásica y la anemia hemolítica. Los distintos tipos de anemia tienen relación con diversas enfermedades y problemas de

  20. Iron Deficiency Anemia: Focus on Infectious Diseases in Lesser Developed Countries

    PubMed Central

    Shaw, Julia G.; Friedman, Jennifer F.

    2011-01-01

    Iron deficiency anemia is thought to affect the health of more than one billion people worldwide, with the greatest burden of disease experienced in lesser developed countries, particularly women of reproductive age and children. This greater disease burden is due to both nutritional and infectious etiologies. Individuals in lesser developed countries have diets that are much lower in iron, less access to multivitamins for young children and pregnant women, and increased rates of fertility which increase demands for iron through the life course. Infectious diseases, particularly parasitic diseases, also lead to both extracorporeal iron loss and anemia of inflammation, which decreases bioavailability of iron to host tissues. This paper will address the unique etiologies and consequences of both iron deficiency anemia and the alterations in iron absorption and distribution seen in the context of anemia of inflammation. Implications for diagnosis and treatment in this unique context will also be discussed. PMID:21738863

  1. Enzyme kinetics and molecular modeling studies of G6PD(Mahidol) associated with acute hemolytic anemia.

    PubMed

    Lu, Hui-Ru; Tang, Qiong-Ling; Wang, Xin; Li, Hong-Jun; Li, Dan-Yi; Yang, Yin-Feng; Tong, Shu-Fen; Zhang, Chun-Hua; Zhu, Yue-Chun

    2011-10-01

    G6PD(Mahidol) enzyme is the most common variant in the Achang Chinese ethnic group and clinically manifests as class II. In this study, G6PD(Mahidol) enzyme was characterized by molecular modeling to understand its kinetics. G6PD(Mahidol), G6PD(G487A) and G6PD(WT) proteins were heterologously expressed in the G6PD-deficient DF213 E. coli strain, purified and their steady-state kinetic parameters were determined. Compared with G6PD(WT), the Km, and Vmax of NADP+ with G6PD(G487A) were about 28-fold and 12-fold lower, respectively. The Ki values of dehydroepiandrosterone (DHEA), NADPH and ATP with G6PD(G487A) showed 29.5-fold, 2.36-fold reduction and 1.83-fold increase, respectively. A molecular modeling of G6PD(G487A) was performed based on the X-ray structure of human G6PD (PDB: 2BH9). It is suggested that Ser-163 might affect the stability of G6PD(G487A) alpha-helix d and beta-strand E, besides the conformation of beta-strand D. In conclusion, the biochemical and structural properties of G6PD(G487A) and G6PD(WT) enzymes are significantly different, which may be responsible for clinical diversity of G6PD deficiencies. PMID:22165289

  2. Refractory autoimmune hemolytic anemia after intestinal transplant responding to conversion from a calcineurin to mTOR inhibitor.

    PubMed

    Acquazzino, Melissa A; Fischer, Ryan T; Langnas, Alan; Coulter, Don W

    2013-08-01

    AIHA is a rare and serious complication of solid organ transplantation. Herein, we report four cases of warm or mixed AIHA in pediatric patients following combined liver, small bowel and pancreas transplant. The hemolysis was refractory to multiple treatment modalities including steroids, rituximab, IVIG, plasmapheresis, cytoxan, discontinuation of prophylactic penicillin, and a change in immunosuppression from tacrolimus to cyclosporine. All patients had resolution or marked improvement of hemolysis after discontinuation of maintenance of CNI and initiation of sirolimus immunosuppression. One patient developed nephrotic syndrome but responded to a change in immunosuppression to everolimus. Three of the four patients continue on immunosuppression with sirolimus or everolimus without further hemolysis, evidence of rejection or medication side effects. Based on our experience and review of similar cases in the literature, we have proposed a treatment algorithm for AIHA in the pediatric intestinal transplant patient population that recommends an early change in immunosuppressive regimen from CNIs to sirolimus therapy. PMID:23730873

  3. Enzymatic and hemolytic properties of Propionibacterium acnes and related bacteria.

    PubMed

    Hoeffler, U

    1977-12-01

    The production of chondroitin sulfatase, hyaluronidase, deoxyribonuclease, gelatinase, phosphatase, lecithinase, and hemolysins was examined in 95 strains of Propionibacterium acnes and four related species of anaerobic, respectively, microaerophilic coryneform bacteria (P. avidum, P. lymphophilum, P. granulosum, and Corynebacterium minutissimum). All enzymes could be demonstrated in at least one representative of the species tested. Those Propionibacterium species most frequently found in acne vulgaris lesions, i.e., P. acnes and P. granulosum, proved to be the most active organisms concerning the production of the enzymes tested. P. avidum, on the other hand, showed the highest rate of hemolytic activity. PMID:201661

  4. Hypothesis: Hemolytic Transfusion Reactions Represent an Alternative Type of Anaphylaxis

    PubMed Central

    Hod, Eldad A.; Sokol, Set A.; Zimring, James C.; Spitalnik, Steven L.

    2009-01-01

    Classical anaphylaxis is the most severe, and potentially fatal, type of allergic reaction, manifested by hypotension, bronchoconstriction, and vascular permeability. Similarly, a hemolytic transfusion reaction (HTR) is the most feared consequence of blood transfusion. Evidence for the existence of an alternative, IgG-mediated pathway of anaphylaxis may be relevant for explaining the pathophysiology of IgG-mediated-HTRs. The purpose of this review is to summarize the evidence for this alternative pathway of anaphylaxis and to present the hypothesis that an IgG-mediated HTR is one example of this type of anaphylaxis. PMID:18830382

  5. Management of postoperative complications: anemia.

    PubMed

    Willett, Laura Rees; Carson, Jeffrey L

    2014-05-01

    Anemia is extremely common following hip fracture. Consistent data from randomized trials show that transfusion of less blood, with a transfusion threshold around 8 g/dL hemoglobin concentration, is preferable to a traditional threshold of 10 g/dL. Adoption of a lower threshold leads to at least equivalent clinical outcomes, with much less exposure to transfusion costs and risks. The most common complication of transfusion is circulatory overload. Future research may elucidate the optimal transfusion threshold for these elderly patients and address the specific needs of subgroups of patients, including those with acute coronary syndrome or chronic kidney disease. PMID:24721367

  6. Anemia in Children and Adolescents with Hypothyroidism

    Microsoft Academic Search

    Jen-Yih Chu; James A. Monteleone; Virginia H. Peden; Edward R. Graviss; Anthony M. Vernava

    1981-01-01

    In a review of 17 adolescents and children (excluding newborns) with definite clinical signs, symptoms, and laboratory findings of hypothyroidism, 11 patients (65%) had anemia. The mean corpuscular volume (MCV) of the red blood cells was either macrocytic or normocytic. The hemoglobin did not correlate with the serum thyroxine level. Anemia occurred only in those patients with heights below the

  7. Serum Erythropoietin (ESF) Titers in Anemia

    Microsoft Academic Search

    G. de Klerk; P. C. J. Rosengarten; R. J. W. M. Vet; R. Goudsmit

    1981-01-01

    Erythropoietin (ESF) titers were determined in sera from patients with different types of anemia using the fetal mouse liver cell bioassay. An inverse relationship was found between hemoglobin concentration and ESF titer. However, ESF titers differed markedly between patients at comparable degrees of anemia. Several groups of patients were distinguished on the basis of the activity of their erythroid bone

  8. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...Animal Products 2 2011-01-01 2011-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  9. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...Animal Products 2 2012-01-01 2012-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  10. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...Animal Products 2 2013-01-01 2013-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  11. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...Animal Products 2 2014-01-01 2014-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  12. The Student with Sickle Cell Anemia.

    ERIC Educational Resources Information Center

    Tetrault, Sylvia M.

    1981-01-01

    Sickle cell anemia is the most common and severe of inherited chronic blood disorders. In the United States, sickle cell anemia is most common among the Black population. Among the most commonly occurring symptoms are: an enlarged spleen, episodes of severe pain, easily contracted infections, skin ulcers, and frequent urination. (JN)

  13. [Effect of methylcobalamin and adenosylcobalamin on the process of hematopoiesis and vitamin B12 exchange in experimental phenylhydrazine-induced anemia in rabbits].

    PubMed

    Tsukerman, E S; Pomerantseva, T Ia; Poznanskaia, A A; Korsova, T L; Klement'eva, I V

    1989-01-01

    Poisoning of rabbits with phenylhydrazine resulted in development of haemolytic hyperchromic anemia accompanied by impairment of hemopoiesis in bone marrow as well as by an increase of total vitamin B12 content in blood. The ration of individual forms of cobalamins was firstly estimated in blood serum of healthy rabbits and of the animals treated with phenylhydrazine. Distinct decrease in the methyl cobalamin content was observed in blood serum during spontaneous recovery. Administration of methyl cobalamin led to complete normalization of some blood and hematopoiesis patterns, as well as to restoration of total cobalamins content and the spectrum of their individual forms. Adenosyl cobalamin exhibited distinctly lower effect on the patterns studied. The data obtained suggest that methyl cobalamin possessed a lot of advantages in treatment of hemolytic anemias. PMID:2741387

  14. Hemolytic activity of venom from crown-of-thorns starfish Acanthaster planci spines

    PubMed Central

    2013-01-01

    Background The crown-of-thorns starfish Acanthaster planci is a venomous species from Taiwan whose venom provokes strong hemolytic activity. To understand the hemolytic properties of A. planci venom, samples were collected from A. planci spines in the Penghu Islands, dialyzed with distilled water, and lyophilized into A. planci spine venom (ASV) powder. Results Both crude venom and ASV cause 50% hemolysis at a concentration of 20 ?g/mL. The highest hemolytic activity of ASV was measured at pH 7.0-7.4; ASV-dependent hemolysis was sharply reduced when the pH was lower than 3 or greater than 8. There was almost no hemolytic activity when the Cu2+ concentration was increased to 10 mM. Furthermore, incubation at 100°C for 30 to 60 minutes sharply decreased the hemolytic activity of ASV. After treatment with the protease ?-chymotrypsin, the glycoside hydrolase cellulase, and the membrane component cholesterin, the hemolytic activity of ASV was significantly inhibited. Conclusions The results of this study provide fundamental information about A. planci spine venom. The hemolytic activity was affected by pH, temperature, metal ions, EDTA, cholesterin, proteases, and glycoside hydrolases. ASV hemolysis was inhibited by Cu2+, cholesterin, ?-chymotrypsin, and cellulose, factors that might prevent the hemolytic activity of venom and provide the medical treatment for sting. PMID:24063308

  15. Large twisted ovarian fibroma associated with Meigs’ syndrome, abdominal pain and severe anemia treated by laparoscopic surgery

    PubMed Central

    2014-01-01

    Background The Meigs' syndrome is a rare but well-known syndrome defined as the triad of benign solid ovarian tumor, ascites, and pleural effusion. Meigs' syndrome always requires surgical treatment. However, the optimal approach for its management has not been sufficiently investigated. Case presentation We report a patient with a large twisted ovarian fibroma associated with Meigs’ syndrome, abdominal pain and severe hemolytic anemia that was treated by laparoscopic surgery. This case highlights the difficulties that may be encountered in the management of patients with Meigs’ syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach and the adverse impact that Meigs’ syndrome can have on the patient’s condition, especially if it is associated with acute pain and severe anemia. Considering the patient’s serious clinical condition and assuming that she had Meigs' syndrome with a twisted large ovarian mass and possible hemolytic anemia, we first concentrated on effective medical management of our patient and chose the most appropriate surgical treatment after laparoscopic examination. The main aim of our initial approach was preoperative management of the anemia. Blood transfusions and glucocorticoid therapy resulted in stabilization of the hemoglobin level and normalization of the bilirubin levels, which confirmed the appropriateness of this approach. Laparoscopic surgery 4 days after admission enabled definitive diagnosis of the tumor, confirmed torsion and removed the bulky ovarian fibroma, resulting in timely resolution of symptoms, short hospitalization, relatively low morbidity and a rapid return to her social and professional life. Conclusions This case highlights the difficulties that may be encountered in the management of patients with Meigs' syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach, and the adverse impact that Meigs' syndrome can have on the patient's condition, especially if it is associated with acute pain and severe anemia. The present case suggests that laparoscopic surgery for potentially large malignant tumors is feasible and safe, but requires an appropriate medical and gynecological oncology expertise. PMID:24962423

  16. Unexplained Aspects of Anemia of Inflammation

    PubMed Central

    Price, Elizabeth A.; Schrier, Stanley L.

    2010-01-01

    Anemia of inflammation (AI), also known as anemia of chronic inflammation or anemia of chronic disease was described over 50 years ago as anemia in association with clinically overt inflammatory disease, and the findings of low plasma iron, decreased bone marrow sideroblasts and increased reticuloendothelial iron. Pathogenic features underlying AI include a mild shortening of red cell survival, impaired erythropoietin production, blunted responsiveness of the marrow to erythropoietin, and impaired iron metabolism mediated by inflammatory cytokines and the iron regulatory peptide, hepcidin. Despite marked recent advances in understanding AI, gaps remain, including understanding of the pathogenesis of AI associated with “noninflammatory” or mildly inflammatory diseases, the challenge of excluding iron deficiency anemia in the context of concomitant inflammation, and understanding more precisely the contributory role of hepcidin in the development of AI in human inflammatory diseases. PMID:20368776

  17. Anticariogenic and Hemolytic Activity of Selected Seed Protein Extracts In vitro conditions

    PubMed Central

    Ishnava, Kalpesh B; Shah, Pankit P.

    2014-01-01

    Objective: This study aimed to assess the anticariogenic and hemolytic activity of crude plant seed protein extracts against tooth decaying bacteria. Materials and Methods: The proteins from seeds of 12 different plants were extracted and used for antimicrobial assay against six different organisms. The extraction was carried out in 10mM of sodium phosphate buffer (pH 7.0). Protein concentrations were determined as described by Bradford method. Anticariogenic activity was studied by agar well diffusion method and Minimum Inhibitory Concentration (MIC) was evaluated by the two-fold serial broth dilution method. Hemolytic activity, treatment of proteinase K and Kinetic study in Mimusops elengi crude seed protein extract. Results: The anticariogenic assay demonstrated the activity of Mimusops elengi against Staphylococcus aureus and Streptococcus pyogenes. A minor activity of Glycine wightii against Streptococcus mutans was also found. The protein content of Mimusops elengi seed protein extract was 5.84mg/ml. The MIC values for Staphylococcus aureus and Streptococcus pyogenes against Mimusops elengi seed protein extract were 364.36?g/ml and 182.19?g/ml, respectively. Kinetic study further elucidated the mode of inhibition in the presence of the Mimusops elengi plant seed protein with respect to time. The concentration of crude extract which gave 50% hemolysis compared to Triton X-100 treatment (HC50) value was 1.58 mg/ml; which is more than five times larger than that of the MIC. Treatment with proteinase K of the Mimusops elengi seed protein resulted in absence of the inhibition zone; which clearly indicates that the activity was only due to protein. Conclusion: Our results showed the prominence of Mimusops elengi plant seed protein extract as an effective herbal medication against tooth decaying bacteria. PMID:25628685

  18. Homozygosity mapping of Fanconi anemia

    SciTech Connect

    Gschwend, M.; Botstein, D. [Stanford Univ., CA (United States); Kruglyak, L. [Whitehead Institute, Cambridge, MA (United States)] [and others

    1994-09-01

    Fanconi anemia (FA) is a rare, recessive, genetically heterogeneous disease characterized by progressive insufficiency of the bone marrow and increased cellular sensitivity to DNA crosslinking agents. Complementation tests among different FA cells have indicated the presence of at least 4 FA-causing genes. One of the genes, FACC, was identified by functional complementation but appears unlikely to account for many phenotypically indistinguishable FA caes. We have begun a linkage study of FA using {open_quotes}homozygosity mapping{close_quotes}, a method that involves genotyping with DNA markers on affected individuals whose parents are related. Because FA is a rare recessive disease, it is most likely that probands are homozygous by descent at the disease locus and, therefore, at nearby DNA markers. Although the probability that any given marker will be homozygous in an inbred individual is high, given markers with moderate heterozygosities, the chance that two unrelated inbred individuals will be homozygous at the same marker is considerably lower. By locating overlapping regions of homozygosity between different families we hope to identify genes that cause FA. Sixteen consanguineous non-FACC FA families from the International Fanconi Anemia Registry at Rockefeller University are under study. An efficient algorithm for data analysis was developed and incorporated into software that can quickly compute exact multipoint lod scores using all markers on an entire chromosome. At the time of this writing, 171 of 229 microsatellite markers spaced at 20 cM intervals across the genome have been analyzed.

  19. Method for analysis of nanoparticle hemolytic properties in vitro.

    PubMed

    Neun, Barry W; Dobrovolskaia, Marina A

    2011-01-01

    Hemolysis is damage to red blood cells (RBCs), which results in the release of the iron-containing protein hemoglobin into plasma. Here we describe an in vitro assay specifically developed for the analysis of nanoparticle hemolytic properties (see Fig. 1). In this assay, analyte nanoparticles are incubated in blood, and hemoglobin is released by damaged cells and converted to red-colored cyanmethemoglobin by reagents. The nanoparticles and undamaged RBCs are then removed by centrifugation, and the amount of cyanmethemoglobin in the supernatant is measured by spectrophotometry. This measured absorbance is compared to a standard curve to determine the concentration of hemoglobin in the supernatant. This hemoglobin concentration is then compared to that in the supernatant of a blood sample treated with a negative control to obtain the percentage of nanoparticle-induced hemolysis. Fig. 1. Schematic illustration of the steps in this in vitro assay to evaluate nanoparticle hemolytic properties. PFH is plasma-free hemoglobin. CMH is cyanmethemoglobin. TBH is total blood hemoglobin. PMID:21116971

  20. Listeria monocytogenes and hemolytic Listeria innocua in poultry.

    PubMed

    Milillo, S R; Stout, J C; Hanning, I B; Clement, A; Fortes, E D; den Bakker, H C; Wiedmann, M; Ricke, S C

    2012-09-01

    Listeria monocytogenes is a ubiquitous, saprophytic, Gram-positive bacterium and occasional food-borne pathogen, often associated with ready-to-eat meat products. Because of the increased consumer interest in organic, all natural, and free range poultry products, it is important to understand L. monocytogenes in the context of such systems. Pasture-reared poultry were surveyed over the course of two 8-wk rearing periods. Cecal, soil, and grass samples were collected for Listeria isolation and characterization. Seven of 399 cecal samples (or 1.75%) were Listeria-positive. All positive cecal samples were obtained from broilers sampled at 2 wk of age. Grass and soil samples were collected from the pasture both before and after introduction of the poultry. Environmental samples collected after introduction of poultry were significantly more likely to contain Listeria (P < 0.001). The results of analytical profile index Listeria, sigB allelic typing, and hlyA PCR tests found that both L. monocytogenes and L. innocua, including hemolytic L. innocua, were recovered from the cecal and environmental (grass/soil) samples. The sigB allelic typing also revealed that (1) positive samples could be composed of 2 or more allelic types; (2) allelic types found in cecal samples could also be found in the environment; and (3) allelic types could persist through the 2 rearing periods. Our data indicate that both pasture-reared poultry and their environment can be contaminated with L. monocytogenes and hemolytic L. innocua. PMID:22912449

  1. What is the optimal treatment for anemia in inflammatory bowel disease?

    PubMed

    Kent, Alexandra J; Blackwell, Victoria J; Travis, Simon P L

    2012-07-01

    Anemia is common in inflammatory bowel disease (IBD), with a prevalence ranging from 8.8% to 73.7%. This wide range reflects the definitions used and the populations studied. Although many patients are reported to be asymptomatic, systematic studies have shown anemia to have a significant impact on quality of life. Consequently treatment should be instituted early. The commonest cause of anemia in IBD is iron deficiency, predominantly related to gastrointestinal blood loss. Anemia of chronic disease often occurs concomitantly, due to cytokine-mediated impaired erythropoiesis and dysregulated iron metabolism. Oral iron is a simple and effective method for treating iron deficiency, but requires long courses of treatment. It is also theoretically implicated with worsening intestinal inflammation, via the production of toxic reactive oxygen species. Intravenous iron avoids these concerns, especially with the development of ferric carboxymaltose, which allow up to 1000mg to be given rapidly. In patients failing to respond to intravenous iron, the anemia of chronic disease is most likely to be causative. In this setting evidence suggests that additional erythropoietin therapy can be effective. Blood transfusions should be avoided as part of routine management and reserved for patients with substantial acute gastro-intestinal bleeding, where there is a risk of hemodynamic compromise. This article discusses the underlying physiology of anemia in IBD, and presents the current evidence supporting treatment options available. PMID:22023204

  2. Primary stroke in a woman with sickle cell anemia responsive to hydroxyurea therapy.

    PubMed

    Ballas, Samir K; Martinez, Ubaldo; Savage, Michael

    2014-01-01

    The most common cause of stroke in children with sickle cell anemia is infarction due to ischemia. In adults, however, stroke is most commonly hemorrhagic in nature. Other causes of stroke in patients with sickle cell disease are very rare. In this short communication, we describe a woman with sickle cell anemia responsive to hydroxyurea (HU) therapy who had primary stroke due to paradoxical embolization caused by a large atrial septal defect. Successful management of the stroke included surgical closure of the defect with trans-esophageal echocardiographic guidance. To the best of our knowledge, this is the first patient with sickle cell anemia and stroke due to congenital heart disease who did not require open heart surgery for successful management. PMID:25238042

  3. Anemia

    MedlinePLUS

    ... in divided doses. For example, if you are prescribed two pills daily, take one in morning with breakfast and the ... getting enough iron, iron pills (supplements) may be prescribed. In extreme ... Iron pills can help when diet alone can't restore the iron level back ...

  4. Anemia

    MedlinePLUS

    ... 2 pregnancies close together Being pregnant with twins, triplets or more Becoming pregnant as a teenager Losing ... of passing these diseases on to your unborn baby. If you or someone in your family has ...

  5. Successful Allogeneic Hematopoietic Stem Cell Transplantation of a Patient Suffering from Type II Congenital Dyserythropoietic Anemia A Rare Case Report from Western India.

    PubMed

    Modi, Gaurang; Shah, Sandip; Madabhavi, Irappa; Panchal, Harsha; Patel, Apurva; Uparkar, Urmila; Anand, Asha; Parikh, Sonia; Patel, Kinnari; Shah, Kamlesh; Revannasiddaiah, Swaroop

    2015-01-01

    The most frequent form of congenital dyserythropoiesis (CDA) is congenital dyserythropoietic anemia II (CDA II). CDA II is a rare genetic anemia in humans, inherited in an autosomally recessive mode, characterized by hepatosplenomegaly normocytic anemia and hemolytic jaundice. Patients are usually transfusion-independent except in severe type. We are here reporting a case of severe transfusion-dependent type II congenital dyserythropoietic anemia in a 5-year-old patient who has undergone allogeneic hematopoietic stem cell transplantation (HSCT) at our bone marrow transplantation centre. Patient has had up until now more than 14?mL/kg/month of packed cell volume (PCV), which he required every 15 to 20 days to maintain his hemoglobin of 10?gm/dL and hematocrit of 30%. His pre-HSCT serum ferritin was 1500?ng/mL and he was on iron chelating therapy. Donor was HLA identical sibling (younger brother). The preparative regimen used was busulfan, cyclophosphamide, and antithymocyte globulin (Thymoglobulin). Cyclosporine and short-term methotrexate were used for graft versus host disease (GVHD) prophylaxis. Engraftment of donor cells was quick and the posttransplant course was uneventful. The patient is presently alive and doing well and he has been transfusion-independent for the past 33 months after HSCT. PMID:25692053

  6. Successful Allogeneic Hematopoietic Stem Cell Transplantation of a Patient Suffering from Type II Congenital Dyserythropoietic Anemia A Rare Case Report from Western India

    PubMed Central

    Modi, Gaurang; Shah, Sandip; Panchal, Harsha; Patel, Apurva; Uparkar, Urmila; Anand, Asha; Parikh, Sonia; Patel, Kinnari; Shah, Kamlesh; Revannasiddaiah, Swaroop

    2015-01-01

    The most frequent form of congenital dyserythropoiesis (CDA) is congenital dyserythropoietic anemia II (CDA II). CDA II is a rare genetic anemia in humans, inherited in an autosomally recessive mode, characterized by hepatosplenomegaly normocytic anemia and hemolytic jaundice. Patients are usually transfusion-independent except in severe type. We are here reporting a case of severe transfusion-dependent type II congenital dyserythropoietic anemia in a 5-year-old patient who has undergone allogeneic hematopoietic stem cell transplantation (HSCT) at our bone marrow transplantation centre. Patient has had up until now more than 14?mL/kg/month of packed cell volume (PCV), which he required every 15 to 20 days to maintain his hemoglobin of 10?gm/dL and hematocrit of 30%. His pre-HSCT serum ferritin was 1500?ng/mL and he was on iron chelating therapy. Donor was HLA identical sibling (younger brother). The preparative regimen used was busulfan, cyclophosphamide, and antithymocyte globulin (Thymoglobulin). Cyclosporine and short-term methotrexate were used for graft versus host disease (GVHD) prophylaxis. Engraftment of donor cells was quick and the posttransplant course was uneventful. The patient is presently alive and doing well and he has been transfusion-independent for the past 33 months after HSCT. PMID:25692053

  7. Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.

    PubMed

    Schrezenmeier, Hubert; Körper, Sixten; Höchsmann, Britta

    2015-02-01

    Aplastic anemia is a rare life-threatening bone marrow failure that is characterized by bicytopenia or pancytopenia in the peripheral blood and a hypoplastic or aplastic bone marrow. The patients are at risk of infection and hemorrhage due to neutropenia and thrombocytopenia and suffer from symptoms of anemia. The main treatment approaches are allogeneic stem cell transplantation and immunosuppression. Here, we review current standard immunosuppression and the attempts that have been made in the past two decades to improve results: review of recent developments also reveals that sometimes not only the advent of new drugs, good ideas and well-designed clinical trials decide the progress in the field but also marketing considerations of pharmaceutical companies. Aplastic anemia experts unfortunately had to face the situation that efficient drugs were withdrawn simply for marketing considerations. We will discuss the current options and challenges in first-line treatment and management of relapsing and refractory patients with an emphasis on adult patients. Some promising new approaches are currently under investigation in prospective, randomized trials. PMID:25572607

  8. Protrusio acetabuli in sickle-cell anemia

    SciTech Connect

    Martinez, S.; Apple, J.S.; Baber, C.; Putman, C.E.; Rosse, W.F.

    1984-04-01

    Of 155 adults with sickle-cell anemia (SS, SC), radiographs of the pelvis or hip demonstrated protrusio acetabuli on at least one side in 14 (3 men and 11 women), as indicated by projection of the acetabular line medial to the ilio-ischial line. All 14 patients had bone changes attributable to sickle-cell anemia, including marrow hyperplasia and osteonecrosis; however, the severity of femoral or acetabular osteonecrosis did not appear directly related to the protrusion. The authors conclude that sickle-cell anemia can predispose to development of protrusio acetabuli.

  9. TNF-? signaling in Fanconi anemia

    PubMed Central

    Du, Wei; Erden, Ozlem; Pang, Qishen

    2013-01-01

    Tumor necrosis factor-alpha (TNF-? is a major pro-inflammatory cytokine involved in systemic inflammation and the acute phase reaction. Dysregulation of TNF production has been implicated in a variety of human diseases including Fanconi anemia (FA). FA is a genomic instability syndrome characterized by progressive bone marrow failure and cancer susceptibility. The patients with FA are often found overproducing TNF-?, which may directly affect hematopoietic stem cell (HSC) function by impairing HSC survival, homing and proliferation, or indirectly change the bone marrow microenvironment critical for HSC homeostasis and function, therefore contribute to disease progression in FA. In this brief review, we discuss the link between TNF-? signaling and FA pathway with emphasis on the implication of inflammation in the pathophysiology and abnormal hematopoiesis in FA. PMID:23890415

  10. Nonregenerative anemia: mechanisms of decreased or ineffective erythropoiesis.

    PubMed

    Grimes, C N; Fry, M M

    2015-03-01

    In veterinary medicine, anemia without an appropriate compensatory hematopoietic response is termed nonregenerative. Nonregenerative anemia is a common clinical entity, occurring as a result of diminished or ineffective erythropoiesis in association with many types of pathology. This article reviews nonregenerative anemia in domestic animals, emphasizing mechanisms of disease, and also covers other conditions associated with nonregenerative anemia in people. Many aspects of nonregenerative anemia in animals are worthy of further investigation, from molecular mechanisms of disease to epidemiologic impacts. PMID:24807888

  11. Factors influencing hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye.

    PubMed

    Yu, Huahua; Li, Cuiping; Li, Ronggui; Xing, Ronge; Liu, Song; Li, Pengcheng

    2007-07-01

    In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU(50) of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40 microg/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and physical factors such as divalent cations, EDTA, (NH(4))(2)SO(4), pH and temperature. In the presence of Mg(2+), Cu(2+), Zn(2+), Fe(2+), Ca(2+) (>or=2 mM), Mn(2+) ((>or=1 mM), EDTA ((>or=2 mM) and (NH(4))(2)SO(4), the hemolytic activity of RFV was reduced. RFV had strong hemolytic activity at the pH 6-10 and the hemolytic ratios were 0.95-1.19. Hemolytic activity was temperature-sensitive and when RFV was pre-incubated at temperatures over 40 degrees C, it was sharply reduced. PMID:17306433

  12. Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura?

    PubMed

    Fakhouri, Fadi; Frémeaux-Bacchi, Véronique

    2007-12-01

    Both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are characterized by thrombotic microangiopathy (TMA), affecting mainly the kidney and brain, respectively. Diagnosis of HUS or TTP has been complicated by the fact that these disorders share several clinical characteristics, and by the dearth of knowledge regarding the pathogenesis of TMA. Advances in the identification of pathogenic features--deficiency of the metalloprotease ADAMTS13 in TTP and association of mutated complement proteins with atypical HUS--have gone some way towards improving clinicians' ability to distinguish between the two diseases. Here, we pose the following question: is it important to patient management that HUS be distinguished from TTP? By discussing what is known about the pathogenesis, clinical features and treatment of these two conditions we address this question, and propose a new nomenclature for TMA. PMID:18033227

  13. Recurrent ocular involvement in pediatric atypical hemolytic uremic syndrome.

    PubMed

    Zheng, Xiaoyu; Gorovoy, Ian R; Mao, Jianhua; Jin, Ji; Chen, Xi; Cui, Qi N

    2014-01-01

    Atypical hemolytic uremic syndrome (HUS) is a subtype of thrombotic microangiopathy associated with complement alternative pathway dysregulation. It is clinically characterized by a relapsing course and a poor prognosis. Multiple organ systems are commonly affected by thrombotic microangiopathy in pediatric atypical HUS; however, ocular involvement is rarely reported. The case of an 11-year-old girl diagnosed as having atypical HUS who presented with bilateral central retinal vein occlusions with macular subhyaloid hemorrhage during her initial onset and ophthalmoplegia, diplopia, and optic disc edema during her relapsing episode 1 year later is described. All ocular manifestations occurred in the convalescence phase of atypical HUS. No other extrarenal complications were found and full recovery was achieved following typical treatment for atypical HUS (ie, plasma infusion, steroid, and supportive therapy). This is thought to be the first reported case of recurrent ocular involvement in pediatric atypical HUS. PMID:25347082

  14. Recurrent ocular involvement in pediatric atypical hemolytic uremic syndrome.

    PubMed

    Zheng, Xiaoyu; Gorovoy, Ian R; Mao, Jianhua; Jin, Ji; Chen, Xi; Cui, Qi N

    2014-01-01

    Atypical hemolytic uremic syndrome (HUS) is a subtype of thrombotic microangiopathy associated with complement alternative pathway dysregulation. It is clinically characterized by a relapsing course and a poor prognosis. Multiple organ systems are commonly affected by thrombotic microangiopathy in pediatric atypical HUS; however, ocular involvement is rarely reported. The case of an 11-year-old girl diagnosed as having atypical HUS who presented with bilateral central retinal vein occlusions with macular subhyaloid hemorrhage during her initial onset and ophthalmoplegia, diplopia, and optic disc edema during her relapsing episode 1 year later is described. All ocular manifestations occurred in the convalescence phase of atypical HUS. No other extrarenal complications were found and full recovery was achieved following typical treatment for atypical HUS (ie, plasma infusion, steroid, and supportive therapy). This is thought to be the first reported case of recurrent ocular involvement in pediatric atypical HUS. PMID:25608228

  15. Molecular Basis for Group B ? -hemolytic Streptococcal Disease

    NASA Astrophysics Data System (ADS)

    Hellerqvist, Carl G.; Sundell, Hakan; Gettins, Peter

    1987-01-01

    Group B ? -hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin.

  16. How Is Sickle Cell Anemia Diagnosed?

    MedlinePLUS

    ... from the NHLBI on Twitter. How Is Sickle Cell Anemia Diagnosed? A simple blood test, done at ... Next >> Featured Video Living With and Managing Sickle Cell Disease (Nicholas) 10/14/2014 Living With and ...

  17. FastStats: Anemia or Iron Deficiency

    MedlinePLUS

    ... What's this? Submit Button NCHS Home Anemia or Iron Deficiency Recommend on Facebook Tweet Share Compartir Data ... for the U.S. Morbidity Percent of persons with iron deficiency (based on the body iron model): Children ...

  18. Anemia caused by low iron - children

    MedlinePLUS

    Anemia - iron deficiency - children ... able to absorb iron well, even though the child is eating enough iron Slow blood loss over ... bleeding in the digestive tract Iron deficiency in children can also be related to lead poisoning .

  19. Avoiding Anemia: Boost Your Red Blood Cells

    MedlinePLUS

    ... our exit disclaimer . Subscribe Avoiding Anemia Boost Your Red Blood Cells If you’re feeling constantly exhausted ... when your body doesn’t have enough healthy red blood cells. You may either have too few ...

  20. Darbepoetin alfa for anemia with myelodysplastic syndrome.

    PubMed

    Seastone, David J; Gerds, Aaron T

    2015-04-01

    The myelodysplastic syndromes are characterized by refractory cytopenias that lead to symptomatic anemia, bleeding, and increased risk for infections. For almost two decades, the use of darbepoetin and other erythropoietin stimulating agents to treat symptomatic anemia in lower-risk myelodysplastic syndromes has been a standard of care. This practice is supported by numerous Phase I/II studies and one Phase III study demonstrating the benefit of using erythropoietin stimulating agents alone, or in combination with granulocyte colony stimulating factor, for treatment of symptomatic anemia with the goal of decreasing red blood cell transfusion requirements. This review summarizes the published experience regarding the use of erythropoietin stimulating agents, with a special focus on darbepoetin, in patients with myelodysplastic syndrome and symptomatic anemia. PMID:25579702

  1. Stroke in sickle cell anemia: alternative etiologies.

    PubMed

    Dowling, Michael M; Quinn, Charles T; Rogers, Zora R; Journeycake, Janna M

    2009-08-01

    Stroke is common in children with sickle cell anemia, but is rarely attributed to the traditional causes of stroke identified in other children. An 11-year-old girl with sickle cell anemia presented with severe headache and was found to have recurrent bilateral multifocal strokes in a cardioembolic pattern. Evaluation revealed the presence of a patent foramen ovale, antiphospholipid antibodies, and elevations in factor VIII and lipoprotein(a). Sickle cell anemia is itself a hypercoagulable state with potential for increased right heart pressures, both of which predispose to paradoxical embolization via right-to-left intracardiac shunting of emboli, thus causing stroke. The present case suggests that the more traditional etiologies for pediatric stroke may also cause stroke in children with sickle cell anemia. PMID:19589461

  2. Therapeutic use of a receptor mimic probiotic reduces intestinal Shiga toxin levels in a piglet model of hemolytic uremic syndrome

    PubMed Central

    2014-01-01

    Background Hemolytic uremic syndrome (HUS) is a systemic and potentially fatal complication of gastroenteritis secondary to Shiga toxin-producing enterohemorrhagic Escherichia coli (EHEC) infection characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal damage. Shiga toxin (Stx), the toxin principle in HUS, is produced locally within the gut following EHEC colonization and is disseminated via the vasculature. Clinical development of HUS currently has no effective treatment and is a leading cause of renal failure in children. Novel post-exposure therapies are currently needed for HUS; therefore, the purpose of this study was to investigate the efficacy of a Stx receptor mimic probiotic in a porcine model of HUS. Edema disease, an infection of swine caused by host adapted Shiga toxin-producing Escherichia coli (STEC) and mediated by Shiga toxin 2e (Stx2e), shares many pathogenic similarities to HUS. In this study, three-week old piglets were inoculated with STEC and 24 hours later treated twice daily with a probiotic expressing an oligosaccharide receptor mimic for Stx2e to determine if the probiotic could reduce intestinal toxin levels. Methods Piglets were orally inoculated with 1010 CFU of STEC strain S1191 eight days after weaning. Beginning day 1 post-inoculation, piglets were treated orally twice daily with 5?×?1011 CFU of either the receptor mimic probiotic or a sham probiotic for 10 days. Intestinal Stx2e levels were assessed daily via Vero cell assay. The efficacy of the probiotic at reducing intestinal Stx2e, vascular lesions, and clinical disease was evaluated with repeated measures ANOVA and Fisher’s exact test as appropriate. Results The probiotic significantly reduced intestinal Stx2e, as reflected by decreased fecal toxin titers on days 3–8 post-inoculation (p?

  3. Successful Treatment of Severe Anemia using Erythropoietin in a Jehovah Witness with Non-Hodgkin Lymphoma

    PubMed Central

    Agapidou, Alexandra; Vakalopoulou, Sofia; Papadopoulou, Theodosia; Chadjiaggelidou, Christina; Garypidou, Vasileia

    2014-01-01

    Blood transfusion many times works in a life-saving way when a patient is facing a critical situation. However, some patients, such as Jehovah’s Witnesses, may refuse their administration because it opposes to their religion beliefs. Thus, clinicians are forced to respect patients’ preferences and seek other treatments in order to overcome the obstacle of the transfusion. In 1989, recombinant human erythropoietin (rHuEPO) was approved by the United States Food and Drug Administration (FDA) for the treatment of anemia associated with chronic renal failure. This is an amino acid glycol-protein that stimulates red blood cell production in the same manner as endogenous erythropoietin. Other treatment indications approved by the FDA include anemia due to chronic kidney disease, anemia secondary to zidovudine therapy in patients with human immunodeficiency virus infection, and anemia secondary to cancer chemotherapy. The drug also has been used for many off-label indications. Many Jehovah’s Witnesses have accepted rHuEPO as a treatment option to maintain and enhance erythropoiesis. This paper reports the case of a 57-year-old Jehovah’s Witness man, who was diagnosed with severe anemia due to aggressive non Hodgkin lymphoma and refused transfusion of blood; thanks to the treatment with rHuEPO he has managed to complete chemotherapy and has survived a life threatening situation. PMID:25568760

  4. Successful Treatment of Severe Anemia using Erythropoietin in a Jehovah Witness with Non-Hodgkin Lymphoma.

    PubMed

    Agapidou, Alexandra; Vakalopoulou, Sofia; Papadopoulou, Theodosia; Chadjiaggelidou, Christina; Garypidou, Vasileia

    2014-11-19

    Blood transfusion many times works in a life-saving way when a patient is facing a critical situation. However, some patients, such as Jehovah's Witnesses, may refuse their administration because it opposes to their religion beliefs. Thus, clinicians are forced to respect patients' preferences and seek other treatments in order to overcome the obstacle of the transfusion. In 1989, recombinant human erythropoietin (rHuEPO) was approved by the United States Food and Drug Administration (FDA) for the treatment of anemia associated with chronic renal failure. This is an amino acid glycol-protein that stimulates red blood cell production in the same manner as endogenous erythropoietin. Other treatment indications approved by the FDA include anemia due to chronic kidney disease, anemia secondary to zidovudine therapy in patients with human immunodeficiency virus infection, and anemia secondary to cancer chemotherapy. The drug also has been used for many off-label indications. Many Jehovah's Witnesses have accepted rHuEPO as a treatment option to maintain and enhance erythropoiesis. This paper reports the case of a 57-year-old Jehovah's Witness man, who was diagnosed with severe anemia due to aggressive non Hodgkin lymphoma and refused transfusion of blood; thanks to the treatment with rHuEPO he has managed to complete chemotherapy and has survived a life threatening situation. PMID:25568760

  5. Characteristics of sickle cell anemia in Yemen.

    PubMed

    Al-Ghazaly, Jameel; Al-Dubai, Waled; Abdullah, Munasser; Al-Mahagri, Altaf; Al-Gharasi, Leila

    2013-01-01

    We studied 136 males and 105 females with sickle cell anemia to determine the characteristics of the disease in Yemen. Their mean age [± SD (standard deviation)] was 12.8 ± 9.5 years (range: 9 months-40 years). Taiz, Hudaydah and Hajjah governorates, in the South-Central and the Northwestern provinces, showed the highest prevalence. Eighty percent of the patients had family history of the disease, 73.0% patients had history of parental consanguinity and 20.7% of death of relative(s) due to the disease; 5.4% patients were older than 30 years of age. Pain, jaundice and infection were the most frequent features. Splenomegaly, cholelithiasis, osteomyelitis, acute chest syndrome (ACS), osteonecrosis and stroke occurred in 12.0, 9.5, 8.7, 6.6, 6.6 and 2.9%, respectively. Priapism and leg ulcers were rare. The mean laboratory values (obtained in the steady state) were: hemoglobin (Hb) 7.9 g/dL, WBC 14.08 × 10(9)/L, platelet 460 × 10(9)/L, reticulocytes 14.5%, lactate dehydrogenase (LDH) 597 U/L, Hb F (?2?2) 16.69%, Hb S [?6(A3)Glu?Val, GAG>GTG] 77.31% and Hb A(2) (?2?2) 1.47%, respectively. There was no significant difference between South-Central and Northwestern provinces regarding clinical events and hematological parameters. PMID:23234436

  6. Gametophyte development in Anemia mexicana Klotzsch

    E-print Network

    Nester, Joan Elizabeth

    1979-01-01

    GAMETOPHYTE DEVELOPMENT IN ANEMIA MEXICANA KLOTZSCH A Thesis by JOAN ELIZABETH NESTER Submitted to the Graduate College of Texas A&M University in partial fulfillment of the requirement for the degree of MASTER OF SCIENCE August 1979 Major... Subject: Botany GAMETOPHYTE DEVELOPMENT IN ANEMIA MEXICANA KLOTZSCH A Thesis by JOAN ELIZABETH NESTER Approved as sty e and content by: rl, udge(~u Chatrman of Committee ember e ber Head o Department August 1979 ABSTRACT Gametophyte Development...

  7. Anemia in cats infected by Trypanosoma evansi

    Microsoft Academic Search

    Aleksandro Schafer da Silva; Patrícia Wolkmer; Márcio Marcio Costa; Sonia Terezinha dos Anjos Lopes; Silvia Gonzalez Monteiro

    2011-01-01

    One of the main characteristics of the trypanosomosis is the development of anemia, although its pathogenesis still remains\\u000a unclear. Therefore, the objective of this study was to discuss the possible pathogenesis of anemia in the infection by Trypanosoma evansi in cats. A study using an experimental model with T. evansi-infected cats reported changes in serum iron levels, alterations in the

  8. Family structure and child anemia in Mexico.

    PubMed

    Schmeer, Kammi K

    2013-10-01

    Utilizing longitudinal data from the nationally-representative Mexico Family Life Survey, this study assesses the association between family structure and iron-deficient anemia among children ages 3-12 in Mexico. The longitudinal models (n = 4649), which control for baseline anemia status and allow for consideration of family structure transitions, suggest that children living in stable-cohabiting and single-mother families and those who have recently experienced a parental union dissolution have higher odds of anemia than those in stable-married, father-present family structures. Interaction effects indicate that unmarried family contexts have stronger associations with anemia in older children (over age five); and, that the negative effects of parental union dissolution are exacerbated in poorer households. Resident maternal grandparents have a significant beneficial effect on child anemia independent of parental family structure. These results highlight the importance of family structure for child micronutrient deficiencies and suggest that understanding social processes within households may be critical to preventing child anemia in Mexico. PMID:23294876

  9. Adolescent anemia screening during ambulatory pediatric visits in the United States.

    PubMed

    Sekhar, Deepa L; Murray-Kolb, Laura E; Wang, Luojun; Kunselman, Allen R; Paul, Ian M

    2015-04-01

    The Centers for Disease Control and Prevention recommends anemia screening for reproductive age women every 5-10 years and annually for those with risk factors. Due to the lower risk of anemia among males, screening for men is recommended only if risk factors exist. The study objective was to examine health care professionals' current anemia screening patterns for male and female adolescents. Data are from the 2001 -2004 National Ambulatory Medical Care Survey, a nationally representative sample of ambulatory visits to primary care practices. The frequency of anemia screening during preventive care visits by 12-21-year-olds was estimated by sex using a reported hemoglobin/hematocrit or complete blood count as an indicator of screening. Multivariable logistic regression identified patient, provider and practice-level factors associated with screening. During the study period, 1,263 preventive care visits occurred for 12-21 year-olds. In bivariate analysis, higher odds of anemia screening were observed for both younger females (OR 1.85; 95 % CI 1.09-3.14) and older males [1.83 (1.02-3.26)] compared to older females (?16 years). In the multivariable model, odds of screening increased with non-white race [3.29 (1.84-5.88)], tobacco use [3.57 (1.94-6.58)], longer visit length [1.03 (1.01-1.06)], and practice site acceptance of managed care plans [2.08 (1.04-4.14)]. Patient sex and age were not statistically significant predictors of screening. Although anemia is more prevalent among older adolescent females, they were not more likely to be screened. This suggests providers are not targeting groups at highest risk of anemia for screening. PMID:25194577

  10. Drag reducing polymers as simple indicators of hemolytic potential in biomechanical devices

    E-print Network

    Shieh, Sarah

    2009-01-01

    An experimental study was carried out to determine if drag reducing polymers can be simple indicators of hemolytic potential in biomechanical devices. Specifically, three different blood pumps, known as a left ventricle ...

  11. Sigma E Regulators Control Hemolytic Activity and Virulence in a Shrimp Pathogenic Vibrio harveyi

    E-print Network

    Rattanama, Pimonsri

    Members of the genus Vibrio are important marine and aquaculture pathogens. Hemolytic activity has been identified as a virulence factor in many pathogenic vibrios including V. cholerae, V. parahaemolyticus, V. alginolyticus, ...

  12. The evolution of hemolytic saponin content in wild and cultivated Alfalfa ( Medicago sativa , Fabaceae)

    Microsoft Academic Search

    Ernest Small; Marian Jurzysta; Constance Nozzolillo

    1990-01-01

    Hemolytic saponin content was determined of the leaves of 1213 plants of different variants ofMedicago sativa s.l. (including wild and cultivated alfalfa), and a close ally,M. papillosa. The latter species had a much higher content than any of the groups ofM. sativa. Medicago sativa ssp. caerulea, the most important ancestor of alfalfa, had a very low content of hemolytic saponins.

  13. Hemolytic properties of synthetic nano- and porous silica particles: the effect of surface properties and the protection by the plasma corona.

    PubMed

    Shi, J; Hedberg, Y; Lundin, M; Odnevall Wallinder, I; Karlsson, H L; Möller, L

    2012-09-01

    Novel silica materials incorporating nanotechnology are promising materials for biomedical applications, but their novel properties may also bring unforeseen behavior in biological systems. Micro-size silica is well documented to induce hemolysis, but little is known about the hemolytic activities of nanostructured silica materials. In this study, the hemolytic properties of synthetic amorphous silica nanoparticles with primary sizes of 7-14 nm (hydrophilic vs. hydrophobic), 5-15 nm, 20 nm and 50 nm, and model meso/macroporous silica particles with pore diameters of 40 nm and 170 nm are investigated. A crystalline silica sample (0.5-10 ?m) is included for benchmarking purposes. Special emphasis is given to investigations of how the temperature and solution complexity (solvent, plasma), as well as the physicochemical properties (such as size, surface charge, hydrophobicity and other surface properties), link to the hemolytic activities of these particles. Results suggests the potential importance of small size and large external surface area, as well as surface charge/structure, in the hemolysis of silica particles. Furthermore, a significant correlation is observed between the hemolytic profile of red blood cells and the cytotoxicity profile of human promyelocytic leukemia cells (HL-60) induced by nano- and porous silica particles, suggesting a potential universal mechanism of action. Importantly, the results generated suggest that the protective effect of plasma towards silica nanoparticle-induced hemolysis as well as cytotoxicity is primarily due to the protein/lipid layer shielding the silica particle surface. These results will assist the rational design of hemocompatible silica particles for biomedical applications. PMID:22522009

  14. A novel atypical hemolytic uremic syndrome-associated hybrid CFHR1/CFH gene encoding a fusion protein that antagonizes factor H-dependent complement regulation.

    PubMed

    Valoti, Elisabetta; Alberti, Marta; Tortajada, Agustin; Garcia-Fernandez, Jesus; Gastoldi, Sara; Besso, Luca; Bresin, Elena; Remuzzi, Giuseppe; Rodriguez de Cordoba, Santiago; Noris, Marina

    2015-01-01

    Genomic aberrations affecting the genes encoding factor H (FH) and the five FH-related proteins (FHRs) have been described in patients with atypical hemolytic uremic syndrome (aHUS), a rare condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ARF. These genomic rearrangements occur through nonallelic homologous recombinations caused by the presence of repeated homologous sequences in CFH and CFHR1-R5 genes. In this study, we found heterozygous genomic rearrangements among CFH and CFHR genes in 4.5% of patients with aHUS. CFH/CFHR rearrangements were associated with poor clinical prognosis and high risk of post-transplant recurrence. Five patients carried known CFH/CFHR1 genes, but we found a duplication leading to a novel CFHR1/CFH hybrid gene in a family with two affected subjects. The resulting fusion protein contains the first four short consensus repeats of FHR1 and the terminal short consensus repeat 20 of FH. In an FH-dependent hemolysis assay, we showed that the hybrid protein causes sheep erythrocyte lysis. Functional analysis of the FHR1 fraction purified from serum of heterozygous carriers of the CFHR1/CFH hybrid gene indicated that the FHR1/FH hybrid protein acts as a competitive antagonist of FH. Furthermore, sera from carriers of the hybrid CFHR1/CFH gene induced more C5b-9 deposition on endothelial cells than control serum. These results suggest that this novel genomic hybrid mediates disease pathogenesis through dysregulation of complement at the endothelial cell surface. We recommend that genetic screening of aHUS includes analysis of CFH and CFHR rearrangements, particularly before a kidney transplant. PMID:24904082

  15. Serogroup-specific bacterial engineered glycoproteins as novel antigenic targets for diagnosis of shiga toxin-producing-escherichia coli-associated hemolytic-uremic syndrome.

    PubMed

    Melli, Luciano J; Ciocchini, Andrés E; Caillava, Ana J; Vozza, Nicolás; Chinen, Isabel; Rivas, Marta; Feldman, Mario F; Ugalde, Juan E; Comerci, Diego J

    2015-02-01

    Human infection with Shiga toxin-producing Escherichia coli (STEC) is a major cause of postdiarrheal hemolytic-uremic syndrome (HUS), a life-threatening condition characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. E. coli O157:H7 is the dominant STEC serotype associated with HUS worldwide, although non-O157 STEC serogroups can cause a similar disease. The detection of anti-O157 E. coli lipopolysaccharide (LPS) antibodies in combination with stool culture and detection of free fecal Shiga toxin considerably improves the diagnosis of STEC infections. In the present study, we exploited a bacterial glycoengineering technology to develop recombinant glycoproteins consisting of the O157, O145, or O121 polysaccharide attached to a carrier protein as serogroup-specific antigens for the serological diagnosis of STEC-associated HUS. Our results demonstrate that using these antigens in indirect ELISAs (glyco-iELISAs), it is possible to clearly discriminate between STEC O157-, O145-, and O121-infected patients and healthy children, as well as to confirm the diagnosis in HUS patients for whom the classical diagnostic procedures failed. Interestingly, a specific IgM response was detected in almost all the analyzed samples, indicating that it is possible to detect the infection in the early stages of the disease. Additionally, in all the culture-positive HUS patients, the serotype identified by glyco-iELISAs was in accordance with the serotype of the isolated strain, indicating that these antigens are valuable not only for diagnosing HUS caused by the O157, O145, and O121 serogroups but also for serotyping and guiding the subsequent steps to confirm diagnosis. PMID:25472487

  16. Deletions affecting hemolytic and toxin activities of Bordetella pertussis adenylate cyclase.

    PubMed Central

    Bellalou, J; Sakamoto, H; Ladant, D; Geoffroy, C; Ullmann, A

    1990-01-01

    The Bordetella pertussis cyaA gene encodes a virulence factor which is a bifunctional protein exhibiting calmodulin-sensitive adenylate cyclase and hemolytic activities (P. Glaser, H. Sakamoto, J. Bellahov, A. Ullmann, and A. Danchin, EMBO J. 7:3997-4004, 1988). We characterized the hemolytic and toxin activities of the 200-kilodalton (kDa) bifunctional (CyaA) protein and showed that, whether cell associated or secreted, the 200-kDa CyaA protein carries hemolytic and toxin functions. The catalytically active 45-kDa form of adenylate cyclase released by proteolytic digestion of the 200-kDa CyaA protein displayed neither hemolytic nor toxin activities. We constructed in-phase deletions in the 3' region of the cyaA gene, which presumably carries the hemolytic determinant, and showed that the resulting proteins exhibited wild-type adenylate cyclase activity and were secreted without processing into culture supernatants. The hemolytic activities of these mutant CyaA proteins were severely reduced, and their toxin activities were abolished. These results suggest that the structural integrity of the 200-kDa CyaA protein is necessary for toxin activity and that distinct structural determinants within the CyaA protein are involved in secretion, pore formation, and entry into target cells. Images PMID:2401563

  17. Hemolytic and antimicrobial activities differ among saponin-rich extracts from guar, quillaja, yucca, and soybean.

    PubMed

    Hassan, Sherif M; Byrd, James A; Cartwright, Aubry L; Bailey, Chris A

    2010-10-01

    Hemolytic and antibacterial activities of eight serial concentrations ranged from 5-666 microg/mL of saponin-rich extracts from guar meal (GM), quillaja, yucca, and soybean were tested in 96-well plates and read by enzyme-linked immunosorbent assay plate-well as 650 nm. Hemolytic assay used a 1% suspension of chicken red blood cells with water and phosphate buffered saline as positive and negative controls, respectively. Antibacterial activity against Staphylococcus aureus, Salmonella typhimurium, and Escherichia coli were evaluated using ampicillin and bacteria without saponin-rich extract as positive and negative controls, respectively. The 100% MeOH GM and commercial quillaja saponin-rich extracts were significantly the highest in both hemolytic and antibacterial activities against all bacteria at the same concentration tested. Soybean saponin-rich extract had no antibacterial activity against any of the bacteria at the concentrations tested while yucca saponin-rich extract had no antibacterial activity against the gram-negative bacteria at the concentrations tested. GM and quillaja saponin-rich extracts were hemolytic, while yucca and soybean saponin-rich extracts were not hemolytic at the concentrations tested. No saponin-rich extract source had antibacterial activity against S. typhimurium or E. coli at the concentrations tested. Both GM and quillaja saponin-rich extracts exhibited antibacterial activity against S. aureus. Saponin-rich extracts from different plant sources have different hemolytic and antibacterial activities. PMID:19915999

  18. Studies on anemia in F1 hybrid mice injected with parental strain lymphoid cells.

    PubMed

    HARRISS, E; CURRIE, C; KRISS, J P; KAPLAN, H S

    1961-06-01

    The survival of (51)Cr-labeled erythrocytes has been studied in F(1) hybrid mice in which wasting disease was produced by injection of parental lymphoid cells taken either from lymph nodes and thymus or from the spleen. Coincident with the development of the disease syndrome, there occurred a severe anemia accompanied by a sudden loss of circulating labeled erythrocytes, whether host or parental. This finding suggests that the anemia is not due solely to specific immunologic reaction of donor tissue against host erythrocytes. PMID:13711856

  19. Epoetin beta for the treatment of chemotherapy-induced anemia: an update

    PubMed Central

    Galli, Luca; Ricci, Clara; Egan, Colin Gerard

    2015-01-01

    Epoetin beta belongs to the class of erythropoiesis-stimulating agents (ESAs) that are currently available to treat anemic patients receiving chemotherapy. Chemotherapy-induced anemia affects a high percentage of cancer patients and, due to its negative effects on disease outcome and the patient’s quality of life, should be treated when first diagnosed. Initial trials with ESAs have shown efficacy in improving quality of life and reducing the need for blood transfusions in patients with chemotherapy-induced anemia. However, recent meta-analyses have provided conflicting data on the impact of ESAs on survival and tumor progression. Here we provide an overview of these recent data and review the role of epoetin beta in the treatment of chemotherapy-induced anemia over the past 20 years. PMID:25784818

  20. Reticulocyte maturity indices in iron deficiency anemia

    PubMed Central

    Wollmann, Muriel; Gerzson, Branca Maria Cerezer; Schwert, Vanessa; Figuera, Rafael Weber; Ritzel, Guilherme de Oliveira

    2014-01-01

    Objective The aim of this study was to analyze the reticulocyte maturity indices (low, medium, and high fluorescence ratios) in iron deficient 1- to 6-year-old children, and identify the prevalence of iron deficiency anemia in this population. Methods The present study included 39 subjects, divided into two groups: control subjects (n = 33), and subjects with iron deficiency anemia (n = 6). The results were analyzed by Student's t-test for comparison of means. Differences were considered significant when two-tailed p-value < 0.05. Results Subjects with iron deficiency anemia presented increases in the proportion of mean (10.3 ± 4.7% vs. 6.0 ± 3.4%; p-value = 0.003), and high fluorescence reticulocytes (2.3 ± 0.87% vs. 0.9 ± 0.9%; p-value = 0.03) compared to the control group. The prevalence of anemia in this population was 15% (n = 6). Conclusion The indices related to immaturity of reticulocytes are higher in the presence of iron deficiency, thus demonstrating a deficiency in the raw material to form hemoglobin and are, therefore, possible early markers of iron deficiency and anemia. We emphasize the need to standardize these indices for use in clinical practice and lab test results. PMID:24624032

  1. Functional characterization of two novel non-synonymous alterations in CD46 and a Q950H change in factor H found in atypical hemolytic uremic syndrome patients.

    PubMed

    Mohlin, Frida C; Nilsson, Sara C; Levart, Tanja Kersnik; Golubovic, Ema; Rusai, Krisztina; Müller-Sacherer, Thomas; Arbeiter, Klaus; Pállinger, Éva; Szarvas, Nóra; Csuka, Dorottya; Szilágyi, Ágnes; Villoutreix, Bruno O; Prohászka, Zoltán; Blom, Anna M

    2015-06-01

    Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation, characterized by hemolytic anemia, thrombocytopenia and acute renal failure. Mutations in complement inhibitors are major risk factors for development of aHUS. The three aHUS patients reported in this study had several previously identified alterations in complement inhibitors; e.g. risk haplotypes in CD46 and factor H but we also identified two novel heterozygous non-synonymous CD46 alterations (p.E142Q and p.G259V). Presence of G259V caused decreased expression of the recombinant mutant CD46 compared to wild type (WT). Western blot analysis showed that the majority of the expressed G259V protein was in the precursor form, suggesting that it is processed less efficiently than WT. Low CD46 expression on the surface of the patient's neutrophils confirmed the in vitro results. Further, G259V had a substantially impaired ability to act as a cofactor to factor I, in the degradation of both C3b and C4b. The E142Q mutant showed neither decreased expression nor impaired function. Two of the patients also had a heterozygous non-synonymous alteration in factor H (p.Q950H), reported previously in aHUS but not functionally tested. This variant showed moderately impaired function in hemolytic assays, both using patient sera and recombinant proteins. The recombinant Q950H also showed a somewhat decreased expression compared to WT but the complement inhibitory function in fluid phase was normal. Taken together, we report a novel CD46 alteration showing both a decreased protein expression and substantially impaired cofactor function (G259V) and another without an effect on expression or cofactor function (E142Q). Moreover, mild consequences of a previously reported aHUS associated rare variant in factor H (Q950H) was also revealed, underlining the clear need for functional characterization of each new aHUS associated mutation. PMID:25733390

  2. 75 FR 49029 - Medicare Program; End-Stage Renal Disease Prospective Payment System

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-12

    ...hereditary hemolytic anemias and sickle cell anemia; lymphoma; Hepatitis B; and...hereditary hemolytic anemias/sickle cell anemia, monoclonal gammopathy, and myelodysplastic...pneumonia, gastrointestinal bleeding, sickle cell anemia, cancer, myelodysplastic...

  3. Clostridium sordellii as a Cause of Fatal Septic Shock in a Child with Hemolytic Uremic Syndrome

    PubMed Central

    Beyers, Rebekah; Baldwin, Michael

    2014-01-01

    Clostridium sordellii is a toxin producing ubiquitous gram-positive anaerobe, mainly associated with trauma, soft tissue skin infections, and gynecologic infection. We report a unique case of a new strain of Clostridium sordellii (not present in the Center for Disease Control (CDC) database) infection induced toxic shock syndrome in a previously healthy two-year-old male with colitis-related hemolytic uremic syndrome (HUS). The patient presented with dehydration, vomiting, and bloody diarrhea. He was transferred to the pediatric critical care unit (PICU) for initiation of peritoneal dialysis (PD). Due to increased edema and intolerance of PD, he was transitioned to hemodialysis through a femoral vascular catheter. He subsequently developed severe septic shock with persistent leukocytosis and hypotension, resulting in subsequent death. Stool culture confirmed Shiga toxin producing Escherichia coli 0157:H7. A blood culture was positively identified for Clostridium sordellii. Clostridium sordelli is rarely reported in children; to our knowledge this is the first case described in a pediatric patient with HUS. PMID:24891968

  4. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.

    PubMed

    Feng, Shuju; Eyler, Stephen J; Zhang, Yuzhou; Maga, Tara; Nester, Carla M; Kroll, Michael H; Smith, Richard J; Afshar-Kharghan, Vahid

    2013-08-22

    Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. We investigated whether genetic variations in the ADAMTS13 gene partially explain the reduced activity known to occur in some patients with aHUS. We measured complement activity and ADAMTS13 function, and completed mutation screening of multiple complement genes and ADAMTS13 in a large cohort of aHUS patients. In over 50% of patients we identified complement gene mutations. Surprisingly, 80% of patients also carried at least 1 nonsynonymous change in ADAMTS13, and in 38% of patients, multiple ADAMTS13 variations were found. Six of the 9 amino acid substitutions in ADAMTS13 were common single nucleotide polymorphisms; however, 3 variants-A747V, V832M, and R1096H- were rare, with minor allele frequencies of 0.0094%, 0.5%, and 0.32%, respectively. Reduced complement and ADAMTS13 activity (<60% of normal activity) were found in over 60% and 50% of patients, respectively. We concluded that partial ADAMTS13 deficiency is a common finding in aHUS patients and that genetic screening and functional tests of ADAMTS13 should be considered in these patients. PMID:23847193

  5. Hemorrhagic retinopathy in an infant with hemolytic-uremic syndrome.

    PubMed

    Geraissate, João Caetano Ávila; Yamamoto, Rafael Eidi; Isaac, David Leonardo Cruvinel; Ávila, Marcos Pereira de

    2014-12-01

    We describe the case of a 23-month-old female infant with a diagnosis of hemolytic uremic syndrome (HUS) and hemorrhagic retinopathy. The patient had a past history of abdominal pain, bloody diarrhea, and acute renal failure. On ophthalmologic examination, indirect ophthalmoscopy revealed extensive areas of flame-shaped hemorrhage, cotton wool spots, macular edema and optic nerve head neovascularization in both eyes. Fluorescein angiography showed severe bilateral retinal ischemia and neovascularization leakage in disk. The patient, who had the visual acuity of 20/1000 in the right eye (OD) and 20/540 in the left eye (OS) at the first examination, was treated with panretinal photocoagulation (PRP) and presented at the end of the 6th month of follow-up improvement to 20/540 in OD and 20/270 in OS. There was also a regression of disc neovascularization, hemorrhages and macular edema. Despite intense retinal ischemia, there were no complications related to angiogenesis such as vitreous hemorrhage and/or neovascular glaucoma. We describe, in this report, the association between hemorrhagic retinopathy with features of Purtscher-like disease and HUS. PMID:25627190

  6. Cholesterol-dependent hemolytic activity of Passiflora quadrangularis leaves.

    PubMed

    Yuldasheva, L N; Carvalho, E B; Catanho, M-T J A; Krasilnikov, O V

    2005-07-01

    Plants used in traditional medicine are rich sources of hemolysins and cytolysins, which are potential bactericidal and anticancer drugs. The present study demonstrates for the first time the presence of a hemolysin in the leaves of Passiflora quadrangularis L. This hemolysin is heat stable, resistant to trypsin treatment, has the capacity to froth, and acts very rapidly. The hemolysin activity is dose-dependent, with a slope greater than 1 in a double-logarithmic plot. Polyethylene glycols of high molecular weight were able to reduce the rate of hemolysis, while liposomes containing cholesterol completely inhibited it. In contrast, liposomes containing phosphatidylcholine were ineffective. The Passiflora hemolysin markedly increased the conductance of planar lipid bilayers containing cholesterol but was ineffective in cholesterol-free bilayers. Successive extraction of the crude hemolysin with n-hexane, chloroform, ethyl acetate, and n-butanol resulted in a 10-fold purification, with the hemolytic activity being recovered in the n-butanol fraction. The data suggest that membrane cholesterol is the primary target for this hemolysin and that several hemolysin molecules form a large transmembrane water pore. The properties of the Passiflora hemolysin, such as its frothing ability, positive color reaction with vanillin, selective extraction with n-butanol, HPLC profile, cholesterol-dependent membrane susceptibility, formation of a stable complex with cholesterol, and rapid erythrocyte lysis kinetics indicate that it is probably a saponin. PMID:16007277

  7. Management of hemolytic-uremic syndrome in children

    PubMed Central

    Grisaru, Silviu

    2014-01-01

    Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%), this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there are no proven treatment options that can directly inactivate the toxin or effectively interfere with the cascade of destructive events triggered by the toxin once it gains access to the bloodstream and binds its receptor. However, HUS is self-limited, and effective supportive management during the acute phase is proven to be a life saver for children affected by HUS. A minority of childhood HUS cases, approximately 5%, are caused by various genetic mutations causing uncontrolled activation of the complement system. These children, who used to have a poor prognosis leading to end-stage renal disease, now have access to exciting new treatment options that can preserve kidney function and avoid disease recurrences. This review provides a summary of the current knowledge on the epidemiology, pathophysiology, and clinical presentation of childhood HUS, focusing on a practical approach to best management measures. PMID:24966691

  8. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome

    PubMed Central

    Feng, Shuju; Eyler, Stephen J.; Zhang, Yuzhou; Maga, Tara; Nester, Carla M.; Kroll, Michael H.

    2013-01-01

    Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. We investigated whether genetic variations in the ADAMTS13 gene partially explain the reduced activity known to occur in some patients with aHUS. We measured complement activity and ADAMTS13 function, and completed mutation screening of multiple complement genes and ADAMTS13 in a large cohort of aHUS patients. In over 50% of patients we identified complement gene mutations. Surprisingly, 80% of patients also carried at least 1 nonsynonymous change in ADAMTS13, and in 38% of patients, multiple ADAMTS13 variations were found. Six of the 9 amino acid substitutions in ADAMTS13 were common single nucleotide polymorphisms; however, 3 variants—A747V, V832M, and R1096H— were rare, with minor allele frequencies of 0.0094%, 0.5%, and 0.32%, respectively. Reduced complement and ADAMTS13 activity (<60% of normal activity) were found in over 60% and 50% of patients, respectively. We concluded that partial ADAMTS13 deficiency is a common finding in aHUS patients and that genetic screening and functional tests of ADAMTS13 should be considered in these patients. PMID:23847193

  9. Treatment of iron deficiency anemia associated with gastrointestinal tract diseases

    PubMed Central

    Bayraktar, Ulas D; Bayraktar, Soley

    2010-01-01

    The gastrointestinal (GI) tract is a common site of bleeding that may lead to iron deficiency anemia (IDA). Treatment of IDA depends on severity and acuity of patients’ signs and symptoms. While red blood cell transfusions may be required in hemodynamically unstable patients, transfusions should be avoided in chronically anemic patients due to their potential side effects and cost. Iron studies need to be performed after episodes of GI bleeding and stores need to be replenished before anemia develops. Oral iron preparations are efficacious but poorly tolerated due to non-absorbed iron-mediated GI side effects. However, oral iron dose may be reduced with no effect on its efficacy while decreasing side effects and patient discontinuation rates. Parenteral iron therapy replenishes iron stores quicker and is better tolerated than oral therapy. Serious hypersensitive reactions are very rare with new intravenous preparations. While data on worsening of inflammatory bowel disease (IBD) activity by oral iron therapy are not conclusive, parenteral iron therapy still seems to be advantageous in the treatment of IDA in patients with IBD, because oral iron may not be sufficient to overcome the chronic blood loss and GI side effects of oral iron which may mimic IBD exacerbation. Finally, we believe the choice of oral vs parenteral iron therapy in patients with IBD should primarily depend on acuity and severity of patients’ signs and symptoms. PMID:20533591

  10. N-terminal amphipathic helix as a trigger of hemolytic activity in antimicrobial peptides: a case study in latarcins.

    PubMed

    Polyansky, Anton A; Vassilevski, Alexander A; Volynsky, Pavel E; Vorontsova, Olga V; Samsonova, Olga V; Egorova, Natalya S; Krylov, Nicolay A; Feofanov, Alexei V; Arseniev, Alexander S; Grishin, Eugene V; Efremov, Roman G

    2009-07-21

    In silico structural analyses of sets of alpha-helical antimicrobial peptides (AMPs) are performed. Differences between hemolytic and non-hemolytic AMPs are revealed in organization of their N-terminal region. A parameter related to hydrophobicity of the N-terminal part is proposed as a measure of the peptide propensity to exhibit hemolytic and other unwanted cytotoxic activities. Based on the information acquired, a rational approach for selective removal of these properties in AMPs is suggested. A proof of concept is gained through engineering specific mutations that resulted in elimination of the hemolytic activity of AMPs (latarcins) while leaving the beneficial antimicrobial effect intact. PMID:19563807

  11. Anemia in hospitalized patients with pulmonary tuberculosis*

    PubMed Central

    Oliveira, Marina Gribel; Delogo, Karina Neves; de Oliveira, Hedi Marinho de Melo Gomes; Ruffino-Netto, Antonio; Kritski, Afranio Lineu; Oliveira, Martha Maria

    2014-01-01

    OBJECTIVE: To describe the prevalence of anemia and of its types in hospitalized patients with pulmonary tuberculosis. METHODS: This was a descriptive, longitudinal study involving pulmonary tuberculosis inpatients at one of two tuberculosis referral hospitals in the city of Rio de Janeiro, Brazil. We evaluated body mass index (BMI), triceps skinfold thickness (TST), arm muscle area (AMA), ESR, mean corpuscular volume, and red blood cell distribution width (RDW), as well as the levels of C-reactive protein, hemoglobin, transferrin, and ferritin. RESULTS: We included 166 patients, 126 (75.9%) of whom were male. The mean age was 39.0 ± 10.7 years. Not all data were available for all patients: 18.7% were HIV positive; 64.7% were alcoholic; the prevalences of anemia of chronic disease and iron deficiency anemia were, respectively, 75.9% and 2.4%; and 68.7% had low body weight (mean BMI = 18.21 kg/m2). On the basis of TST and AMA, 126 (78.7%) of 160 patients and 138 (87.9%) of 157 patients, respectively, were considered malnourished. Anemia was found to be associated with the following: male gender (p = 0.03); low weight (p = 0.0004); low mean corpuscular volume (p = 0.03);high RDW (p = 0; 0003); high ferritin (p = 0.0005); and high ESR (p = 0.004). We also found significant differences between anemic and non-anemic patients in terms of BMI (p = 0.04), DCT (p = 0.003), and ESR (p < 0.001). CONCLUSIONS: In this sample, high proportions of pulmonary tuberculosis patients were classified as underweight and malnourished, and there was a high prevalence of anemia of chronic disease. In addition, anemia was associated with high ESR and malnutrition. PMID:25210963

  12. Risk factors associated with anemia, iron deficiency and iron deficiency anemia in rural Nepali pregnant women.

    PubMed

    Makhoul, Zeina; Taren, Douglas; Duncan, Burris; Pandey, Pooja; Thomson, Cynthia; Winzerling, Joy; Muramoto, Myra; Shrestha, Ram

    2012-05-01

    We conducted a cross sectional study to investigate risk factors associated with severe anemia [hemoglobin (Hb) < 8.0 g dl(-1)] and poor iron status among Nepali pregnant women. Socio-demographic, anthropometric, health and dietary data were collected from 3,531 women living in the southeastern plains of Nepal. Stool samples were analyzed for intestinal helminthes. Dark adaptation was assessed using the Night Vision Threshold Test (NVTT). Hb levels were measured in all subjects to detect anemia and the soluble transferrin receptor (sTfR) was measured among a subsample of 479 women. The iron status categories were: 1) normal (Hb> or = 11.0 g/dl and sTfR < or = 8.5 mg/l); 2) anemia without iron deficiency (Hb<11.0 g/dl and sTfR < or = 8.5 mg/l); 3) iron deficiency without anemia (Hb > or = 11.0 g/dl and sTfR>8.5 mg/l); and 4) iron deficiency anemia (IDA): (Hb<11.0 g/dl and sTfR>8.5 mg/l). Factors associated with severe anemia and poor iron status were determined using logistic regression. Hookworm infection increased the risk for developing severe anemia [adjusted odds ratio (AOR): 4.26; 95% CI 1.67-10.89; p<0.01] and IDA [relative risk ratio (RRR): 2.18; 95% CI 1.14-4.16; p<0.05]. Impaired dark adaptation was a common risk factor for iron deficiency with and without anemia. Intake of iron supplements as tablets and/or tonic was protective against severe anemia, anemia without iron deficiency and IDA. Dietary heme iron was significantly associated with iron deficiency without anemia (RRR: 0.1; 95% CI 0.02-0.47; p<0.01). These results indicate the risk factors varied by classification and multiple approaches are needed to reduce anemia and associated nutrient deficiencies. PMID:23077854

  13. Managing Anemia in the Cancer Patient: Old Problems, Future Solutions

    Microsoft Academic Search

    MICHAEL S. GORDON

    Anemia and associated symptoms commonly mani- fest in cancer patients and may have a considerable impact on outcomes. Preliminary studies suggest that overall survival and locoregional control following radi- ation therapy may be compromised by anemia, and recent preliminary data also suggest that anemia may be related to poorer outcomes following chemotherapy. Health-related quality of life of cancer patients is

  14. Short Report Menstruation Does Not Cause Anemia: Endometrial Thickness

    E-print Network

    Lummaa, Virpi

    Short Report Menstruation Does Not Cause Anemia: Endometrial Thickness Correlates Positively for iron-deficiency anemia. This study tested whether normal, premenopausal women's luteal endometrial), and therefore whether a high ET put women at risk for anemia. Endometrial thickness can be con- sidered

  15. Iron deficiency anemia in heart failure.

    PubMed

    Arora, Natasha P; Ghali, Jalal K

    2013-07-01

    Anemia and iron deficiency are quite prevalent in patients with heart failure (HF) and may overlap. Both anemia and iron deficiency are associated with worse symptoms and adverse clinical outcomes. In the past few years, there has been an enormous interest in the subject of iron deficiency and its management in patients with HF. In this review, the etiology and relevance of iron deficiency, iron metabolism in the setting of HF, studies on iron supplementation in patients with HF and potential cardiovascular effects of subclinical iron overload are discussed. PMID:22948485

  16. Genetic modulation of sickle cell anemia

    SciTech Connect

    Steinberg, M.H. [Univ. of Mississippi School of Medicine, Jackson, MS (United States)

    1995-05-01

    Sickle cell anemia, a common disorder associated with reduced life span of the red blood cell and vasoocclusive events, is caused by a mutation in the {Beta}-hemoglobin gene. Yet, despite this genetic homogeneity, the phenotype of the disease is heterogeneous. This suggests the modulating influence of associated inherited traits. Some of these may influence the accumulation of fetal hemoglobin, a hemoglobin type that interferes with the polymerization of sickle hemoglobin. Another inherited trait determines the accumulation of {alpha}-globin chains. This review focuses on potential genetic regulators of the phenotype of sickle cell anemia. 125 refs., 6 figs., 3 tabs.

  17. The Invisible Malady: Sickle Cell Anemia

    PubMed Central

    Savitt, Todd L.

    1981-01-01

    Though several articles have appeared on the history of sickle cell anemia in the United States, none has dealt with the dissemination of information from the scientific community to the public. It is an interesting commentary on our society that 60 years have passed before this important but racially oriented disease has reached the public forum. In this article, the author tries to describe the major events in the history of sickle cell anemia and to explain why it has not been publicized. PMID:7021863

  18. Characteristics of hemolytic activity induced by skin secretions of the frog Kaloula pulchra hainana

    PubMed Central

    2013-01-01

    Background The hemolytic activity of skin secretions obtained by stimulating the frog Kaloula pulchra hainana with diethyl ether was tested using human, cattle, rabbit, and chicken erythrocytes. The skin secretions had a significant concentration-dependent hemolytic effect on erythrocytes. The hemolytic activity of the skin secretions was studied in the presence of osmotic protectants (polyethylene glycols and carbohydrates), cations (Mg2+, Ca2+, Ba2+, Cu2+, and K+), or antioxidants (ascorbic acid, reduced glutathione, and cysteine). Results Depending on their molecular mass, osmotic protectants effectively inhibited hemolysis. The inhibition of skin hemolysis was observed after treatment with polyethylene glycols (1000, 3400, and 6000 Da). Among divalent cations, only 1 mM Cu2+ markedly inhibited hemolytic activity. Antioxidant compounds slightly reduced the hemolytic activity. Conclusions The results suggested that skin secretions of K. pulchra hainana induce a pore-forming mechanism to form pores with a diameter of 1.36-2.0 nm rather than causing oxidative damage to the erythrocyte membrane. PMID:24499077

  19. Iron-deficiency anemia caused by a proton pump inhibitor.

    PubMed

    Hashimoto, Rintaro; Matsuda, Tomoki; Chonan, Akimichi

    2014-01-01

    A 59-year-old man was orally administered rabeprazole, a proton pump inhibitor (PPI), for gastroesophageal reflux disease, after which he gradually developed iron-deficiency anemia. The anemia did not improve following the administration of ferrous fumarate, and endoscopic screening of the entire gastrointestinal tract, including the small intestine, did not reveal any findings indicating the cause of the anemia. The patient was then switched from rabeprazole to famotidine and the anemia was cured within three months. There is much debate as to whether the long-term use of PPIs causes iron-deficiency. However, this case strongly suggests that PPIs can induce iron-deficiency anemia. PMID:25318791

  20. Diagnosis and treatment of cancer-related anemia.

    PubMed

    Gilreath, Jeffrey A; Stenehjem, David D; Rodgers, George M

    2014-02-01

    Cancer-related anemia (CRA) is due to multiple etiologies, including chemotherapy-induced myelosuppression, blood loss, functional iron deficiency, erythropoietin deficiency due to renal disease, marrow involvement with tumor as well as other factors. The most common treatment options for CRA include iron therapy, erythropoietic-stimulating agents (ESAs), and red cell transfusion. Safety concerns as well as restrictions and reimbursement issues surrounding ESA therapy for CRA have resulted in suboptimal treatment. Similarly, many clinicians are not familiar or comfortable using intravenous iron products to treat functional iron deficiency associated with CRA. This article summarizes our approach to treating CRA and discusses commonly encountered clinical scenarios for which current clinical guidelines do not apply. PMID:24532336

  1. Endometriosis presenting with hemorrhagic ascites, severe anemia, and shock.

    PubMed

    Morgan, Trent L; Tomich, Eric B; Heiner, Jason D

    2013-01-01

    Hemorrhagic ascites due to endometriosis is an exceedingly uncommon diagnosis rarely reported in the medical literature. We present a case of a 27-year-old woman who presented to the emergency department for flank and neck pain and was found to be hypotensive with massive hemorrhagic ascites and severe anemia. After emergency department resuscitation and hospitalization, her condition was found to be due to complications of endometriosis. A paracentesis of more than 4000 mL of bloody ascitic fluid revealed no evidence of cancer, and she was discharged on hospital day 3 with hormone therapy and no recurrence of symptoms upon outpatient follow-up. This case illustrates the clinical management, diagnostic approach, and underlying etiology of an infrequent but life-threatening complication of endometriosis. PMID:22809773

  2. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  3. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  4. Hb F in sickle cell anemia

    Microsoft Academic Search

    A. D. Adekile; T. H. J. Huisman

    1993-01-01

    We have reviewed the methodology for an accurate quantitation of Hb F in the blood of patients with sickle cell anemia, values observed in hundreds of patients of different (racial or ethnic) backgrounds and with differences in severity of the disease, and the various factors that affect the level of Hb F. The latter include sex, age, genetic background or

  5. Respiration Unaffected by Anemia in Chemodenervated Cats

    Microsoft Academic Search

    Herbert L. Borison; Jeffrey H. Hurst; Lawrence E. McCarthy

    1982-01-01

    The effect of acute isovolemic anemia on the control of respiration was examined in chemodenervated decerebrate cats to determine whether the peripheral chemoreceptors are normally responsible for the reported respiratory indifference to a reduction in blood hemoglobin. The tidal volume\\/CO2 response lines and the respiratory frequency\\/ tidal volume relationship were determined during inhalation of air and of oxygen with the

  6. Regulation of equine infectious anemia virus expression

    Microsoft Academic Search

    Wendy Maury; S. Dak

    1998-01-01

    Equine infectious anemia virus (EIAV) is an ungulate lentivirus that is related to human immunodeficiency virus (HIV). Much of the understanding of lentiviral gene regulation comes from studies using HIV. HIV studies have provided insights into molecular regulation of EIAV expression; however, much of the regulation of EIAV expression stands in stark contrast to that of HIV. This review provides

  7. [Disseminated lymphangiomatosis: A rare cause of anemia].

    PubMed

    Ben Abdallah Chabchoub, R; Kamoun, F; Hidouri, S; Nouri, A; Hachicha, M; Mahfoudh, A

    2015-04-01

    Disseminated lymphangiomatosis is a congenital lymphovenous vascular malformation. It can occur in different regions, some of which are unusual. The treatment of this vascular malformation is based on surgical excision, sclerotherapy, or recombinant interferon therapy. We report the case of disseminated lymphangiomatosis in a 13-year-old girl who presented with anemia. PMID:25725973

  8. Anemia identified one month after renal transplantation is predictive of anemia identified after twelve months.

    PubMed

    Imamovi?, Goran; Zerem, Enver; Omerovi?, Safet

    2009-08-01

    The aim of this study was to evaluate whether anemia identified earlier than 3 months postengraftment in modern era could be predictive of anemia at 12 months. Cross-sectional and cohort studies based on retrospective analysis of existing clinical records were performed. Data on recipient's age at transplantation, follow-up serum creatinine (SCR) and hemoglobin (Hb) on day 7 (D7), at month 1 (M1) and at month 3 (M3) postengraftment were collected. Outcome was anemia identified at 12 months (M12) postengraftment. There were 75 patients on D7, 74 at M1 and 61 at M3. Multiple linear regression model that included recipient's age at transplantation, Hb and creatinine on D7 and tested the risk for anemia at M12 retained only the age in the model, with the coefficient of 0,84 (P=0,001). The same model at M1 retained Hb and age, with the coefficients of 0,26 (P=0,03) and 0,81 (P=0,0002), respectively and at M3 it retained Hb and age, with the coefficients of 0,41 (P=0,004) and 0,70 (P=0,003), respectively. Anemia identified at M1 after renal transplantation is predictive of anemia at M12. PMID:19754477

  9. Alloantibodies to a paternally derived RBC KEL antigen lead to hemolytic disease of the fetus/newborn in a murine model

    PubMed Central

    Stowell, Sean R.; Henry, Kate L.; Smith, Nicole H.; Hudson, Krystalyn E.; Halverson, Greg R.; Park, Jaekeun C.; Bennett, Ashley M.; Girard-Pierce, Kathryn R.; Arthur, C. Maridith; Bunting, Silvia T.; Zimring, James C.

    2013-01-01

    Exposure to nonself red blood cell (RBC) antigens, either from transfusion or pregnancy, may result in alloimmunization and incompatible RBC clearance. First described as a pregnancy complication 80 years ago, hemolytic disease of the fetus and newborn (HDFN) is caused by alloimmunization to paternally derived RBC antigens. Despite the morbidity/mortality of HDFN, women at risk for RBC alloimmunization have few therapeutic options. Given that alloantibodies to antigens in the KEL family are among the most clinically significant, we developed a murine model with RBC-specific expression of the human KEL antigen to evaluate the impact of maternal/fetal KEL incompatibility. After exposure to fetal KEL RBCs during successive pregnancies with KEL-positive males, 21 of 21 wild-type female mice developed anti-KEL alloantibodies; intrauterine fetal anemia and/or demise occurred in a subset of KEL-positive pups born to wild type, but not agammaglobulinemic mothers. Similar to previous observations in humans, pregnancy-associated alloantibodies were detrimental in a transfusion setting, and transfusion-associated alloantibodies were detrimental in a pregnancy setting. This is the first pregnancy-associated HDFN model described to date, which will serve as a platform to develop targeted therapies to prevent and/or mitigate the dangers of RBC alloantibodies to fetuses and newborns. PMID:23801629

  10. Genetics Home Reference: Fanconi anemia

    MedlinePLUS

    ... when the process of making new copies of DNA, called DNA replication, is blocked due to DNA damage. The FA pathway sends certain proteins to the area of damage, which trigger DNA repair so DNA replication can continue. The FA ...

  11. Studies on the hemolytic activity of tentacle extracts of jellyfish Rhopilema esculentum Kishinouye: application of orthogonal test.

    PubMed

    Yu, Huahua; Xing, Ronge; Liu, Song; Li, Cuiping; Guo, Zhanyong; Li, Pengcheng

    2007-02-20

    The present work is first reporting the hemolytic activity of venom from jellyfish Rhopilema esculentum Kishinouye extracted by different phosphate buffer solutions and incubated at different temperature according to the orthogonal test L6(1) x 3(6). Of the seven controllable independent variables, incubated temperature and phenylmethylsulfonyl fluoride (PMSF) had strongest effect on the hemolytic activity. PMID:16890282

  12. Acceleration of epithelial cell syndecan-1 shedding by anthrax hemolytic virulence factors

    PubMed Central

    Popova, Taissia G; Millis, Bryan; Bradburne, Chris; Nazarenko, Svetlana; Bailey, Charles; Chandhoke, Vikas; Popov, Serguei G

    2006-01-01

    Background It has been recently reported that major pathogens Staphylococcus aureus and Pseudomonas aeruginosa accelerate a normal process of cell surface syndecan-1 (Synd1) ectodomain shedding as a mechanism of host damage due to the production of shedding-inducing virulence factors. We tested if acceleration of Synd1 shedding takes place in vitro upon treatment of epithelial cells with B. anthracis hemolysins, as well as in vivo during anthrax infection in mice. Results The isolated anthrax hemolytic proteins AnlB (sphingomyelinase) and AnlO (cholesterol-binding pore-forming factor), as well as ClnA (B. cereus homolog of B. anthracis phosphatidyl choline-preferring phospholipase C) cause accelerated shedding of Synd1 and E-cadherin from epithelial cells and compromise epithelial barrier integrity within a few hours. In comparison with hemolysins in a similar range of concentrations, anthrax lethal toxin (LT) also accelerates shedding albeit at slower rate. Individual components of LT, lethal factor and protective antigen are inactive with regard to shedding. Inhibition experiments favor a hypothesis that activities of tested bacterial shedding inducers converge on the stimulation of cytoplasmic tyrosine kinases of the Syk family, ultimately leading to activation of cellular sheddase. Both LT and AnlO modulate ERK1/2 and p38 MAPK signaling pathways, while JNK pathway seems to be irrelevant to accelerated shedding. Accelerated shedding of Synd1 also takes place in DBA/2 mice challenged with Bacillus anthracis (Sterne) spores. Elevated levels of shed ectodomain are readily detectable in circulation after 24 h. Conclusion The concerted acceleration of shedding by several virulence factors could represent a new pathogenic mechanism contributing to disruption of epithelial or endothelial integrity, hemorrhage, edema and abnormal cell signaling during anthrax infection. PMID:16464252

  13. Serum transferrin receptor distinguishes the anemia of chronic disease from iron deficiency anemia.

    PubMed

    Ferguson, B J; Skikne, B S; Simpson, K M; Baynes, R D; Cook, J D

    1992-04-01

    Recent studies have shown that the serum transferrin receptor is a sensitive, quantitative measure of tissue iron deficiency. This study was undertaken to determine the serum transferrin receptor's ability to distinguish iron-deficiency anemia from the anemia of chronic inflammation and to identify iron deficiency in patients with liver disease. The mean transferrin receptor level in 17 normal controls was 5.36 +/- 0.82 mg/L compared with 13.91 +/- 4.63 mg/L in 17 patients with iron-deficiency anemia (p less than 0.001). The mean serum receptor level was normal in all 20 patients with acute infection, including five with acute hepatitis, and was also normal in 8 of 10 anemic patients with chronic liver disease. Receptor levels were in the normal range in all but 4 of 41 patients with anemia of chronic disease. We conclude that unlike serum ferritin levels, which are disproportionately elevated in relation to iron stores in patients with inflammation or liver disease, the serum transferrin receptor level is not affected by these disorders and is therefore a reliable laboratory index of iron deficiency anemia. PMID:1583389

  14. HIV Symptom Burden and Anemia among HIV-Positive Individuals: Cross-Sectional Results of a Community-Based Positive Living with HIV (POLH) Study in Nepal

    PubMed Central

    Martin, Catherine; Poudel-Tandukar, Kalpana; Poudel, Krishna C.

    2014-01-01

    Background Previous research has reported high rates of anemia in people living with HIV/AIDS (PLWHA) in hospital or tertiary care settings. The objective of this community-based study was to measure the prevalence of anemia and describe the risk factors, with a specific emphasis on HIV symptom burden, in PLWHA in the Kathmandu Valley, Nepal. Methods We conducted a cross-sectional survey of 319 PLWHA residing in the Kathmandu Valley, Nepal. We recruited participants from five non-governmental organizations in the Kathmandu Valley. Descriptive statistics and multivariable logistic regression analyses were used. Results Our study found a 55.8% prevalence of anemia in PLWHA in the Kathmandu Valley. The prevalence of anemia among the participants with first, second, third, and fourth quartiles of HIV symptom burden was 44.8%, 49.3%, 60.3%, and 69.6%, respectively. Compared to the participants with lowest level of HIV symptom burden, the participants with highest level of HIV symptom burden were more likely to have anemia (adjusted odds ratio?=?2.14; 95% confidence interval?=?1.07 to 4.30). Conclusion Due to a high prevalence of anemia in a community-based sample of PLWHA, HIV patients should be counseled on their risk of developing anemia and encouraged to seek timely care for HIV symptoms. PMID:25551656

  15. Cerebral vasculopathy in children with sickle cell anemia.

    PubMed

    Fasano, Ross M; Meier, Emily R; Hulbert, Monica L

    2015-01-01

    Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population. However, despite aggressive hematological management, many children with cerebral vasculopathy due to SCA have progressive vasculopathy and recurrent strokes; therefore, more effective therapies, such as revascularization surgery and curative hematopoietic stem cell transplant, are urgently needed. PMID:25294561

  16. Fanconi anemia associated with moyamoya disease in Saudi Arabia

    PubMed Central

    Al-Hawsawi, Zakaria M.; Al-Zaid, Mohamed A.; Barnawi, Ashwaq I.; Yassine, Saadeddine M.

    2015-01-01

    We report a 10-year-old Saudi girl who has Fanconi anemia (FA) and was admitted due to acute hemiplegia, of the right side. She had a previous attack of left side hemiplegia that resolved spontaneously. The brain magnetic resonance angiography showed a cerebrovascular pattern of moyamoya disease. She underwent partially matched related donor stem cell transplantation (SCT), but unfortunately died 3 months later with post SCT complications. The association of moyamoya disease with FA is uncommon, and is rarely reported in the literature. Although this condition may be acquired, it is considered a truly congenital defect in FA, and to identify the etiology of this association furthermore genetic mutation analysis is needed. PMID:25719591

  17. Schilling evaluation of pernicious anemia: current status

    SciTech Connect

    Zuckier, L.S.; Chervu, L.R.

    1984-09-01

    The Schilling examination remains a popular means of evaluating in vivo absorption of vitamin B/sub 12/. When absorption is abnormally low, the test may be repeated with addition to exogenous intrinsic factor (IF) in order to correct the IF deficiency that characterizes pernicious anemia. A dual-isotope variation provides a means of performing both stages of the test simultaneously, thereby speeding up the test and reducing dependence on complete urine collection. In vivo studies indicate that, when administered simultaneously, the absorption of unbound B/sub 12/ is elevated, and IF-bound B/sub 12/ is reduced, in pernicious-anemia patients, relative to the classic two-stage examination. A number of clinical studies indicate significant difficulty in resolving clincial diagnoses with the dual-tracer test. An algorithm is offered for selecting the most suitable variation of the Schilling test to improve the accuracy of test results and the ease of performance.

  18. Animal Models of Anemia of Inflammation

    PubMed Central

    Rivera, Seth; Ganz, Tomas

    2009-01-01

    Anemia of inflammation (AI) is a complex multi-organ response to inflammatory disorders. Because AI can result from many infectious and non-infectious inflammatory diseases, multiple mechanisms may contribute to its pathogenesis including iron restriction, direct erythropoietic suppression, shortened red cell survival or frank hemolysis. Animal models have been helpful in the study of the mechanisms of AI and its potential treatments but each model reflects distinct aspects of this heterogeneous syndrome. It is therefore important to study a variety of models of AI. This review focuses on the use of infectious and noninfectious mouse models of inflammation that have been shown to manifest anemia. We review many of the models reported in the literature or developed in our laboratory, and discuss their respective merits and drawbacks. PMID:19786203

  19. Diagnosis and classification of pernicious anemia.

    PubMed

    Bizzaro, Nicola; Antico, Antonio

    2014-01-01

    Pernicious anemia (PA) is a complex disorder consisting of hematological, gastric and immunological alterations. Diagnosis of PA relies on histologically proven atrophic body gastritis, peripheral blood examination showing megaloblastic anemia with hypersegmented neutrophils, cobalamin deficiency and antibodies to intrinsic factor and to gastric parietal cells. Anti-parietal cell antibodies are found in 90% of patients with PA, but have low specificity and are seen in atrophic gastritis without megaloblastic anemia as well as in various autoimmune disorders. Anti-intrinsic factor antibodies are less sensitive, being found in only 60% of patients with PA, but are considered highly specific for PA. The incidence of PA increases with age and is rare in persons younger than 30 years of age. The highest prevalence is seen in Northern Europeans, especially those in the United Kingdom and Scandinavia, although PA has been reported in virtually every ethnic group. Because of the complexity of the diagnosis, PA prevalence is probably underestimated and no reliable data are available on the risk of gastric cancer as the end-stage evolution of atrophic gastritis in these patients. PMID:24424200

  20. THE TRANSFER OF RAT ANEMIA TO NORMAL ANIMALS

    PubMed Central

    Ford, William W.; Eliot, Calista P.

    1928-01-01

    Fifty-eight white and hooded rats have been splenectomized and all of them have shown a more or less severe anemia and an infection of the red blood cells with Bartonella muris. Another strain of white rats obtained from Littlestown showed no anemia and no bartonellas in the blood after splenectomy, until exposed to infected rats. Others of these Littlestown rats, kept in the laboratory for some time before operation and exposed to infected rats, came down with bartonella anemia within 6 days after splenectomy. Whole blood or the washed red blood corpuscles from splenectomized rats which show bartonellas and anemia will produce a similar condition in young rats when injected intraperitoneally. Adult rats of strains which harbor the virus (as demonstrated by splenectomy) cannot be infected by injection. Intravenous inoculation of young normal rabbits with blood from an infected rat will sometimes produce a similar infection and anemia in the rabbit, and the virus can then be transferred back to young rats. The virus of rat anemia may be transferred from young normal rat to young normal rat with the appearance of Bartonella muris and the production of anemia. In the early transfers the disease may be fatal, but it usually becomes milder in successive passages. Although we have not yet been able to cultivate Bartonella muris and prove its etiological relationship to rat anemia by inoculation of cultures, we have added to the evidence that Bartonella muris is the cause of the anemia. Washed red blood corpuscles, containing bartonellas, will produce the anemia in the usual way while plasma from the same cells will either fail to produce it altogether or only after a prolonged incubation period. Blood heated to 57°C. for ½ hour fails to produce anemia or the appearance of bartonellas in the blood of inoculated animals. From these observations the following conclusions may be drawn: 1. All rats which harbor Bartonella muris ratti come down with a more or less severe anemia after splenectomy. 2. Young rats which have not yet developed an immunity undergo the typical anemia after intraperitoneal injection of blood from a splenectomized animal in the early stages of the anemia. 3. Young rabbits may show bartonellas and develop anemia following intravenous inoculation of infected blood. 4. The virus of rat anemia and Bartonella muris ratti may be transferred from normal animal to normal animal for successive generations. Such strains have now been transferred for five, nine and thirty generations. 5. The resistance of rats to bartonella anemia is almost wholly dependent on the spleen. Other organs do not take over this function of protection as shown by the relapse of splenectomized rats many months after recovery. Young rats which have recovered from an attack of anemia are not protected by this previous infection from the invasion of the virus following splenectomy. Adult splenic tissue mixed with infected blood before injection does not inhibit or neutralize the virus. 6. The virus of rat anemia is highly contagious and rats exposed to infection acquire it in some unknown way. 7. Bartonella muris ratti represents the virus of rat anemia or at least cannot be separated from the virus because: (a) The anemia in splenectomized and injected animals is always preceded by the appearance of bartonellas and the grade of anemia is proportional to the degree of infection with bartonellas. (b) Washed corpuscles containing bartonellas always produce anemia. Plasma either fails to do so, or produces a mild anemia after a long incubation period with a few bartonellas in the blood. (c) The thermal death point of virus and bartonella is the same. PMID:19869499

  1. Assessment of phytochemicals, antioxidant, anti-lipid peroxidation and anti-hemolytic activity of extract and various fractions of Maytenus royleanus leaves

    PubMed Central

    2013-01-01

    Background Maytenus royleanus is traditionally used in gastro-intestinal disorders. The aim of this study was to evaluate the methanol extract of leaves and its derived fractions for various antioxidant assays and for its potential against lipid peroxidation and hemolytic activity. Methods Various parameters including scavenging of free-radicals (DPPH, ABTS, hydroxyl and superoxide radical), hydrogen peroxide scavenging, Fe3+ to Fe2+ reducing capacity, total antioxidant capacity, anti-lipid peroxidation and anti-hemolytic activity were investigated. Methanol extract and its derived fractions were also subjected for chemical constituents. LC-MS was also performed on the methanol extract. Results Qualitative analysis of methanol extract exhibited the presence of alkaloids, anthraquinones, cardiac glycosides, coumarins, flavonoids, saponins, phlobatannins, tannins and terpenoids. LC-MS chromatogram indicated the composition of diverse compounds including flavonoids, phenolics and phytoestrogens. Methanol extract, its ethyl acetate and n-butanol fractions constituted the highest amount of total phenolic and flavonoid contents and showed a strong correlation coefficient with the IC50 values for the scavenging of DPPH, hydrogen peroxide radicals, superoxide radicals, anti-lipid peroxidation and anti-hemolytic efficacy. Moreover, n-butanol fraction showed the highest scavenging activity for ABTS radicals and for reduction of Fe3+ to Fe2+. Conclusions Present results suggested the therapeutic potential of Maytenus royleanus leaves, in particular, methanol extract, ethyl acetate and n-butanol fraction as therapeutic agent against free-radical associated damages. The protective potential of the extract and or fraction may be attributed due to the high concentration of phenolic, flavonoid, tannins and terpenoids. PMID:23800043

  2. Antiproliferative, Cytotoxic and Hemolytic Activities of a Triterpene Glycoside from Psolus patagonicus and Its Desulfated Analog

    Microsoft Academic Search

    Valeria P. Careaga; Carlos Bueno; Claudia Muniain; Laura Alché; Marta S. Maier

    2009-01-01

    Background: The major triterpene glycoside of the sea cucumber Psolus patagonicus and its desulfated analog were tested for their antiproliferative, cytotoxic and hemolytic activities, and their effect on NF-?B activation. Methods: The antiproliferative action of glycosides 1 and 2 were determined on 3 tumor cell lines. Their effect on the activation of NF-?B was evaluated by indirect immunofluorescence assay staining

  3. A Novel Integrative Conjugative Element Mediates Genetic Transfer from Group G Streptococcus to Other  Hemolytic Streptococci

    Microsoft Academic Search

    Mark R. Davies; Josephine Shera; Gary H. Van Domselaar; Kadaba S. Sriprakash; David J. McMillan

    2009-01-01

    Lateral gene transfer is a significant contributor to the ongoing evolution of many bacterial pathogens, including -hemolytic streptococci. Here we provide the first characterization of a novel integrative conjugative element (ICE), ICESde3396, from Streptococcus dysgalactiae subsp. equisimilis (group G streptococcus (GGS)), a bacterium commonly found in the throat and skin of humans. ICESde3396 is 64 kb in size and encodes

  4. Carbon Nanotubes Inhibit the Hemolytic Activity of the Pore-Forming Toxin Pyolysin

    E-print Network

    Le Roy, Robert J.

    Carbon Nanotubes Inhibit the Hemolytic Activity of the Pore-Forming Toxin Pyolysin Apraku David Article Address correspondence to tangxw@uwaterloo.ca ABSTRACT Functionalized carbon nanotubes have oxidized and non-covalently PEGlyated single-walled carbon nanotubes (SWNTs), which were previously

  5. The hemolytic and physiological activities of mixtures of some phenoxy and organophosphorous herbicides.

    PubMed

    Kleszczy?ska, Halina; Bonarska, Dorota; Bielecki, Krzysztof; Sarapuk, Janusz

    2003-01-01

    Experiments were performed investigating the potential to improve the biological activity of some phenoxy and organophosphorous compounds by using them in binary mixtures. The compounds were: 2,4-dichlorophenoxyacetic acid (1) and its sodium salt (2), dibutyl 1-butylamino-1-cyclohexanephosphonate (3) and diethyl 9-butylamino-9-fluorenephosphonate (4), all widely used as herbicides. There were two test methods: the inhibition of cucumber (Cucumis sativus) growth induced by one single herbicide or by equimolar binary mixtures of herbicides; and, in parallel, the hemolytic efficiency of separate compounds or their mixtures. The hemolytic properties of the compounds were studied as hemolysis is generally a good measure of their toxicity, especially in the case of lipophilic compounds. Pig erythrocytes were used as good models for the determination of toxicity and the kinetics of red blood cell hemolysis. In the plant-based experiments, binary mixtures were found to display additive type toxicity. The compounds' hemolytic activities were of additive or antagonistic types. In some combinations, the addition of a second component did not change the hemolytic efficiency of the first component, and vice versa. PMID:12655357

  6. Identification of a Vibrio furnissii Oligopeptide Permease and Characterization of Its In Vitro Hemolytic Activity? †

    PubMed Central

    Wu, Tung-Kung; Wang, Yu-Kuo; Chen, Yi-Chin; Feng, Jen-Min; Liu, Yen-Hsi; Wang, Ting-Yi

    2007-01-01

    We describe purification and characterization of an oligopeptide permease protein (Hly-OppA) from Vibrio furnissii that has multifaceted functions in solute binding, in in vitro hemolysis, in antibiotic resistance, and as a virulence factor in bacterial pathogenesis. The solute-binding function was revealed by N-terminal and internal peptide sequences of the purified protein and was confirmed by discernible effects on oligopeptide binding, by accumulation of fluorescent substrates, and by fluorescent substrate-antibiotic competition assay experiments. The purified protein exhibited host-specific in vitro hemolytic activity against various mammalian erythrocytes and apparent cytotoxicity in CHO-K1 cells. Recombinant Hly-OppA protein and an anti-Hly-OppA monoclonal antibody exhibited and neutralized the in vitro hemolytic activity, respectively, which further confirmed the hemolytic activity of the gene product. In addition, a V. furnissii hly-oppA knockout mutant caused less mortality than the wild-type strain when it was inoculated into BALB/c mice, indicating the virulence function of this protein. Finally, the in vitro hemolytic activity was also confirmed with homologous ATP-binding cassette-type transporter proteins from other Vibrio species. PMID:17873048

  7. Cytotoxicity and hemolytic activity of jellyfish Nemopilema nomurai (Scyphozoa: Rhizostomeae) venom

    Microsoft Academic Search

    Changkeun Kang; Al Munawir; Mijin Cha; Eun-Tae Sohn; Hyunkyoung Lee; Jong-Shu Kim; Won Duk Yoon; Donghyun Lim; Euikyung Kim

    2009-01-01

    The recent bloom of a giant jellyfish Nemopilema nomurai has caused a danger to sea bathers and fishery damages in the waters of China, Korea, and Japan. The present study investigated the cytotoxic and hemolytic activities of crude venom extract of N. nomurai using a number of in vitro assays. The jellyfish venom showed a much higher cytotoxic activity in

  8. Human protein C concentrate in the treatment of hemolytic uremic syndrome

    E-print Network

    Manstein, Dietmar J.

    Human protein C concentrate in the treatment of hemolytic uremic syndrome Burkhard Wermter1 and abnormal D-dimer and PAI-1 levels could be normalised. All of the patients recovered a nearly normal kidney and the lack of side effects in the treatment with human PC concentrate administration in our patients yield

  9. Comparing micellar, hemolytic, and antibacterial properties of di-and tricarboxyl dendritic amphiphiles

    E-print Network

    Falkinham, Joseph

    amphiphiles Bhadreshkumar B. Maisuria a, , Marcelo L. Actis a,à , Shauntrece N. Hardrict a,§ , Joseph O April 2011 Keywords: Dendritic amphiphiles Staphylococcus aureus MRSA Critical micelle concentrations Hemolytic activities a b s t r a c t Homologous dicarboxyl dendritic amphiphiles--RCONHC(CH3)(CH2CH2COOH)2

  10. LATEST STRATEGY IN RENAL ANEMIA MANAGEMENT IN PERITONEAL DIALYSIS PATIENTS

    Microsoft Academic Search

    Wai Kei Lo

    The target of renal anemia correction with erythropoietin stimulating agents (ESAs) has been traditionally set at a hemoglobin (Hb) level of 11 - 12 g\\/dL. However, a trend has arisen of progressively increasing the Hb level to beyond 12 g\\/dL. Recent randomized control trials (RCTs) on correction of renal anemia in chronic kidney disease patients found that normalization of anemia

  11. A novel ubiquitin ligase is deficient in Fanconi anemia

    Microsoft Academic Search

    Amom Ruhikanta Meetei; Johan P de Winter; Annette L Medhurst; Michael Wallisch; Quinten Waisfisz; Henri J van de Vrugt; Anneke B Oostra; Zhijiang Yan; Chen Ling; Colin E Bishop; Maureen E Hoatlin; Hans Joenje; Weidong Wang

    2003-01-01

    Fanconi anemia is a recessively inherited disease characterized by congenital defects, bone marrow failure and cancer susceptibility. Cells from individuals with Fanconi anemia are highly sensitive to DNA-crosslinking drugs, such as mitomycin C (MMC). Fanconi anemia proteins function in a DNA damage response pathway involving breast cancer susceptibility gene products, BRCA1 and BRCA2 (refs. 1,2). A key step in this

  12. Hepcidin expression in anemia of chronic disease and concomitant iron-deficiency anemia.

    PubMed

    Cheng, Pan-pan; Jiao, Xiao-yang; Wang, Xue-hua; Lin, Jing-hua; Cai, Ying-mu

    2011-03-01

    Hepcidin is a key hormone governing mammalian iron homeostasis and may be directly or indirectly involved in the development of most iron deficiency/overload and inflammation-induced anemia. The objective of this study was to investigate the expression of hepcidin in anemia of chronic disease. To characterize serum hepcidin, iron and inflammatory indicators associated with anemia of chronic disease (ACD), we studied ACD, ACD concomitant iron-deficiency anemia (ACD/IDA), pure IDA and acute inflammation (AcI) patients and analyzed the associations between hepcidin levels and inflammation parameters in various types of anemia. Serum hepcidin levels in patient groups were statistically different, from high to low: ACD, AcI > ACD/IDA > the control > IDA. Serum ferritin levels were significantly increased in ACD and AcI patients but were decreased significantly in ACD/IDA and IDA. Elevated serum EPO concentrations were found in ACD, ACD/IDA and IDA patients but not in AcI patients and the controls. A positive correlation between hepcidin and IL-6 levels only existed in ACD/IDA, AcI and the control groups. A positive correlation between hepcidin and ferritin was marked in the control group, while a negative correlation between hepcidin and ferritin was noted in IDA. The significant negative correlation between hepcidin expression and reticulocyte count was marked in both ACD/IDA and IDA groups. All of these data demonstrated that hepcidin might play role in pathogenesis of ACD, ACD/IDA and IDA, and it could be a potential marker for detection and differentiation of these anemias. PMID:20499129

  13. Treatment of Anemia in Inflammatory Bowel Disease– Systematic Review and Meta-Analysis

    PubMed Central

    Avni, Tomer; Bieber, Amir; Steinmetz, Tali; Leibovici, Leonard; Gafter-Gvili, Anat

    2013-01-01

    Background Anemia is considered the most common systemic complication of inflammatory bowel disease (IBD). We aimed to provide all available evidence regarding the safety and efficacy of therapy existing today to correct anemia in IBD. Methods Systematic review and meta-analysis of randomized controlled trials that compared any treatment for anemia in patients with IBD. We searched electronic databases, conference proceedings and clinical trials registries. Two reviewers independently extracted data from included trials. The primary outcome was the effect of treatment for anemia in IBD on the hemoglobin (Hb) response, defined as rate of patients who achieved an increase of 2 g/dl in Hb concentration at the end of the follow-up. Secondary outcomes included disease severity scores, iron indices, Hb levels, inflammatory markers, adverse effects, and mortality. Dichotomous data were analysed by calculating the relative risk (RR) for each trial with the uncertainty in each result being expressed using 95% confidence intervals (CI). A fixed effect model was used, except in the event of significant heterogeneity between the trials (P<0.10, I2>40%), in which we used a random effects model. Results Nine trials fulfilled the inclusion criteria, to a total of 973 patients. We were able to perform meta-analysis for intravenous (IV) versus oral iron and for ESAs versus placebo. IV iron was associated with a higher rate of achieving Hb response in comparison to oral iron; RR 1.25 (95% CI 1.04–1.51, I2?=?2%, 4 trials), CRP levels and disease activity indexes were not significantly affected by IV iron. IV iron was associated with a decrease in adverse events that required discontinuation of intervention and without an increase in serious adverse. Discussion Treatment for anemia in IBD should include IV iron and not oral iron replacement, due to improved Hb response, no added toxicity and no negative effect on disease activity. PMID:24312441

  14. Experimental comparison of hemolytic and nonhemolytic Ornithobacterium rhinotracheale field isolates in vivo.

    PubMed

    Walters, J; Evans, R; LeRoith, T; Sriranganathan, N; McElroy, A; Pierson, F W

    2014-03-01

    Ornithobacterium rhinotracheale (ORT) is a nonhemolytic, gram-negative, pleomorphic, rod-shaped bacterium that causes upper and lower respiratory tract disease in poultry. Recently, hemolytic strains of ORT have been isolated with increasing frequency from field outbreaks. A study was conducted to determine whether the hemolytic phenotype is associated with any change in virulence. Briefly, 225 turkey poults, vaccinated against hemorrhagic enteritis at 4 wk of age, were randomly divided into nine replicates housed in separate rooms: three sham treatment controls (25 poults/replicate), three challenged with a nonhemolytic (NH) field isolate (24 poults/replicate), and three challenged with a hemolytic (H) field isolate (24 poults/replicate). Nine days postvaccination, poults were inoculated intratracheally with either 0.2 ml sterile phosphate-buffered saline (PBS), 2 x 10(8) colony-forming units (CFU) of the NH isolate in 0.2 ml PBS, or 2 x 10(8) CFU of the H isolate in 0.2 ml PBS. Serum and body weights were obtained at 0, 7, 14, and 21 days postinoculation (dpi). Tissues were taken for culture and histopathology from five randomly selected poults/replicates at 7, 14, and 21 dpi. When compared with poults inoculated with the H isolate or controls, those inoculated with the NH isolate showed a highly significant depression in weight gain at 7 dpi. NH poults also had significantly higher levels of antibody against ORT at 14 and 21 dpi. Reisolations decreased over time and, by 21 dpi, only the NH phenotype could be found. Based on a Likert-type scale, poults inoculated with the NH isolate had significantly higher histopathologic lesion scores in lung tissue at 7, 14, and 21 dpi. Results suggest that nonhemolytic field isolates are more virulent then hemolytic ones. These findings are unusual because hemolytic phenotypes are often more virulent in other bacterial species. PMID:24758117

  15. Iron deficiency anemia--bridging the knowledge and practice gap.

    PubMed

    Shander, Aryeh; Goodnough, Lawrence T; Javidroozi, Mazyar; Auerbach, Michael; Carson, Jeffrey; Ershler, William B; Ghiglione, Mary; Glaspy, John; Lew, Indu

    2014-07-01

    Despite its high prevalence, anemia often does not receive proper clinical attention, and detection, evaluation, and management of iron deficiency anemia and iron-restricted erythropoiesis can possibly be an unmet medical need. A multidisciplinary panel of clinicians with expertise in anemia management convened and reviewed recent published data on prevalence, etiology, and health implications of anemia as well as current therapeutic options and available guidelines on management of anemia across various patient populations and made recommendations on the detection, diagnostic approach, and management of anemia. The available evidence confirms that the prevalence of anemia is high across all populations, especially in hospitalized patients. Anemia is associated with worse clinical outcomes including longer length of hospital stay, diminished quality of life, and increased risk of morbidity and mortality, and it is a modifiable risk factor of allogeneic blood transfusion with its own inherent risks. Iron deficiency is usually present in anemic patients. An algorithm for detection and management of anemia was discussed, which incorporated iron study (with primary emphasis on transferrin saturation), serum creatinine and glomerular filtration rate, and vitamin B12 and folic acid measurements. Management strategies included iron therapy (oral or intravenous), erythropoiesis-stimulating agents, and referral as needed. PMID:24931617

  16. Biomarkers for the differentiation of anemia and their clinical usefulness

    PubMed Central

    Northrop-Clewes, Christine A; Thurnham, David I

    2013-01-01

    The World Health Organization defines anemia as the point at which the amount of hemoglobin in the circulation falls below World Health Organization cutoffs for specific age and sex groups. Anemia is a worldwide problem of complex etiology and is associated with many factors. The purpose of this review was to describe the biomarkers used to identify the nature of anemia in patients and in the community. The important biomarkers are the automated red cell counts, tests for nutritional deficiencies, hemoglobinopathies, and inflammation. Diseases are important potential initiators of anemia, but biomarkers of specific diseases are not included in this review, only the underlying feature common to all disease – namely, inflammation. PMID:23687454

  17. Determinants of anemia among preschool children in rural, western Kenya.

    PubMed

    Foote, Eric M; Sullivan, Kevin M; Ruth, Laird J; Oremo, Jared; Sadumah, Ibrahim; Williams, Thomas N; Suchdev, Parminder S

    2013-04-01

    Although anemia in preschool children is most often attributed to iron deficiency, other nutritional, infectious, and genetic contributors are rarely concurrently measured. In a population-based, cross-sectional survey of 858 children 6-35 months of age in western Kenya, we measured hemoglobin, malaria, inflammation, sickle cell, ?-thalassemia, iron deficiency, vitamin A deficiency, anthropometry, and socio-demographic characteristics. Anemia (Hb < 11 g/dL) and severe anemia (Hb < 7 g/dL) prevalence ratios (PRs) for each exposure were determined using multivariable modeling. Anemia (71.8%) and severe anemia (8.4%) were common. Characteristics most strongly associated with anemia were malaria (PR: 1.7; 95% confidence interval [CI] = 1.5-1.9), iron deficiency (1.3; 1.2-1.4), and homozygous ?-thalassemia (1.3; 1.1-1.4). Characteristics associated with severe anemia were malaria (10.2; 3.5-29.3), inflammation (6.7; 2.3-19.4), and stunting (1.6; 1.0-2.4). Overall 16.8% of anemia cases were associated with malaria, 8.3% with iron deficiency, and 6.1% with inflammation. Interventions should address malaria, iron deficiency, and non-malarial infections to decrease the burden of anemia in this population. PMID:23382166

  18. Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-

    PubMed Central

    Özlü, Onur

    2012-01-01

    A 37-year-old woman diagnosed with sickle cell anemia (SCA), beta (+) thalassemia, Crohn's disease, and liver dysfunction was scheduled for laparoscopic cholecystectomy (LC) due to acute cholecystitis with gall bladder. Regional anesthesia was performed. An epidural catheter was inserted into the 9-10 thoracal epidural space and then 15 ml of 0.5% bupivacaine was injected through the catheter. The level of sensorial analgesia tested with pinprick test reached up to T4. Here we describe the first case of the combination of sickle cell anemia (SCA), beta (+) thalassemia, and Crohn's disease successful anesthetic management with attention to hemodynamics, particularly with regards to liver dysfunction. PMID:23115690

  19. 21 CFR 862.1365 - Glutathione test system.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...Glutathione measurements are used in the diagnosis and treatment of certain drug-induced hemolytic (erythrocyte destroying) anemias due to an inherited enzyme deficiency. (b) Classification. Class I (general controls). The device is exempt...

  20. Iron-Hepcidin Dysmetabolism, Anemia and Renal Hypoxia, Inflammation and Fibrosis in the Remnant Kidney Rat Model

    PubMed Central

    Garrido, Patrícia; Ribeiro, Sandra; Fernandes, João; Vala, Helena; Bronze-da-Rocha, Elsa; Rocha-Pereira, Petronila; Belo, Luís; Costa, Elísio; Santos-Silva, Alice; Reis, Flávio

    2015-01-01

    Anemia is a common complication of chronic kidney disease (CKD) that develops early and its severity increases as renal function declines. It is mainly due to a reduced production of erythropoietin (EPO) by the kidneys; however, there are evidences that iron metabolism disturbances increase as CKD progresses. Our aim was to study the mechanisms underlying the development of anemia of CKD, as well as renal damage, in the remnant kidney rat model of CKD induced by 5/6 nephrectomy. This model of CKD presented a sustained degree of renal dysfunction, with mild and advanced glomerular and tubulointerstitial lesions. Anemia developed 3 weeks after nephrectomy and persisted throughout the protocol. The remnant kidney was still able to produce EPO and the liver showed an increased EPO gene expression. In spite of the increased EPO blood levels, anemia persisted and was linked to low serum iron and transferrin levels, while serum interleukin (IL)-6 and high sensitivity C-reactive protein (hs-CRP) levels showed the absence of systemic inflammation. The increased expression of duodenal ferroportin favours iron absorption; however, serum iron is reduced which might be due to iron leakage through advanced kidney lesions, as showed by tubular iron accumulation. Our data suggest that the persistence of anemia may result from disturbances in iron metabolism and by an altered activity/function of EPO as a result of kidney cell damage and a local inflammatory milieu, as showed by the increased gene expression of different inflammatory proteins in the remnant kidney. In addition, this anemia and the associated kidney hypoxia favour the development of fibrosis, angiogenesis and inflammation that may underlie a resistance to EPO stimuli and reduced iron availability. These findings might contribute to open new windows to identify putative therapeutic targets for this condition, as well as for recombinant human EPO (rHuEPO) resistance, which occurs in a considerable percentage of CKD patients. PMID:25867633

  1. Diagnosis and management of iron deficiency anemia.

    PubMed

    Powers, Jacquelyn M; Buchanan, George R

    2014-08-01

    Iron deficiency anemia (IDA) is a common hematologic condition, affecting a substantial proportion of the world's women and young children. Optimal management of IDA requires an accurate diagnosis, identification and correction of the underlying cause, provision of medicinal iron therapy, and confirmation of treatment success. There are limited data to support current treatment approaches regarding oral iron preparation, dosing, monitoring, and duration of therapy. New intravenous iron agents have improved safety profiles, which may foster their increased utilization in the treatment of patients with IDA. Clinical trials focused on improving current treatment standards for IDA are sorely needed. PMID:25064710

  2. Aplastic Anemia in Adolescents and Young Adults

    PubMed Central

    DeZern, Amy E.; Guinan, Eva C.

    2014-01-01

    Adolescent and young adult patient presentations of aplastic anemia require a particular perspective on both diagnosis and treatment. This unique age group necessitates a thorough diagnostic evaluation to ensure the etiology, acquired or inherited, is sufficiently determined. The treatment options include human leukocyte antigen-identical sibling hematopoietic cell transplantation or immunosuppressive therapy, and both require attention to the specific medical and social needs of these adolescents and young adults. Longitudinal surveillance throughout life for the development of late complications of the disease and treatment is mandatory. PMID:25228559

  3. Microradiographic Study of Odontologic Tissues in Cooley's Anemia

    Microsoft Academic Search

    Narendar N. Soni; Frank E. Barbee; Angella D. Ferguson; Barbara A. Parrish

    1966-01-01

    SYNOPSIS IN INTERLINGUA STUDIO MICRORADIOGRAPHIC DE Tissu ODONTOLOGIC IN ANEMIA DE COOLEY.—Le presente studio esseva effectuate pro determinar le effectos de anemia de Cooley super le configuration del mineralisation e le morphologia del dentes e de lor structuras de supporto. Sectiones mollite esseva obtenite ab specimens includitein bioplastico. Examines microradiographic revelava que le dentina esseva mal mineralisate e que inusual

  4. Neurologic Complications After Allogeneic Marrow Transplantation for Sickle Cell Anemia

    Microsoft Academic Search

    Mark C. Walters; Keith M. Sullivan; Francoise Bernaudin; Gerard Souillet; Jean-Pierre Vannier; F. Leonard Johnson; Carl Lenarsky; Darleen Powars; Nancy Bunin; Kwaku Ohene-Frempong; Donna Wall; G. Michel; E. Plouvier; P. Bodigoni; P. Lutz; Jean E. Sanders; Dana C. Matthews; Frederick R. Appelbaum; Rainer Storb

    1995-01-01

    ARROW transplantation from HLA-identical siblings is effective therapy in children with nonmalignant disorders, including aplastic anemia, congenital immunode- ficiency syndromes, thalassemia major, and certain inborn errors of metabolism.',2 Its use in the treatment of sickle cell anemia has been limited to date but initial reports confirm that bone marrow transplantation is curative treatment for this di~order.~\\

  5. Etiology of Strokes in Children with Sickle Cell Anemia

    ERIC Educational Resources Information Center

    DeBaun, Michael R.; Derdeyn, Colin P.; McKinstry, Robert C., III

    2006-01-01

    The most devastating complication of sickle cell anemia is cerebral infarction, affecting [approximately]30% of all individuals with sickle cell anemia. Despite being one of the most common causes of stroke in infants and children, the mechanism of cerebral infarction in this population has not been extensively studied and is poorly understood.…

  6. Correction of Anemia with Epoetin Alfa in Chronic Kidney Disease

    Microsoft Academic Search

    Ajay K. Singh; Lynda Szczech; Kezhen L. Tang; Huiman Barnhart; Shelly Sapp; Marsha Wolfson; Donal Reddan

    2006-01-01

    Background Anemia, a common complication of chronic kidney disease, usually develops as a consequence of erythropoietin deficiency. Recombinant human erythropoietin (epoetin alfa) is indicated for the correction of anemia associated with this condition. However, the optimal level of hemoglobin correction is not defined. Methods In this open-label trial, we studied 1432 patients with chronic kidney disease, 715 of whom were

  7. Liver Function and Anemia Pathogenesis in Iranian Traditional Medicine

    PubMed Central

    Yarjou, Saeedeh; Sadeghpour, Omid; Nazem, Esmail; Emami, Amir Hossein

    2014-01-01

    Background: Nutritional deficiency, bleeding, and inflammation are three main causes of anemia. On the other hand, erythropoietin (EPO) production, iron availability, and a healthy bone marrow are essential for erythropoiesis. Recently, recombinant human erythropoietin (rhEPO) has been used to treat the patients already taking iron supplements. In Iranian traditional medicine literature, much has been written about anemia and its treatment. Objectives: This study aimed to review Iranian traditional medicine theories in the treatment of anemia. Materials and Methods: In the present study, authors have attempted to find the etiology and pathogenesis of anemia in Iranian traditional Medicine Literature and the views of ancient physicians in this field. Results: Our findings proved that Iranian traditional medicine defined anemia as blood humor production dysfunction, which is caused by several reasons mainly hepatic dysfunction. Thus, liver is supposed to be the most important organ in this regard. This will make considering hepatoprotective medicines in anemia even in patients with non-hepatic problems. Conclusions: The article presents the relation between anemia and liver dysfunction viewed by Iranian traditional medicine as an important finding in designing a treatment plan. Despite the new findings on the evaluation of liver function (i.e. laboratory tests), it seems that further studies for earlier evaluation of liver function in anemia are highly required.

  8. Genetics Home Reference: Iron-refractory iron deficiency anemia

    MedlinePLUS

    ... PubMed Recent literature OMIM Genetic disorder catalog Conditions > Iron-refractory iron deficiency anemia On this page: Description Genetic changes ... names Glossary definitions Reviewed July 2014 What is iron-refractory iron deficiency anemia? Iron-refractory iron deficiency ...

  9. Cerebral Blood Flow and Metabolism in Pernicious Anemia

    Microsoft Academic Search

    PERITZ SCHEINBERG

    2010-01-01

    P EIiNICIOUS ANEMIA is a deficiency disease which involves the metab- olism of at least the hematologic, gastiointestinal and nervous systems. Signs and symptoms occurring from the disease may result directly from the effects of the deficiency on cell metabolism or more indirectly l)ecause of the anemia. The tongue and nervous system may he involved with little or no cvi-

  10. Role of Helicobacter pylori infection in Hispanic patients with anemia.

    PubMed

    Ortiz, Melissa; Rosado-Carrión, Bárbara; Bredy, Rafael

    2014-01-01

    Pernicious anemia represents the final phase of a process that begins with Helicobacter pylori-associated gastritis and evolves through progressive levels of atrophy until loss of parietal cell mass. Numerous studies have suggested an association between H. pylori infection, unexplained iron deficiency anemia and cobalamin deficiency. Our research question was to determine whether there is an association between with H. pylori infection and development of anemia in Hispanic patients. This cross sectional pilot study involved data analysis of individual from years 2010-2012 examining the association between H. pylori infection and hemoglobin levels in patients with Hispanic ethnicity. A total of 189 records were evaluated, of which 33 fulfilled the inclusion criteria. The study sample was divided in two groups. Group-A: 5 subjects with H. pylori infection and anemia; Group-B: 28 patients with H. pylori without anemia. Fisher exact test applied between categorical variables to determine the statistical significance of symptoms comparing anemic vs. non-anemic H. pylori infected patients yielded a p = 0.0027. In addition, restoration of anemia in two subjects following eradication therapy without previous iron or cobalamin replacement therapy suggested a potential role of this bacterium in the development of anemia in Hispanics. In conclusion, from the results of this study a potential association between Helicobacter pylori infection and anemia in Hispanic patients is suggested. Restoration of hemoglobin after eradication of bacteria further supports this concept. PMID:25065045

  11. Anemia and decline in physical performance among older persons

    Microsoft Academic Search

    Brenda W. J. H Penninx; Jack M Guralnik; Graziano Onder; Luigi Ferrucci; Robert B Wallace; Marco Pahor

    2003-01-01

    PurposeAnemia is prevalent in old age and is potentially modifiable, but its effects on physical function have not been determined. We examined whether anemia in older persons increases the risk of subsequent decline in physical function, as measured by objective performance-based tests.

  12. Acute psychosis: a presentation of cyanocobalamin deficiency megaloblastic anemia.

    PubMed

    Tripathi, A K; Verma, S P; Himanshu, D

    2010-09-01

    Cyanocobalamin deficiency is not rare in India. Patients present with megaloblastic anemia, pancytopenia and sometimes neuropsychiatric manifestations. Subacute combined degeneration of the cord, peripheral neuropathy, dementia, psychotic depression and paranoid schizophrenia are well reported. We are reporting a case of cyanocobalamine deficiency anemia who presented with acute psychosis which readily reversed on cyanocobalamin replacement. PMID:21886392

  13. Acute Psychosis: A Presentation of Cyanocobalamin Deficiency Megaloblastic Anemia

    PubMed Central

    Tripathi, A. K.; Himanshu, D.

    2010-01-01

    Cyanocobalamin deficiency is not rare in India. Patients present with megaloblastic anemia, pancytopenia and sometimes neuropsychiatric manifestations. Subacute combined degeneration of the cord, peripheral neuropathy, dementia, psychotic depression and paranoid schizophrenia are well reported. We are reporting a case of cyanocobalamine deficiency anemia who presented with acute psychosis which readily reversed on cyanocobalamin replacement. PMID:21886392

  14. Mild Anemia and Pregnancy Outcome in a Swiss Collective

    PubMed Central

    Bencaiova, Gabriela; Breymann, Christian

    2014-01-01

    Background. Over half of all women in the world experience anemia during their pregnancy. Our aim was to investigate the relation between hemoglobin and iron status examined in second trimester and pregnancy outcome. Methods. In a prospective longitudinal study, 382 pregnant women were included. Blood samples were examined for hematological status and serum ferritin between 16 and 20 weeks and for hemoglobin before delivery. The adverse maternal and perinatal outcomes were determined. Regression analysis was performed to establish if anemia and low serum ferritin are risk factors for pregnancy complications. Results. There was no increase of complications in women with mild anemia and in women with depleted iron stores. The finding showed that mild iron deficiency anemia and depleted iron stores are not risk factors for adverse outcomes in iron supplemented women. Conclusions. Mild anemia and depleted iron stores detected early in pregnancy were not associated with adverse maternal and perinatal outcomes in iron supplemented women. PMID:25478229

  15. Purification, characterization and activities of two hemolytic and antibacterial proteins from coelomic fluid of the annelid Eisenia fetida andrei

    Microsoft Academic Search

    Alexandra Milochau; Marguerite Lassègues; Pierre Valembois

    1997-01-01

    The coelomic fluid of the earthworm Eisenia fetida andrei exhibits antibacterial, hemolytic and hemagglutinating activities. These activities are mainly mediated by two proteins, named fetidins, of apparent molecular mass 40 kDa and 45 kDa, respectively. For the first time, the two proteins have been purified to homogeneity from dialysed coelomic fluid by means of anion-exchange chromatography. Three peaks had hemolytic

  16. Portal, superior mesenteric and splenic vein thrombosis secondary to hyperhomocysteinemia with pernicious anemia: a case report

    PubMed Central

    2014-01-01

    Introduction Acute portomesenteric vein thrombosis is an uncommon but serious condition with potential sequelae, such as small-bowel gangrene and end-stage hepatic failure. It is known to be caused by various pro-thrombotic states, including hyperhomocysteinemia. We describe what is, to the best of our knowledge, the first reported case of concomitant thrombosis of portal, superior mesenteric and splenic veins due to hyperhomocysteinemia secondary to pernicious anemia and no other risk factors. Case presentation A 60-year-old Indian man presented with epigastric pain, diarrhea and vomiting. An abdominal imaging scan showed that he had concomitant pernicious anemia and concomitant portal, superior mesenteric and splenic vein thrombosis. A work-up for the patient’s hypercoagulable state revealed hyperhomocysteinemia, an undetectable vitamin B12 level and pernicious anemia with no other thrombophilic state. He developed infarction with perforation of the small bowel and subsequent septic shock with multi-organ dysfunction syndrome, and he ultimately died due to progressive hepatic failure. Conclusion This report demonstrates that pernicious anemia, on its own, can lead to hyperhomocysteinemia significant enough to lead to lethal multiple splanchnic vein thrombosis. Our case also underscores the need to (1) consider portomesenteric thrombosis in the differential diagnosis of epigastric abdominal pain, (2) perform a complete thrombotic work-up to elucidate metabolic abnormalities that could be contributing to a pro-thrombotic state and (3) initiate aggressive measures, including early consideration of multi-visceral transplantation, in order to avoid decompensation and a significant adverse outcome. PMID:25155131

  17. Hemolytic staining of the endocardium of the left heart chambers: a new sign for autopsy diagnosis of freshwater drowning.

    PubMed

    Zátopková, Lenka; Hejna, Petr; Janík, Martin

    2015-03-01

    Despite the availability of modern imaging and molecular tools, traditional autopsy, and laboratory findings remain the gold standard for the diagnosis of drowning. This article presents two cases of freshwater drowning in which hemolytic staining of the endocardium of the left heart chambers was observed at autopsy. One case was a suicidal drowning of an 84-year-old man, and the other case was an accidental drowning of an 86-year-old woman. In both cases, there was marked hemolytic staining of the endocardium of the left atrium and ventricle. The endocardium of the right heart chambers was clear and transparent in appearance. Hemolytic intimal staining of the aortic root was observed in one case. Gettler's test was positive in both cases. Hemolytic discoloration of the endocardium of the left heart chambers after freshwater drowning is analogous to hemolytic staining of the aortic root. Both staining patterns result from the hypo-osmolar hemolysis that occurs in the left heart chambers and systemic circulation after hypotonic fluid passes across the alveolocapillary membrane. Hemolytic discoloration of the endocardium of the left heart chambers at autopsy may support a diagnosis of freshwater drowning. PMID:25326681

  18. Grafting synthetic transmembrane units to the engineered low-toxicity ?-hemolysin to restore its hemolytic activity.

    PubMed

    Ui, Mihoko; Harima, Kousuke; Takei, Toshiaki; Tsumoto, Kouhei; Tabata, Kazuhito V; Noji, Hiroyuki; Endo, Sumire; Akiyama, Kimio; Muraoka, Takahiro; Kinbara, Kazushi

    2014-12-01

    The chemical modification of proteins to provide desirable functions and/or structures broadens their possibilities for use in various applications. Usually, proteins can acquire new functions and characteristics, in addition to their original ones, via the introduction of synthetic functional moieties. Here, we adopted a more radical approach to protein modification, i.e., the replacement of a functional domain of proteins with alternative chemical compounds to build "cyborg proteins." As a proof of concept model, we chose staphylococcal ?-hemolysin (Hla), which is a well-studied, pore-forming toxin. The hemolytic activity of Hla mutants was dramatically decreased by truncation of the stem domain, which forms a ?-barrel pore in the membrane. However, the impaired hemolytic activity was significantly restored by attaching a pyrenyl-maleimide unit to the cysteine residue that was introduced in the remaining stem domain. In contrast, negatively charged fluorescein-maleimide completely abolished the remaining activity of the mutants. PMID:25267196

  19. Multiple cavitations in posterior reversible leukoencephalopathy syndrome associated with hemolytic-uremic syndrome.

    PubMed

    Fujii, Katsunori; Matsuo, Kaoru; Takatani, Tomozumi; Uchikawa, Hideki; Kohno, Yoichi

    2012-04-01

    We describe a 4-year-old boy with posterior reversible leukoencephalopathy syndrome associated with hemolytic-uremic syndrome. He exhibited bloody stool by Escherichia coli O157: H7 infection with acute renal failure. He subsequently presented high blood pressure, followed by visual disturbance and loss of consciousness. Brain MRI revealed bilateral occipital high intensities by T2-weighted images and high value by apparent diffusion coefficient map, thus we made a diagnosis of posterior reversible leukoencephaly syndrome associated with hemolytic-uremic syndrome. In spite of immediate blood pressure control, occipital lesions developed day by day, resulting in multiple subcortical cavitations. Although posterior reversible leukoencephalopathy syndrome is originally characterized by reversible vasogenic edema, this case rarely resulted in irreversible changes with cystic formation. We concluded that precipitating factors, i.e., clotting abnormalities, Shiga toxin, vasospasms and endothelial dysfunction might have synergistically induced irreversible brain infarcts, and caused unusual cavitations. PMID:21723058

  20. Studies on the nature of hemolytic effect induced by ethylene glycol alkyl ethers.

    PubMed

    Starek, Andrzej

    2008-01-01

    The nature of hemolytic effect induced by ethylene glycol alkyl ethers was analyzed taking into account G-6-PDH activity, ATP, pyruvate and thiols levels in peripheral blood of rats treated with single doses of 2-ethoxyethanol and 2-butoxyethanol. In addition, the susceptibility to autoxidation of rat erythrocyte lipids was evaluated. A decrease of ATP level in a dose-dependent manner and an increase in protein- and nonprotein-bound sulfhydryl groups were observed. These results indicate that an acute hemolytic effect of ethylene glycol alkyl ethers is not associated with alterations in G-6-PDH activity and the susceptibility of erythrocyte lipids to autoxidation. Increases in protein- and nonprotein-bound sulfhydryl groups seem to indicate the selective hemolysis of the aged erythrocytes. The increase in pyruvate and thiol levels may protect erythrocytes against the appearance of oxidative stress. PMID:19051582

  1. Correlation among Antioxidant, Antimicrobial, Hemolytic, and Antiproliferative Properties of Leiothrix spiralis Leaves Extract

    PubMed Central

    de Freitas Araújo, Marcelo Gonzaga; Hilário, Felipe; Vilegas, Wagner; dos Santos, Lourdes Campaner; Brunetti, Iguatemy Lourenço; Sotomayor, Claudia Elena; Bauab, Tais Maria

    2012-01-01

    The biological activities of a plant extract depend on a complex sum of individual properties including the antioxidant activity. Several biological activities protect against the harmful action of reactive oxygen species (ROS), and here we focused our attention on the relationship between the biological activities tested and the antioxidant properties. In this study, the total flavonoid content as well as the antioxidant, antimicrobial, hemolytic and cytotoxicity activities of the methanolic extract of Leitothrix spiralis leaves were evaluated. The extract showed a total flavonoid content of 19.26% and the chemical characterization by HPLC-PAD confirmed the presence of flavonoids as the major secondary metabolite compounds. Significant antioxidant activity (IC50 = 1.743 ?g/mL ± 0.063) was demonstrated and was effective against Gram-negative organisms and all Candida strains tested, and showed an ability to inhibit hyphal formation. Non-hemolytic and antiproliferative activity could be demonstrated. PMID:22942765

  2. The epidemiology of aplastic anemia in Thailand

    PubMed Central

    Issaragrisil, Surapol; Kaufman, David W.; Anderson, Theresa; Chansung, Kanchana; Leaverton, Paul E.; Shapiro, Samuel; Young, Neal S.

    2006-01-01

    Aplastic anemia has been linked to environmental exposures, from chemicals and medical drugs to infectious agents. The disease occurs more frequently in Asia than in the West, with incidence rates 2- to 3-fold higher. We report updated results of an epidemiologic study conducted in Thailand from 1989 to 2002, in which 541 patients and 2261 controls were enrolled. Exposures were determined by in-person interview. We observed significantly elevated relative risk estimates for benzene (3.5) and other solvents (2.0) and for sulfonamides (5.6), thiazides (3.8), and mebendazole (3.0). Chloramphenicol use was infrequent, and no significant association was observed. Agricultural pesticides were implicated in Khonkaen (northeastern Thailand). There were significant associations with organophosphates (2.1), DDT (6.7), and carbamates (7.4). We found significant risks for farmers exposed to ducks and geese (3.7) and a borderline association with animal fertilizer (2.1). There was a significant association in Khonkaen with drinking other than bottled or distilled water (2.8). Nonmedical needle exposure was associated in Bangkok and Khonkaen combined (3.8). Most striking was the large etiologic fraction in a rural region accounted for by animal exposures and drinking of water from sources such as wells, rural taps, and rainwater, consistent with an infectious etiology for many cases of aplastic anemia in Thailand. PMID:16254144

  3. The Fanconi anemia pathway and ubiquitin

    PubMed Central

    Jacquemont, Céline; Taniguchi, Toshiyasu

    2007-01-01

    Fanconi anemia (FA) is a rare genetic disorder characterized by aplastic anemia, cancer/leukemia susceptibility and cellular hypersensitivity to DNA crosslinking agents, such as cisplatin. To date, 12 FA gene products have been identified, which cooperate in a common DNA damage-activated signaling pathway regulating DNA repair (the FA pathway). Eight FA proteins form a nuclear complex harboring E3 ubiquitin ligase activity (the FA core complex) that, in response to DNA damage, mediates the monoubiquitylation of the FA protein FANCD2. Monoubiquitylated FANCD2 colocalizes in nuclear foci with proteins involved in DNA repair, including BRCA1, FANCD1/BRCA2, FANCN/PALB2 and RAD51. All these factors are required for cellular resistance to DNA crosslinking agents. The inactivation of the FA pathway has also been observed in a wide variety of human cancers and is implicated in the sensitivity of cancer cells to DNA crosslinking agents. Drugs that inhibit the FA pathway may be useful chemosensitizers in the treatment of cancer. Publication history: Republished from Current BioData's Targeted Proteins database (TPdb; ). PMID:18047734

  4. Postpartum thrombotic microangiopathy revealed as atypical hemolytic uremic syndrome successfully treated with eculizumab: a case report

    PubMed Central

    2014-01-01

    Introduction Differential diagnosis of thrombotic microangiopathies can be difficult. Atypical hemolytic uremic syndrome is a rare, life-threatening disease caused by uncontrolled chronic activation of alternative complement pathway, resulting in microvascular thrombosis, organ ischemia and damage. Prognosis is poor: up to 65 percent of patients require dialysis or have kidney damage of varying severity or die despite plasma exchange/plasma infusion treatment. Case presentation We describe the case of a 23-year-old woman of Hellenic origin who, after a preeclampsia-induced premature delivery, developed thrombotic microangiopathy with renal failure, tonicoclonic seizures, anasarca edema and hypertension. Intensive plasma exchange was initiated twice daily, in parallel to dialysis for one month. Three months later, our patient was discharged with nondialysis-dependent renal failure and without signs of hemolysis. Three months after discharge our patient was readmitted with cardiomyopathy (left ventricular ejection fraction of 25 percent) and signs and symptoms of thrombotic microangiopathy. Our patient was diagnosed with atypical hemolytic uremic syndrome and was started on eculizumab (a complement inhibitor), which improved clinical and laboratory parameters. However, a transient pause in treatment resulted in thrombotic microangiopathy relapse, which was rapidly blocked with reintroduction of eculizumab treatment. During long-term eculizumab treatment, thrombotic microangiopathy manifestations were inhibited and renal and cardiac function restored, with no need for other invasive treatments. Conclusions Establishing the diagnosis of atypical hemolytic uremic syndrome in patients presenting with thrombotic microangiopathy is challenging since common symptoms are shared with other conditions like Shiga toxin-producing Escherichia coli hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. The described case illustrates the complexity and importance of rapid diagnosis in a rare disease and the need for appropriate and specific treatment for best long-term outcomes. PMID:25219386

  5. Bacteremia caused by hemolytic, high-level gentamicin-resistant Enterococcus faecalis.

    PubMed Central

    Huycke, M M; Spiegel, C A; Gilmore, M S

    1991-01-01

    Between 1 January 1984 and 31 December 1987, 206 enterococcal blood isolates at the University of Wisconsin Hospital and Clinics were analyzed for high-level aminoglycoside resistance (hereafter high-level aminoglycoside resistance is simply referred to as "resistance") and hemolysin production. Of 190 Enterococcus faecalis isolates, 68 (35.8%) were resistant to gentamicin. Of these 68 strains, 67 (98.5%) contained a gene coding for the bifunctional aminoglycoside-modifying 6'-aminoglycoside acetyltransferase-2"-aminoglycoside phosphotransferase [AAC(6')-APH(2")] enzyme. Of 190 isolates, 85 (44.7%) were hemolytic and contained a gene coding for component A of the enterococcal hemolysin. Sixty-two of 68 (91.2%) gentamicin-resistant isolates but only 23 of 122 (18.8%) gentamicin-susceptible isolates were hemolytic (P less than 0.001). Twelve of the hemolytic, gentamicin-resistant E. faecalis blood isolates, but only 2 of 9 nonhemolytic or gentamicin-susceptible isolates, had identical chromosomal DNA restriction endonuclease digestion patterns, suggesting a common derivation for these strains. A historical cohort study from 1 July 1985 to 31 March 1987 identified by regression analysis postsurgical intensive care unit status (odds ratio [OR], 5.0; 95% confidence interval [CI], 1.1 to 22.8) and prior treatment with an expanded- or broad-spectrum cephalosporin (OR, 3.0; 95% CI, 0.9 to 10.1) as risk factors for gentamicin-resistant E. faecalis bacteremia. Patients with hemolytic, gentamicin-resistant E. faecalis bacteremia had a fivefold-increased risk for death within 3 weeks of their bacteremia compared with patients with nonhemolytic, gentamicin-susceptible strains (95% CI, 1.0 to 25.4). Images PMID:1929336

  6. Shiga Toxin–Producing Escherichia coli Infections Associated with Hemolytic Uremic Syndrome, Italy, 1988–2000

    PubMed Central

    Caprioli, Alfredo; Minelli, Fabio; Gianviti, Alessandra; De Petris, Laura; Edefonti, Alberto; Montini, Giovanni; Ferretti, Alfonso; De Palo, Tommaso; Gaido, Maurizio; Rizzoni, Gianfranco

    2003-01-01

    The mean annual incidence of hemolytic uremic syndrome in persons <15 years of age in Italy from 1988 to 2000 was 0.28 per 100,000 population. Laboratory investigations showed that Shiga toxin–producing Escherichia coli (STEC) infection occurred in 73.1% of patients. STEC O157 was the most common serotype, but a considerable number of cases were from infections by non-O157 STEC. PMID:12533290

  7. Cholelithiasis following Escherichia coli O157?:?H7-associated hemolytic uremic syndrome

    Microsoft Academic Search

    John R. Brandt; Mark W. Joseph; Laurie S. Fouser; Phillip I. Tarr; Israel Zelikovic; Ruth A. McDonald; Ellis D. Avner; Nancy G. McAfee; Sandra L. Watkins

    1998-01-01

    .   Sequelae of Escherichia coli O157?:?H7-associated hemolytic uremic syndrome (HUS) 2?–?3 years following an outbreak in Washington State have been prospectively\\u000a studied to identify predictors of adverse sequelae. Logistic regression analysis was used to examine associations between\\u000a findings in the acute course and long-term renal and gastrointestinal outcomes. Twenty-one percent of patients had gastrointestinal\\u000a sequelae, which included cholelithiasis resulting in

  8. Anti-Factor H Autoantibodies Associated with Atypical Hemolytic Uremic Syndrome

    Microsoft Academic Search

    Marie-Agnes Dragon-Durey; Chantal Loirat; Sylvie Cloarec; Marie-Alice Macher; Jacques Blouin; Hubert Nivet; Laurence Weiss; Wolf Herman Fridman; Veronique Fremeaux-Bacchi

    2005-01-01

    Several studies have demonstrated genetic predisposition in non-shigatoxin-associated hemolytic uremic syndrome (HUS), involving regulatory proteins of the complement alternative pathway: Factor H (FH) and membrane co-factor protein (CD46). Regarding the observations of thrombotic thrombocytopenic purpura patients, in whom a von Willebrand factor protease (ADAMST-13) deficiency may be inherited or acquired secondary to IgG antibodies, it was speculated that HUS might

  9. Model system to study the influence of aggregation on the hemolytic potential of silica nanoparticles.

    PubMed

    Thomassen, Leen C J; Rabolli, Virginie; Masschaele, Kasper; Alberto, Gabriele; Tomatis, Maura; Ghiazza, Mara; Turci, Francesco; Breynaert, Eric; Martra, Gianmario; Kirschhock, Christine E A; Martens, Johan A; Lison, Dominique; Fubini, Bice

    2011-11-21

    A well-defined silica nanoparticle model system was developed to study the effect of the size and structure of aggregates on their membranolytic activity. The aggregates were stable and characterized using transmission electron microscopy, dynamic light scattering, nitrogen adsorption, small-angle X-ray scattering, infrared spectroscopy, and electron paramagnetic resonance. Human red blood cells were used for assessing the membranolytic activity of aggregates. We found a decreasing hemolytic activity for increasing hydrodynamic diameter of the nanoparticle aggregates, in contrast to trends observed for isolated particles. We propose here a qualitative model that considers the fractal structure of the aggregates and its influence on membrane deformation to explain these observations. The open structure of the aggregates means that only a limited number of primary particles, from which the aggregates are built up, are in contact with the cell membrane. The adhesion energy is thus expected to decrease resulting in an overall lowered driving force for membrane deformation. Hence, the hemolytic activity of aggregates, following an excessive deformation of the cell membrane, decreases as the aggregate size increases. Our results indicate that the aggregate size and structure determine the hemolytic activity of silica nanoparticle aggregates. PMID:21928780

  10. Hemolytic activity and platelet aggregation inhibitory effect of vipoxin's basic sPLA2 subunit

    PubMed Central

    Goranova, Yana; Pantcheva, Ivayla; Atanasov, Vasil; Danchev, Dobri; Petrova, Svetla

    2013-01-01

    In the present study we evaluated the effect of secreted phospholipase A2 (sPLA2) (the toxic subunit of the heterodimeric neurotoxin vipoxin, isolated from the Bulgarian long-nosed viper Vipera ammodytes meridionalis) on hemolysis, erythrocyte morphology and platelet aggregation. Hemolytic activity of sPLA2 was examined in the presence of saturated (palmitic) and unsaturated (oleic) fatty acids and it was found that oleic acid increased the hemolytic activity of sPLA2 in a concentration-dependent manner, compared to the effect of palmitic acid and controls. The addition of heparin to red blood cells (RBC) suspension containing sPLA2 or mixture of sPLA2 and the corresponding fatty acid led to an inhibition of hemolytic activity. The effect of sPLA2 on RBC morphology resulted in formation of echinocytes (spherocyte subtype), suggesting that RBC could be the possible targets attacked by sPLA2. Vipoxin sPLA2 inhibited (in a dose-dependent manner) platelet aggregation when arachidonic acid and collagen were used as inducers, while in the case of ADP its inhibitory effect was inappreciable. PMID:24678250

  11. A 2-year-old boy with hemolytic uremic syndrome and pneumocephalus.

    PubMed

    Martin, Sarah E; Allen, Steven D; Faught, Phillip; Hawley, Dean A; Bonnin, Jose M; Hattab, Eyas M

    2012-01-01

    Clostridium septicum infection following hemolytic uremic syndrome is rare and carries a poor prognosis, especially when the brain is involved. We report a case of a previously healthy 2-year-old boy who presented with two days of anuria and bloody diarrhea. He was admitted to the local children's hospital with a diagnosis of hemolytic uremic syndrome, presumably secondary to E. coli O157. He soon required intubation and was noted to have fixed and dilated pupils. Head CT revealed left frontal subcortical white matter vasogenic edema and scattered pockets of pneumocephalus. The patient expired 14 hours after admission. Antemortem blood cultures grew C. septicum. Gross pathologic examination of the brain revealed a large intraparenchymal cerebral hemorrhage in the left frontal and parietal lobes. There was extensive cystic changes as well. Microscopic examination revealed vacuolization and diffuse colonization with rod-shaped bacteria, but without the expected tissue response. There have been only six previously reported cases of C. septicum infection following hemolytic uremic syndrome, four of which had brain involvement. Mortality rate is high, with the only known survivor among those with brain involvement having a brain abscess rather than diffuse pneumocephalus. PMID:22150928

  12. Atypical Hemolytic-Uremic Syndrome: A Case Report and Literature Review

    PubMed Central

    Rafiq, Arsalan; Tariq, Hassan; Abbas, Naeem; Shenoy, Roopalekha

    2015-01-01

    Patient: Female, 59 Final Diagnosis: Atyipcal hemolytic uremic syndrome Symptoms: Delirium • headache Medication: — Clinical Procedure: — Specialty: Hematology Objective: Rare disease Background: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal dysfunction. It is a disease related to genetic mutations in the alternative complement pathway and has a distinct pathophysiology but is difficult to differentiate from other thrombotic microangiopathies. Case Report: We present a case of a 59-year-old female patient who presented with accelerated hypertension, acute renal failure, hemolysis, and encephalopathy. She was managed with antihypertensive medication, but her encephalopathy did not improve. Evaluation resulted in our impression of the disease being atypical hemolytic-uremic syndrome. The patient continued to be managed with good blood pressure control and later was started on eculizumab, but evaluation of response to therapy was hindered by the patient’s non-compliance with therapy and follow-up appointments. Conclusions: We have a very limited understanding of the genetics and epidemiology of atypical HUS, and the overlapping clinical features sometimes delay diagnosis and initiation of appropriate treatment of this rare disease. PMID:25708146

  13. Streptolysin S of Streptococcus anginosus exhibits broad-range hemolytic activity.

    PubMed

    Asam, Daniela; Mauerer, Stefanie; Spellerberg, Barbara

    2015-04-01

    Streptococcus anginosus is a commensal of mucous membranes and an emerging human pathogen. Some strains, including the type strain, display a prominent ?-hemolytic phenotype. A gene cluster (sag), encoding a variant of streptolysin S (SLS) has recently been identified as the genetic background for ?-hemolysin production in S. anginosus. In this study, we further characterized the hemolytic and cytolytic activity of the S. anginosus hemolysin in comparison with other streptococcal hemolysins. The results indicate that SLS of S. anginosus is a broad-range hemolysin able to lyse erythrocytes of different species, including horse, bovine, rabbit and even chicken. The hemolytic activity is temperature dependent, and a down-regulation of the hemolysin expression is induced in the presence of high glucose levels. Survival assays indicate that in contrast to other streptococcal species, S. anginosus does not require SLS for survival in the presence of human granulocytes. Cross-complementation studies using the sagB and sagD genes of Streptococcus pyogenes and Streptococcus dysgalactiae subsp. equisimilis demonstrated functional similarities to the S. anginosus SLS. Nevertheless, distinct differences to other streptolysin S variants were noted and provide further insights into the molecular mechanisms of SLS pathogen host interactions. PMID:25381594

  14. A high prevalence of biochemical evidence of vitamin B12 or folate deficiency does not translate into a comparable prevalence of anemia.

    PubMed

    Metz, Jack

    2008-06-01

    Based on biochemical evidence, a high prevalence of biochemical evidence of vitamin B12 or folate deficiency has been reported in a number of areas in the world. The evidence that these biochemical abnormalities lead to a comparable prevalence of anemia is reviewed. The overall contribution of vitamin B12 deficiency to the global burden of anemia is probably not significant, except perhaps in women and their infants and children in vegetarian communities. In developed countries, folate-deficiency anemia is uncommon. In some developing countries, this anemia is still seen, but there are no comprehensive data on the relative prevalence compared with anemia due to malaria, iron-deficiency, hemoglobinopathy, and HIV disease. It seems unlikely that folate deficiency makes a major contribution to the burden of anemia in developing countries. Iron-deficiency anemia may coexist with vitamin B12 and especially folate deficiency, and may confound the hematological features of the vitamin deficiencies whose prevalence would then be underestimated. Supplementation of the diet of pregnant women with folic acid can virtually eliminate folate-deficiency anemia in these women. There are very few data on the hematological effect of vitamin B12 supplementation or fortification at the population level. The addition of vitamin B12 to the supplementation of the diet of pregnant women with iron and folic acid does not produce an increased hematological response, at least in nonvegetarian populations. There are numerous reports of the effect of folic acid fortification of food on tests of folate status, but only a single published report on the hematological response was found. PMID:18709883

  15. Prevalence of anemia in patients with type II diabetes and mild to moderate chronic kidney disease and the impact of anti-RAS medications.

    PubMed

    Dousdampanis, Periklis; Trigka, Konstantina; Fourtounas, Costas

    2014-05-01

    Anemia is a common feature of diabetes and chronic kidney disease (CKD) mainly due to erythropoietin (EPO) deficiency and uremic toxicity. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) have been established as first-choice medications for the treatment of diabetic nephropathy. However, there are conflicting data regarding their impact on hemoglobin levels in patients with diabetic nephropathy. We evaluated the prevalence of anemia in 101 patients with diabetes mellitus type II and CKD at stage III-IV (group A) compared with 101 non-diabetic patients with similar renal function (group B). Moreover, we evaluated the impact of ACE inhibitors and ARBs on patients' anemia. Anemia was observed in 60 patients in group A and in 47 patients in group B (P < 0.01). Thirty-one (31) patients in group A and 19 patients in group B were receiving exogenous EPO for correction of renal anemia (P <0.05). Mean values of hemoglobin did not show significant differences (12.5 ± 1.8 vs 12.6 ± 1.7 g/dL) between the two groups. Seventy-five patients in group A and 52 patients in group B were receiving ACE inhibitors and/or ARBs (P <0.01), but, after multivariate analysis, we could not detect any association between anemia and the prescription of these medications. Anemia is more common in diabetic patients with CKD stage III-IV than in non-diabetic patients with similar renal function. Our results indicate that ACE inhibitors and ARBs are not a significant cause of anemia for both populations. PMID:24821151

  16. ADHERENCE TO HYDROXYUREA IN CHILDREN WITH SICKLE CELL ANEMIA

    PubMed Central

    Thornburg, Courtney D.; Calatroni, Agustin; Telen, Marilyn; Kemper, Alex R.

    2014-01-01

    Objective(s) To assess adherence to hydroxyurea in children with sickle cell anemia (SCA), to evaluate the association between adherence and hematologic profile, and to identify barriers and facilitators of adherence. Study design Seventy-five children with SCA on hydroxyurea were recruited for a single-institution cross-sectional study. The primary outcome was association between treatment adherence and percent fetal hemoglobin (HbF). Results Good adherence was estimated at 82% by visual analog scale; 84% by Morisky score; 85% by medical provider report; 77% by clinic visits; and 49% based on pharmacy refills. Increase in HbF was moderately associated with good adherence as measured by the parent/proxy Morisky score (r= ?0.39; 95% CI= ?0.58, ?0.17; p<0.01) and prescription refills (r =0.39; 95% CI= 0.16, 0.57; p<0.01). The number of pharmacy refills and the Morisky score explained 23% of the variation in HbF response. Conclusions Adherence was ?75% by 4 out of 5 measures. Pharmacy refills and Modified Morisky Scale may be used to identify: children at high risk for poor response due to non-adherence and children with good adherence with poor response due to individual pharmacodynamics. Future research should prospectively compare adherence measures and evaluate methods to improve treatment adherence. PMID:19880135

  17. Simplification of an Erythropoiesis Model for Design of Anemia Management Protocols in End Stage Renal Disease

    E-print Network

    Massachusetts at Amherst, University of

    Simplification of an Erythropoiesis Model for Design of Anemia Management Protocols in End Stage be used for anemia management protocol (AMP) design based on formal feedback control methods. · In end of anemia; EPO resistance is often observed. A shortened RBC life-span further contributes to the anemia

  18. The Role of Anemia in Congestive Heart Failure and Chronic Kidney Insufficiency

    Microsoft Academic Search

    Donald S. Silverberg; Dov Wexler; Adrian Iaina

    2004-01-01

    Anemia is a major problem in patients with chronic kidney insuffi- ciency.The development of recombinant human erythropoietin has enabled physicians to correct this anemia. Although anemia has not been considered to be a common or important contributor to congestive heart failure, anemia of any cause can lead to car- diac damage and eventually congestive heart failure. Our joint renal-cardiac heart

  19. Congenital dyserythropoietic anemia, type II with SEC23B exon 12 c.1385 A ? G mutation, and pseudo-Gaucher cells in two siblings.

    PubMed

    Sharma, Prashant; Das, Reena; Bansal, Deepak; Trehan, Amita

    2015-03-01

    Objective and importance Congenital dyserythropoietic anemia (CDA) represents a genotypically and phenotypically heterogeneous group of disorders. CDA type II, the most frequent variant, was recently shown to be caused by mutations in the gene encoding the secretory COPII component SEC23B. We report two siblings hailing from Punjab in northern India with classical CDA type II where this mutation was demonstrated. Clinical presentation A 7-year-old girl presented with transfusion-dependent anemia, splenomegaly, and progressive growth failure since 1 year of age. Her 5-year-old brother was similarly afflicted, but there was no other family history. Extensive prior work-up for hemolytic anemia, storage and metabolic disorders, and infectious diseases was negative. Hemoglobin was 71 g/l with normal leukocyte, platelet, and corrected reticulocyte counts. Bone marrow examination revealed marked normoblastic erythroid hyperplasia with dyserythropoiesis (36%) and the presence of bi- and multinucleated erythroblasts with equal-sized nuclei. Many pseudo-Gaucher cells were also seen. Iron stores were increased although ring sideroblasts were absent. Hereditary erythrocyte multinuclearity with positive acidified serum (HEMPAS) test revealed lysis of the red cells in four out of five control sera. Technique Genomic DNA sequencing of the SEC23B exon 12 revealed homozygosity for c.1385 A ? G; Y462C mutations in both siblings. Conclusion CDA has traditionally been a difficult diagnosis to establish, since it requires exclusion of other causes of dyserythropoiesis and the performance of complex tests including HEMPAS and electron microscopy for confirmation. The availability of molecular genetic testing for SEC23B promises to streamline and hasten the diagnostic process for this rare and intriguing disease. PMID:24801240

  20. Immunoasssay chromatographic antigen test for rapid diagnosis of Group A beta hemolytic Streptococcus pharyngitis in children: A cross/ sectional study

    PubMed Central

    Noorbakhsh, S; Tabatabaei, A; Farhadi, M; Ebrahimi, Taj F

    2011-01-01

    Background and Objective Group A beta-hemolytic streptococcus (GABHS) is an important pharyngotonsillitis etiologic agent in children. The objective of this study was diagnosis of streptococcal pharyngitis based on rapid antigen detection test and conventional pharyngeal culture. Materials and Methods The rapid GABHS antigen detection test was compared to culture on blood agar, the gold standard for the diagnosis of this etiologic agent. Results Streptococcal antigen was detected in pharyngeal specimens of 34.5% of cases by rapid strip test. We detected group A Streptococcus in 17.2% of pharyngeal culture. There was no agreement between two methods ( PV < 0.1). The negative pharyngeal culture results are probably due to antibiotic usage in 43.2% of patients. Positive rapid test results in pharyngeal swab was age dependent ( P < 0.05). There was good correlation between observing the “petechia in pharynx of patients” and positive rapid test in pharyngeal swab (P < 0.004). Throat culture results were relatated to previous antibiotic usage ( P < 0.03). Conclusion The rapid test in pharyngeal swab is helpful for rapid diagnosis and treatment of GABHS pharyngitis. Diagnosis of GABHS pharyngitis based on soley clinical findings is misleading in the majority of cases. Petechia observed in pharynx of the cases was highly predictive of streptococcal pharyngitis. PMID:22347590