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1

Hemolytic anemias due to hemoglobinopathies.  

PubMed

Hemoglobinopathies responsible for hemolytic anemias may be divided into two groups. The first one corresponds to thalassemias and the second to the presence of a structurally abnormal hemoglobin (Hb). In thalassemia, the primary biochemical abnormality is a quantitative defect in the biosynthesis of one type of Hb chain. This defect leads to an overall deficit of Hb accumulation in the erythrocyte (hypochromia) together with the presence of an excess of the normally synthesized chains. The unpaired subunits which are less soluble than HbA precipitate, bind to the membrane and ultimately lead to hemolysis. In the second group, the hemolytic anemia is a direct consequence of the physicochemical properties of the structurally abnormal Hb. This molecule may polymerize, precipitate or crystallize within the red blood cell (RBC) leading to membrane alterations and to the destruction of the cell. This chapter will emphasize several examples of structurally abnormal Hbs, such as sickle cell disease and congenital Heinz body hemolytic anemia (CHBHA). PMID:8813715

Poyart, C; Wajcman, H

1996-04-01

2

Hemolytic Anemia  

MedlinePLUS

... from the NHLBI on Twitter. What Is Hemolytic Anemia? Hemolytic anemia (HEE-moh-lit-ick uh-NEE-me-uh) ... blood cells to replace them. However, in hemolytic anemia, the bone marrow can't make red blood ...

3

Hemolytic anemia  

MedlinePLUS

Jager U, Lechner K. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al., ... Price EA, Schrier SS. Extrinsic nonimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al., ...

4

Hemolytic anemia due to warm autoantibodies.  

PubMed

The diagnosis of autoimmune hemolytic anemia (AHA) requires evidence of shortened red blood cell (RBC) survival mediated by autoantibodies directed against autologous RBCs. About 80 percent of patients with AHA have warm-reactive antibodies of the IgG isotype; the remainder exhibit cold-reactive autoantibodies. Typical patients exhibit anemia, reticulocytosis, spherocytes and polychromasia on the blood film and a positive direct antiglobulin test (DAT). Increased indirect serum bilirubin, urinary urobilinogen and serum lactate dehydrogenase (LDH), and decreased serum haptoglobin are not required for the diagnosis, but are frequently present. Patients with AHA and no underlying associated disease are said to have primary or idiopathic AHA. AHA in patients with associated autoimmune disease and certain malignant or infectious diseases is classified as secondary. The etiology of AHA is unknown. Patients with symptomatic anemia require transfusion of RBCs. Prednisone and splenectomy may provide long term remission. Rituximab, intravenous immunoglobulin, immunosuppressive drugs and danazol have been effective in refractory cases and for patients who are poor candidates for surgery. PMID:17904259

Packman, Charles H

2008-01-01

5

Coomb抯 Positive Hemolytic Anemia Due To Insect Bite  

PubMed Central

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

2007-01-01

6

HEMOLYTIC ANEMIA Erythrocytes premature  

E-print Network

9/16/2013 1 HEMOLYTIC ANEMIA Erythrocytes premature destruction SCHISTOCYTES & SPHEROCYTES 路 Gallstones 路 Dark or Red Urine 路 Symptoms of Anemia 路 Thinning of Cortical Bone 路 Extramedullary RBC Defects 路 Immunohemolytic Anemias #12;9/16/2013 3 INTRINSIC DEFECTS 路 Membrane Defects

7

[Autoimmune hemolytic anemia].  

PubMed

Diagnosis of autoimmune hemolytic anemia (AIHA) requires both serologic evidence of an autoantibody and hemolysis. Based on the characteristic temperature reactivity of the autoantibody to red cell membranes, AIHA is classified into warm AIHA or cold AIHA (cold agglutinin disease and paroxysmal cold hemoglobinuria). Sensitized RBCs are destructed by intravascular and/or extravascular hemolysis. On the basis of etiology, AIHA are classified as idiopathic or secondary. The common cause of secondary AIHA is lymphoproliferative disorders, autoimmune diseases, and infections. The first line therapy of patients with warm AIHA is glucocorticoids and primary treatment for cold AIHA is avoiding cold exposure. The other standard treatments include splenectomy and immunosuppressive drugs. Recently, rituximab, a monoclonal anti-CD20 antibody, has been used in refractory AIHA with excellent responses. PMID:18326320

Karasawa, Masamitsu

2008-03-01

8

Hemolytic anemia due to pyruvate kinase deficiency: characterization of the enzymatic activity from eight patients.  

PubMed Central

We have studied the red cell pyruvate kinase (PK) variants from eight patients representing five families with pyruvate kinase deficiency-associated hemolytic anemia. The kinetic properties, electrophoretic mobilities, and immunological reactivity with anti-normal red cell pyruvate kinase were determined. The patients differ in the severity of their clinical condition and in the molecular properties of their red cell pyruvate kinase variants. The most seriously affected patient (PK Beaverton) has no electrophoretically demonstrable red cell isozymes. The activity present is due to the M2 isozyme, however red cell isozyme can be detected immunologically. PK Molalla and PK Lake Oswego are thermolabile variants with normal kinetic parameters. PK Molalla, in addition, has altered electrophoretic mobility. PK Multnomah and PK Milwaukie have decreased affinity for the substrate phosphoenolpyruvate, and PK Multnomah also has altered electrophoretic mobility. PK Coos Bay shows electrophoretic variation and a slightly decreased affinity for phosphoenolpyruvate consistent with an increased modulating effect of fructose-1,6-diphosphate. Images Fig. 1 Fig. 2 PMID:463878

Black, J A; Rittenberg, M B; Bigley, R H; Koler, R D

1979-01-01

9

How Is Hemolytic Anemia Treated?  

MedlinePLUS

... of red blood cell destruction. Blood and Marrow Stem Cell Transplant In some types of hemolytic anemia, such ... their normal lifespan is over. Blood and marrow stem cell transplants may be used to treat these types ...

10

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

PubMed

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癈 (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

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

2015-01-01

11

Drug-induced immune hemolytic anemia  

MedlinePLUS

... Jager U, Lechner K. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, ... EA, Schrier SL, Extrinsic nonimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, ...

12

Neonatal hemolytic anemia due to inherited harderoporphyria: clinical characteristics and molecular basis.  

PubMed

Porphyrias, a group of inborn errors of heme synthesis, are classified as hepatic or erythropoietic according to clinical data and the main site of expression of the specific enzymatic defect. Hereditary coproporphyria (HC) is an acute hepatic porphyria with autosomal dominant inheritance caused by deficient activity of coproporphyrinogen III oxidase (COX). Typical clinical manifestations of the disease are acute attacks of neurological dysfunction; skin photosensitivity may also be present. We report a variant form of HC characterized by a unifying syndrome in which hematologic disorders predominate: harderoporphyria. Harderoporphyric patients exhibit jaundice, severe chronic hemolytic anemia of early onset associated with hepatosplenomegaly, and skin photosensitivity. Neither abdominal pain nor neuropsychiatric symptoms are observed. COX activity is markedly decreased. In a first harderoporphyric family, with three affected siblings, a homozygous K404E mutation has been previously characterized. In the present study, molecular investigations in a second family with neonatal hemolytic anemia and harderoporphyria revealed two heterozygous point mutations in the COX gene. One allele bore the missense mutation K404E previously described. The second allele bore an A-->G transition at the third position of the donor splice site in intron 6. This new COX gene mutation resulted in exon 6 skipping and the absence of functional protein production. In contrast with other COX gene defects that produce the classical hepatic porphyria presentation, our data suggest that the K404E substitution (either in the homozygous or compound heterozygous state associated with a mutation leading to the absence of functional mRNA or protein) is responsible for the specific hematologic clinical manifestations of harderoporphyria. PMID:9454777

Lamoril, J; Puy, H; Gouya, L; Rosipal, R; Da Silva, V; Grandchamp, B; Foint, T; Bader-Meunier, B; Dommergues, J P; Deybach, J C; Nordmann, Y

1998-02-15

13

Treatment of autoimmune hemolytic anemias  

PubMed Central

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 7085% of patients and should be slowly tapered over a time period of 612 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. 8090% 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

Zanella, Alberto; Barcellini, Wilma

2014-01-01

14

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

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 慚osul (homozygote), PK 態ukarest1,2, PK 慔amburg1, PK 慘鰈n1, and PK 慐ssen (compound heterozygote). PK 慚osul showed normal positive cooperative substrate

M. Lakomek; P. Huppke; B. Neubauer; A. Pekrun; H. Winkler; W. Schr鰐er

1994-01-01

15

Human aldolase A deficiency associated with a hemolytic anemia: thermolabile aldolase due to a single base mutation.  

PubMed Central

Fructose-1,6-bisphosphate aldolase A (fructose-bisphosphate aldolase; EC 4.1.2.13) deficiency is an autosomal recessive disorder associated with hereditary hemolytic anemia. To clarify the molecular mechanism of the deficiency at the nucleotide level, we have cloned aldolase A cDNA from a patient's poly(A)+ RNA that was expressed in cultured lymphoblastoid cells. Nucleotide analysis of the patient's aldolase A cDNA showed a substitution of a single nucleotide (adenine to guanine) at position 386 in a coding region. As a result, the 128th amino acid, aspartic acid, was replaced with glycine (GAT to GGT). Furthermore, change of the second letter of the aspartic acid codon extinguished a F ok I restriction site (GGATG to GGGTG). Southern blot analysis of the genomic DNA showed the patient carried a homozygous mutation inherited from his parents. When compared with normal human aldolase A, the patient's enzyme from erythrocytes and from cultured lymphoblastoid cells was found to be highly thermolabile, suggesting that this mutation causes a functional defect of the enzyme. To further examine this possibility, the thermal stability of aldolase A of the patient and of a normal control, expressed in Escherichia coli using expression plasmids, was determined. The results of E. coli expression of the mutated aldolase A enzyme confirmed the thermolabile nature of the abnormal enzyme. The Asp-128 is conserved in aldolase A, B, and C of eukaryotes, including an insect, Drosophila, suggesting that the Asp-128 of the aldolase A protein is likely to be an amino acid residue with a crucial role in maintaining the correct spatial structure or in performing the catalytic function of the enzyme. Images PMID:2825199

Kishi, H; Mukai, T; Hirono, A; Fujii, H; Miwa, S; Hori, K

1987-01-01

16

Immunotherapy Treatments of Warm Autoimmune Hemolytic Anemia  

PubMed Central

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梥ometimes 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

Gu, Wangang

2013-01-01

17

Autoimmune hemolytic anemia caused by cold agglutinins in a young pregnant woman.  

PubMed

Autoimmune hemolytic anemia is a rare disorder. A 34-year old woman presented with thrombophlebitis after her first delivery, during puerperium. A high titer of cold agglutinins was found. Lymphomas, systemic lupus erythematosus, and tumors were excluded. She conceived again. Due to the anemia she had frequent blood transfusions and she delivered at 38 weeks of gestation. PMID:16854701

Batalias, Labros; Trakakis, Eftihios; Loghis, Costas; Salabasis, Costas; Simeonidis, George; Karanikolopoulos, Panagiotis; Kassanos, Demetrios; Salamalekis, Emmanuel

2006-04-01

18

Idiopathic Heinz body hemolytic anemia in newborn infants.  

PubMed

Heinz body hemolytic anemia developed in six full-term infants while at home during the first 2 weeks of life. The disorder first manifested as hyperbilirubinemia. However, in all cases, severe anemia (hemoglobin concentration 49-73 g/L) developed during the 4-12 days of hospitalization. The infants had not been exposed to known oxidants, and their erythrocytes were not glucose-6-phosphate dehydrogenase (G6PD) deficient and contained no unstable hemoglobin. It is hypothesized that in these newborn infants, Heinz body hemolytic anemia developed as a result of ingestion of an oxidant contained in feedings. The nature of this agent is as yet unknown. PMID:2712239

Ballin, A; Brown, E J; Zipursky, A

1989-01-01

19

Current approaches for the treatment of autoimmune hemolytic anemia.  

PubMed

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

Jaime-P閞ez, Jos Carlos; Rodr韌uez-Mart韓ez, Marisol; G髆ez-de-Le髇, Andr閟; Tar韓-Arzaga, Luz; G髆ez-Almaguer, David

2013-10-01

20

Impairment of Bone Health in Pediatric Patients with Hemolytic Anemia  

PubMed Central

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

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

2014-01-01

21

Red blood cell vesiculation in hereditary hemolytic anemia  

PubMed Central

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

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

2013-01-01

22

Hemolytic anemia associated with heterograft replacement of the mitral valve.  

PubMed

The first case of overt hemolytic anemia following mitral valve replacement with a porcine heterograft is reported. Cardiac catheterization failed to reveal a paravalvular leak or valvular incompetence to account for the hemolysis. Red cell traumatization by the Dacron-covered Stellite ring and stent is suggested as the cause of hemolysis with the porcine heterograft. PMID:567264

Myers, T J; Hild, D H; Rinaldi, M J

1978-08-01

23

Heinz-body hemolytic anemia associated with phenazopyridine and sulfonamide.  

PubMed

A 27-year-old white woman developed Heinz-body hemolytic anemia following multiple courses of oral phenazopyridine and trimethoprim-sulfamethoxazole. Her diagnosis was supported by the finding of bite cells on peripheral blood smear. The patient's rapid recovery and reversal of abnormal laboratory parameters were consistent with an acquired hemolytic disorder. This case should sensitize the clinician to the development of drug-induced oxidative hemolysis, its clinical features, and its reversibility. It is also important that the clinician recognize those drugs capable of causing this disorder and appreciate the methods available to establish the diagnosis. PMID:2786291

Ponte, C D; Lewis, M J; Rogers, J S

1989-02-01

24

Hemolytic Anemia and Metabolic Acidosis: Think about Glutathione Synthetase Deficiency.  

PubMed

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

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

2015-02-01

25

Hemolytic anemia in wild seaducks caused by marine oil pollution.  

PubMed

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

Yamato, O; Goto, I; Maede, Y

1996-04-01

26

Pleural solitary fibrous tumor complicated with autoimmune hemolytic anemia.  

PubMed

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

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

27

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

PubMed

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

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

28

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

PubMed Central

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

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

29

An outbreak of Heinz body positive hemolytic anemia in chronic hemodialysis patients.  

PubMed

During the four month period, from December 1988 to March 1989, there was an outbreak of Heinz body positive hemolytic anemia in 34 patients undergoing hemodialysis in a 500-bed hospital, Seoul, Korea. The episodes of hemolysis were not reduced by changing the charcoal column and reverse osmosis system, or by adding ascorbic acid to the dialysate. The concentrations of nitrate, copper, aluminum and zinc in the treated water were all within the standards for hemodialysis. The chloramine concentration of the treated water was over 0.6 mg/L, markedly exceeding the allowable level of 0.1 mg/L. This high level of chloramine was proved to be due to the contamination of the water source by raw sewage. After we changed the source of water supply to another, no more episodes of hemolytic anemia occurred. It is concluded that chloramine is one of the major contaminants causing dialysis-induced hemolytic anemia and regular determinations are necessary, especially during winter and dry seasons. PMID:8031729

Pyo, H J; Kwon, Y J; Wee, K S; Kwon, S Y; Lee, C H; Kim, S; Lee, J S; Cho, S H; Cha, C W

1993-07-01

30

Microangiopathic hemolytic anemia (MAHA) as paraneoplastic syndrome in metastasized signet ring cell carcinomas: case reports and review of the literature.  

PubMed

We report on two spontaneous cases of microangiopathic hemolytic anemia (MAHA) as first manifestation due to metastasized signet ring carcinoma, one of gastric and one of unknown origin. The patients presented with an acute onset of Coombs negative hemolytic anemia and fragmentocytes in the peripheral blood smear which are typical for MAHA. These case reports present MAHA as a rare paraneoplastic syndrome in patients with metastasized signet ring carcinoma. Parallel to symptomatic treatment we started chemotherapy treatment (ELF and PLF regimen, respectively). In both cases we were able to control the MAHA and cancer progression. PMID:16088769

Arkenau, H-T; M黶sig, O; Buhr, T; Jend, H H; Porschen, R

2005-08-01

31

Immune-mediated hemolytic anemia associated with trimethoprim-sulphamethoxazole administration in a horse.  

PubMed Central

A 10-year-old, thoroughbred gelding was administered sulphonamide drugs during surgical treatment of guttural pouch mycosis. The horse became anemic and a diagnosis of immune-mediated hemolytic anemia was made after other causes of anemia had been ruled out. The anemia resolved after the drugs were withdrawn. PMID:9524723

Thomas, H L; Livesey, M A

1998-01-01

32

A Fetal Hemolytic Anemia in a Child with Cytomegalovirus Infection  

PubMed Central

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

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

2014-01-01

33

Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: diagnosis and classification.  

PubMed

Thrombotic microangiopathies (TMAs) include several diseases, most prominently are thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS). TMAs are characterized by profound thrombocytopenia, microangiopathic hemolytic anemia and organ ischemia. In most cases TTP results from deficiency of ADAMTS13, the von Willebrand factor-cleaving protease leading to increase of ultra-large von Willebrand factor (ULVWF) multimers. Congenital TTP is due to mutations in the gene of ADAMTS13 whereas acquired TTP is due to production of autoantibodies against ADAMTS13. In both cases severe deficiency of ADAMTS13 exists. However, the presence of ADAMTS13 activity does not rule out TTP. Diagnostic criteria of TTP are based on clinical features of neurologic and renal disfunction along with anemia and thrombocytopenia, low ADAMTS13 activity, and the presence of ULVWF. The standard treatment of TTP includes plasma exchange, protein A immunoabsobtion, immunosuppressive drugs, CD20 antibodies against B cells, and splenectomy. HUS is commonly caused by infection with Shiga-toxin produced by Escherichia coli. HUS is characterized by thrombocytopenia, anemia, renal impairment and diarrhea. Rarely, atypical HUS appears as a consequence of mutations related to the alternative pathway for the compliment system. Plasmapheresis in HUS is not efficient. Alternatively, plasma therapy and in some cases dialysis are used. TMA diseases may be associated with other infections, bone marrow transplantation, pregnancy, systemic vasculitis, and certain drugs. PMID:24418304

Shenkman, Boris; Einav, Yulia

2014-01-01

34

Incidence of congenital hemolytic anemias in young cholelithiasis patients  

PubMed Central

AIM: To clarify the incidence of congenital hemolytic anemias (CHA) in young cholelithiasis patients and to determine a possible screening test based on the results. METHODS: Young cholelithiasis patients (< 35 years) were invited to our outpatient clinic. Participants were asked for comorbidities and family history. The number of gallstones were recorded. Blood samples were obtained to perform a complete blood count, standard Wright-Giemsa staining, reticulocyte count, hemoglobin (Hb) electrophoresis, serum lactate dehydrogenase and bilirubin levels, and lipid profile. RESULTS: Of 3226 cholecystectomy patients, 199 were under 35 years, and 190 with no diagnosis of CHA were invited to take part in the study. Fifty three patients consented to the study. The median age was 29 years (range, 17-35 years), 5 were male and 48 were female. Twelve patients (22.6%) were diagnosed as thalassemia trait and/or ?ron-deficiency anemia. Hb levels were significantly lower (P = 0.046), and mean corpuscular volume (MCV) and hematocrit levels were slightly lower (P = 0.072 and 0.082, respectively) than normal. There was also a significantly lower number of gallstones with the diagnosis (P = 0.007). CONCLUSION: In endemic regions, for young cholelithiasis patients (age under 35) with 2-5 gallstones, the clinician/surgeon should pay attention to MCV and Hb levels as indicative of CHA. PMID:21086564

Ezer, Ali; Torer, Nurkan; Nursal, Tarik Zafer; Kizilkilic, Ebru; Caliskan, Kenan; Colakoglu, Tamer; Moray, Gokhan

2010-01-01

35

When pure is not so pure: chloramine-related hemolytic anemia in home hemodialysis patients.  

PubMed

Worldwide, chloramines are used as the preferred disinfectant for city water supplies. Although they have distinct advantages compared with chlorine and are deemed harmless to the general population, hemodialysis (HD) patients are at risk from chloramine-induced hemolytic anemia. In recent years, this has been highlighted in regional dialysis units but not as frequently in the home HD group. We report on 2 home HD patients who succumbed to severe oxidative hemolysis due to high mains water chloramine concentrations. Both patients were extensively investigated for other cause of anemia before a definitive diagnosis was reached. Delays in diagnosing this uncommon condition can be costly in terms of significant morbidity and excessive usage of recombinant erythropoietin and blood transfusion. Prevention primarily involves enforcing strict water quality control and establishing regular communication with water supply boards and home HD patients. Double (inline) carbon filters should be installed in patient's homes as an effective means for removing high incoming chloramine concentrations. PMID:20618875

Junglee, Naushad A; Rahman, Saeed U; Wild, Mike; Wilms, Anke; Hirst, Sarah; Jibani, Mahdi; Seale, Jim R C

2010-07-01

36

Specific macrothrombocytopenia/hemolytic anemia associated with sitosterolemia.  

PubMed

Sitosterolemia (phytosterolemia) is a rare inherited sterol storage disorder, characterized by significantly elevated plasma levels of plant sterols. The clinical features of sitosterolemia are xanthomas, premature atherosclerosis, arthritis, and, occasionally, liver function impair and hematologic abnormalities. This disorder is caused by mutations of ABCG5/ABCG8 genes. We report here the clinical, laboratory, and molecular genetic features of 13 patients with sitosterolemia from eight unrelated families who had specific hematologic problems of macrothrombocytopenia, hemolytic anemia, and splenomegaly besides the major clinical manifestations. The peripheral blood films showed some unique features: large platelets surrounded by a circle of vacuoles, and various abnormal erythrocyte shapes, especially stomatocyte. According to these distinct changes of blood cell morphology, we identified two sitosterolemia patients who lacked the classical clinical phenomena. All the patients had been misdiagnosed with immune thrombocytopenia (ITP), Evans syndrome, or secondary ITP with delay being 28.8 years between symptom onset and correct diagnosis. These results indicate that sitosterolemia is certainly not as rare as originally thought. The phenomena of macrothrombocytopenia/hemolysis might represent a new platelet disorder. Plasma plant sterols and ABCG5/ABCG8 genes should be analyzed when such hematologic abnormalities are unexplained. PMID:24166850

Wang, Zhaoyue; Cao, Lijuan; Su, Yanhua; Wang, Gaifeng; Wang, Ruijuan; Yu, Ziqiang; Bai, Xia; Ruan, Changgeng

2014-03-01

37

Rumen bacteria are involved in the onset of onion-induced hemolytic anemia in sheep.  

PubMed

The mechanism of onion-induced hemolytic anemia in ruminants was investigated. The ether-extract obtained from the mixture of rumen fluid and onion juice incubated at 38.5 degrees C for 9 hr induced oxidative damage in sheep erythrocytes in vitro, indicating the production of certain oxidants in the mixture. The increase of the oxidative effect in the mixture was inhibited completely by the removal of rumen microorganisms and partly by treatment with antibiotics and by oxygen gas. The sheep fed onions (50 g/kg body weight/day) for 15 days developed more severe Heinz body hemolytic anemia than did the sheep fed the equivalent amount of onions with 5 g/day ampicillin sodium salt. The results indicated that certain rumen bacteria appear to be involved in the onset of onion-induced hemolytic anemia in sheep. PMID:10342287

Selim, H M; Yamato, O; Tajima, M; Maede, Y

1999-04-01

38

[Primary Sj鰃ren's syndrome with lymphocytic interstitial pneumonia, autoimmune hemolytic anemia and an endobronchial amyloid nodule].  

PubMed

A 73-year-old woman was admitted to our hospital for examination of anemia, dry feeling of oral and eyes with chest abnormal shadow in October, 1999. Chest radiograph showed interstitial shadows difference and CT showed small nodular opacities in both lung fields. Laboratory examination showed direct Coombs test was positive, and the serum levels of indirect bilirubin and haptoglobin were elevated. Anti-SS-A antibody and anti-SS-B antibody were positive with hypergammaglobulinemia. The serum levels of SP-D and KL-6 were elevated. Lip biopsy showed atrophy and lymphocyte infiltration of the salivary glands. According to these findings, she was diagnosed as primary Sj鰃ren's syndrome with autoimmune hemolytic anemia. In addition, bronchoscopic examination showed an endobronchial polyp in the right B8, and the biopsy specimen showed AL type amyloid deposits. In order to obtain pathological diagnosis of lung lesions, we performed lung biopsy by video-assisted thoracoscopic surgery. The biopsy specimen showed severe infiltration of lymphocytes and the plasma cells around peribronchiole with lymphoepithelial lesions, suggesting malignant lymphoma. However, immunohistochemistry did not show monoclonal profile and Southern blot hybridization assay demonstrated no rearrangement of JH gene. Lung lesions were diagnosed as lymphocytic interstitial pneumonia due to Sj鰃ren's syndrome. Steroid therapy was performed and followed by improvement of anemia and pulmonary lesions. Malignant lymphoma has not been involved for eight years after a diagnosis. PMID:19068774

Kitada, Junya; Yamad, Gen; Ochi, Takashi; Tanaka, Shintaro; Itoh, Takayuki; Watanabe, Atsushi; Satoh, Masaaki; Takahashi, Hiroki

2008-11-01

39

Hemolytic anemia and progressive neurologic impairment: think about triosephosphate isomerase deficiency.  

PubMed

We have reported the first Tunisian case of triosephosphate isomerase (TPI) deficiency in a 2-year-old girl. She was the first child of a nonconsanguineous couple. The disease included a neonatal onset of chronic hemolytic anemia, recurrent low-respiratory infections then progressive neurological involvement. The diagnosis was made after her death from the TPI values of her parents who exhibited intermediate enzyme deficiency. Molecular study of TPI genes showed that the father and the mother are heterozygous for Glu105Asp mutation. Pediatricians must be alert to the differential diagnosis in patients having hemolytic anemia and other concomitant manifestations. PMID:24840153

Aissa, Khaoula; Kamoun, Fatma; Sfaihi, Lamia; Ghedira, Elyes Slim; Aloulou, Hajer; Kamoun, Thouraya; Pissard, Serge; Hachicha, Mongia

2014-08-01

40

Post-transfusion hypertension and seizure in congenital hemolytic anemia: a case report and literature review.  

PubMed

A rare syndrome of hypertension, seizures and intracranial bleed has been reported among patients with congenital hemolytic anemia who underwent multiple blood transfusions. We report this syndrome in a 12-year-old Malay girl with hemoglobin E-beta-thalassemia, who underwent intensive transfusion and subsequently had headache, visual loss, severe hypertension and seizures. A comprehensive literature review revealed 30 patients with this syndrome, of whom 15 had intracranial bleed and 12 among these 15 died. A less-intensive transfusion regimen among patients with chronic hemolytic anemia and prompt detection and management of hypertension may prevent this potentially fatal syndrome. PMID:24473404

Ngim, Chin Fang; Ng, Chen Siew; Lai, Nai Ming

2014-06-01

41

Heinz body hemolytic anemia induced by DQ-2511, a new anti-ulcer drug, in dogs.  

PubMed

DQ-2511, a new anti-ulcer drug, was administered to beagle dogs for 4 weeks to investigate the mechanism whereby this drug induced hemolytic anemia and its reversibility in comparison with beta-acetylphenylhydrazine. Hemolytic anemia accompanied by an increase in the number of cells containing Heinz bodies that was preceded by a marked decrease in blood-reduced glutathione concentration was observed in dogs receiving 600 mg/kg of DQ-2511, but only a slight increase in the methemoglobin level was noted. beta-Acetylphenylhydrazine, however, caused hemolytic anemia accompanied by marked increases in both Heinz body-containing cells and methemoglobin concentration, but the blood-reduced glutathione concentration was not decreased consistently with the formation of Heinz bodies. Hemolytic anemia disappeared after a 4-week recovery period in the dogs that received DQ-2511. These results suggest that decreases in reduced glutathione in erythrocytes play an important role in the anemia and Heinz body formation induced by DQ-2511, but not by beta-acetylphenylhydrazine. PMID:8449384

Ohno, H; Tojo, H; Kakihata, K; Nomura, M; Takayama, S

1993-02-01

42

New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children  

PubMed Central

Background Autoimmune hemolytic anemia is a rare condition in children. Little is known about its initial presentation and the subsequent progression of the disease. Design and Methods Since 2004, a national observational study has been aiming to thoroughly describe cases and identify prognostic factors. Patients from all French hematologic pediatric units have been included if they had a hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test and hemolysis. Evans syndrome was defined by the association of autoimmune hemolytic anemia and immunological thrombocytopenic purpura. Data from patients medical records were registered from birth to last follow-up. Autoimmune hemolytic anemia was classified as primary or secondary. Remission criteria, qualifying the status of anemia at last follow-up, were used with the aim of identifying a subgroup with a favorable prognosis in continuous complete remission. Results The first 265 patients had a median age of 3.8 years at diagnosis. In 74% of cases the direct antiglobulin test was IgG/IgG+C3d. Consanguinity was reported in 8% of cases and first degree familial immunological diseases in 15% of cases. Evans syndrome was diagnosed in 37% of cases. Autoimmune hemolytic anemia was post-infectious in 10%, immunological in 53% and primary in 37% of cases. After a median follow-up of 3 years, 4% of children had died, 28% were still treatment-dependent and 39% were in continuous complete remission. In multivariate analysis, IgG and IgG+C3d direct antiglobulin tests were associated with a lower rate of survival with continuous complete remission (adjusted hazard ratio, 0.43; 95% confidence interval, 0.210.86). Conclusions This nationwide French cohort is the largest reported study of childhood autoimmune hemolytic anemia. The rarity of this condition is confirmed. Subgroups with genetic predisposition and underlying immune disorders were identified. PMID:21228033

Aladjidi, Nathalie; Leverger, Guy; Leblanc, Thierry; Picat, Marie Quitterie; Michel, G閞ard; Bertrand, Yves; Bader-Meunier, Brigitte; Robert, Alain; Nelken, Brigitte; Gandemer, Virginie; Savel, H閘鑞e; Stephan, Jean Louis; Fouyssac, Fanny; Jeanpetit, Julien; Thomas, Caroline; Rohrlich, Pierre; Baruchel, Andr; Fischer, Alain; Ch阯e, Genevi鑦e; Perel, Y.

2011-01-01

43

Biomarkers of polycyclic aromatic hydrocarbon (PAH)-associated hemolytic anemia in oiled wildlife.  

PubMed

Polycyclic aromatic hydrocarbons (PAHs) in crude oil cause a range of adverse effects in oiled seabirds, one of the most common being hemolytic anemia via oxidative attack of erythrocytes by PAH metabolites resulting in hemoglobin leakage and formation of Heinz bodies. In such cases, haptoglobin and ferritin are up-regulated to sequester free Hb and iron in the circulation. We investigated these plasma proteins as biomarkers of PAH-induced Heinz body hemolytic anemia in oiled seabirds. Concentration ranges of PAHs, HAP and FT in plasma samples were 10-184 ng/ml, 0-2.6 mg/ml and 0-7.6 ng/ml, respectively. Dose-response relationships between plasma PAH exposure and haptoglobin and ferritin (FT) were investigated, and evidence of erythrocyte Heinz body formation studied in 50 oiled common guillemots stranded on the Norfolk Wash coast (East England). Haptoglobin was negatively correlated, and FT was positively correlated with PAH exposure. Heinz bodies were also observed confirming the toxic mechanism causing hemolytic anemia and counts were positively correlated with exposure. Our results support the application of these complementary biomarkers to assess hemolytic effects of oiling in wildlife biomonitoring, which also discriminate the influence of hemolytic versus inflammatory effects in oiled guillemots. PMID:17674967

Troisi, Gera; Borjesson, Lars; Bexton, Steve; Robinson, Ian

2007-11-01

44

Phosphatidylserine Exposure and Red Cell Viability in Red Cell Aging and in Hemolytic Anemia  

Microsoft Academic Search

Phosphatidylserine (PS) normally localizes to the inner leaflet of cell membranes but becomes exposed in abnormal or apoptotic cells, signaling macrophages to ingest them. Along similar lines, it seemed possible that the removal of red cells from circulation because of normal aging or in hemolytic anemias might be triggered by PS exposure. To investigate the role of PS exposure in

Franz Edward Boas; Linda Forman; Ernest Beutler

1998-01-01

45

HbA1C - overall glycemia marker and hemolytic anemia indicator.  

PubMed

Glycated hemoglobin A1C reflects a mean glycemia over the preceding 3 months (erythrocyte life span). In diabetes management, target value is set below 6.5%, to reduce the risk of chronic complications. However, there are different conditions that lead to a shortened lifespan of erythrocytes, resulting in falsely low HbA1C value. Case presented involves a 72-year-old patient with history of diabetes and possible iatrogenic-induced autoimmune hemolytic anemia. Thus, HbA1C may be a screening test for hemolysis in non-diabetic patients with hemolytic anemia, but in diabetic population with hemolytic disease it is considered to be a very poor marker for both, overall glycemia and haemolysis. PMID:22926387

Jandri? Balen, Marica; Lukenda, Vesna; Jandri?, Ivan; Ragu, Antonija; Zukanovi?, Sidbela; Mi歬i?, Bla瀍nka

2012-08-01

46

Severe hemolytic anemia associated with the homozygous state for an unstable hemoglobin variant (Hb Bushwick).  

PubMed

We have investigated a 13-year-old girl from first cousin parents who presented with severe hemolytic anemia. Hematologic studies showed unstable hemoglobin (Hb) disease (chronic Heinz body anemia), and DNA analysis showed that the patient was homozygous for the previously reported abnormal Hb called Hb Bushwick (beta 74E18 gly-->val). Hb Bushwick is unstable in vitro and in vivo. In addition, using globin chain biosynthetic studies, we show that the beta (Bushwick) chains are unstable. Six members of the patient's family were heterozygous for Hb Bushwick and had a compensated hemolytic disorder. By contrast, the homozygous patient had chronic anemia caused by a combination of hemolysis and ineffective erythropoiesis that was subject to severe exacerbation concomitant with infection. Thus, although unstable Hb disease is correctly regarded as dominant, we clearly see a dosage effect in its expression, whereby the homozygous state is still compatible with life although the red blood cells contain nearly 100% unstable Hb. PMID:7655024

Srivastava, P; Kaeda, J S; Roper, D; Vulliamy, T J; Buckley, M; Luzzatto, L

1995-09-01

47

Autoimmune hemolytic anemia occurred prior to evident nephropathy in a patient with chronic hepatitis C virus infection: case report  

PubMed Central

Background Renal involvement in patients with chronic hepatitis C virus infection has been suggested to be due to a variety of immunological processes. However, the precise mechanism by which the kidneys are damaged in these patients is still unclear. Case presentation A 66 year old man presented with the sudden onset of autoimmune hemolytic anemia. Concomitant with a worsening of hemolysis, his initially mild proteinuria and hemoglobinuria progressed. On admission, laboratory tests revealed that he was positive for hepatitis C virus in his blood, though his liver function tests were all normal. The patient displayed cryoglobulinemia and hypocomplementemia with cold activation, and exhibited a biological false positive of syphilic test. Renal biopsy specimens showed signs of immune complex type nephropathy with hemosiderin deposition in the tubular epithelial cells. Conclusions The renal histological findings in this case are consistent with the deposition of immune complexes and hemolytic products, which might have occurred as a result of the patient's underlying autoimmune imbalance, autoimmune hemolytic anemia, and chronic hepatitis C virus infection. PMID:12946280

Ohsawa, Isao; Uehara, Yuki; Hashimoto, Sumiko; Endo, Morito; Fujita, Takayuki; Ohi, Hiroyuki

2003-01-01

48

The kinetics of hematopoiesis in the light horse III. The hematological response to hemolytic anemia.  

PubMed

The hematological response to acetylphenylhydrazine hemolytic anemia was studied in three standardbred horses. The lifespan of erythrocytes produced during the most severe phase of the anemia were measured with 75-selenomethionine and found to be 144 days as compared to the 139 day lifespan in response to hemorrhagic anemia or 155 days in normal standardbred horses measured previously using the same technique. The erythrocyte counts returned to initial values in 42 days (37, 34 and 54 days) a mean erythrocyte production of 6.4 times 10-12 erythrocytes/day. The mean hemoglobin production was 0.31 gm/kg body weight/day as compared to 0.11 gm Hb/kg/day previously observed in response to hemorrhagic anemia. The mean increase in erythrocyte mean cell volume was 12 mu-3 during the acute response phase to hemolytic anemia in contrast to the absence of a significant increase in the mean cell volume as previously observed during response to hemorrhagic anemia. Free Heinz bodies separated from erythrocytes during the acute phase could not be differentiated from platelets on the hemocytometer counting chamber with standard techniques. PMID:1139414

Lumsden, H J; Valli, V E; McSherry, B J; Robinson, G A; Claxton, M J

1975-07-01

49

The kinetics of hematopoiesis in the light horse III. The hematological response to hemolytic anemia.  

PubMed Central

The hematological response to acetylphenylhydrazine hemolytic anemia was studied in three standardbred horses. The lifespan of erythrocytes produced during the most severe phase of the anemia were measured with 75-selenomethionine and found to be 144 days as compared to the 139 day lifespan in response to hemorrhagic anemia or 155 days in normal standardbred horses measured previously using the same technique. The erythrocyte counts returned to initial values in 42 days (37, 34 and 54 days) a mean erythrocyte production of 6.4 times 10-12 erythrocytes/day. The mean hemoglobin production was 0.31 gm/kg body weight/day as compared to 0.11 gm Hb/kg/day previously observed in response to hemorrhagic anemia. The mean increase in erythrocyte mean cell volume was 12 mu-3 during the acute response phase to hemolytic anemia in contrast to the absence of a significant increase in the mean cell volume as previously observed during response to hemorrhagic anemia. Free Heinz bodies separated from erythrocytes during the acute phase could not be differentiated from platelets on the hemocytometer counting chamber with standard techniques. PMID:1139414

Lumsden, H J; Valli, V E; McSherry, B J; Robinson, G A; Claxton, M J

1975-01-01

50

Peroxiredoxin II is essential for preventing hemolytic anemia from oxidative stress through maintaining hemoglobin stability.  

PubMed

The pathophysiology of oxidative hemolytic anemia is closely associated with hemoglobin (Hb) stability; however, the mechanism of how Hb maintains its stability under oxidative stress conditions of red blood cells (RBCs) carrying high levels of oxygen is unknown. Here, we investigated the potential role of peroxiredoxin II (Prx II) in preventing Hb aggregation induced by reactive oxygen species (ROS) using Prx II knockout mice and RBCs of patients with hemolytic anemia. Upon oxidative stress, ROS and Heinz body formation were significantly increased in Prx II knockout RBCs compared to wild-type (WT), which ultimately accelerated the accumulation of hemosiderin and heme-oxygenase 1 in the Prx II knock-out livers. In addition, ROS-dependent Hb aggregation was significantly increased in Prx II knockout RBCs. Interestingly, Prx II interacted with Hb in mouse RBCs, and their interaction, in particular, was severely impaired in RBCs of patients with thalassemia (THAL) and sickle cell anemia (SCA). Hb was bound to the decameric structure of Prx II, by which Hb was protected from oxidative stress. These findings suggest that Prx II plays an important role in preventing hemolytic anemia from oxidative stress by binding to Hb as a decameric structure to stabilize it. PMID:22960070

Han, Ying-Hao; Kim, Sun-Uk; Kwon, Tae-Ho; Lee, Dong-Seok; Ha, Hye-Lin; Park, Doo-Sang; Woo, Eui-Jeon; Lee, Sang-Hee; Kim, Jin-Man; Chae, Ho-Byoung; Lee, Sang Yeol; Kim, Bo Yeon; Yoon, Do Young; Rhee, Sue Goo; Fibach, Eitan; Yu, Dae-Yeul

2012-09-28

51

Hemolytic anemia and methemoglobinemia in a preterm baby as a complication of antenatal intraamnial injection of toluidine blue.  

PubMed

A late preterm infant was born 4.5 h after intraamniotic injection of 90 mg of Toluidine blue to confirm premature rupture of membranes. Due to the fetal exposition to the dye, the entire body of the patient was blue stained and the baby suffered from methemoglobinemia, Heinz' body positive hemolytic anemia and hyperbilirubinaemia requiring exchange transfusion. These complications underline that antenatal exposition of toluidine blue may result in considerable postnatal infant morbidity. Therefore intraamniotic application of toluidine blue should be discouraged. PMID:23519748

Mehler, K; Oberthuer, A; Weisshaar, G; Valter, M; Vierzig, A; Eifinger, F

2013-09-01

52

Coombs-Negative Autoimmune Hemolytic Anemia in Crohn抯 Disease  

PubMed Central

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抯 disease. The main types of anemia in patients with Crohn抯 disease are iron deficiency anemia and anemia of chronic disease. Although patients with Crohn抯 disease may experience various type of anemia, autoimmune hemolytic anemia (AIHA) in patients with Crohn抯 disease, especially Coombs-negative AIHA, is very rare. Case Report: A 41-year-old woman with Crohn抯 disease presented to our emergency room (ER) with dark urine, dizziness, and shortness of breath. The activity of Crohn抯 disease had been controlled, with Crohn抯 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抯 disease with chronic anemia, diagnosed by red blood cell-bound immunoglobulin G (RBC-IgG) and treated with steroids therapy. PMID:25488633

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

2014-01-01

53

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

PubMed Central

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

Mamoune, Asmaa; Bahuau, Michel; Hamel, Yamina; Serre, Val閞ie; 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

54

Sensitivity to a Metabolite of Diclofenac as a Cause of Acute Immune Hemolytic Anemia  

Microsoft Academic Search

A 75-year-old woman taking the nonsteroidal anti-inflam- RBCs by the patient's serum and was identified as the gluc- matory drug diclofenac (DCF) presented with acute Coombs- uronide ester of 4*-OH DCF by proton nuclear magnetic reso- positive hemolytic anemia and subsequently developed re- nance (NMR) analysis. Studies with a panel of RBCs showed nal failure. A drug-dependent antibody specific for

D. Bougie; S. T. Johnson; L. A. Weitekamp; R. H. Aster

55

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

PubMed

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

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

2014-09-01

56

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

PubMed Central

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-Barr 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

Biliciler, Suur; Wahed, Amer; Sheikh, Kazim

2014-01-01

57

Anemia  

MedlinePLUS

Anemia is a condition in which the body does not have enough healthy red blood cells. Red ... provide oxygen to body tissues. Other types of anemia include: Anemia due to B12 deficiency Anemia due ...

58

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 ...

59

Iron-rich drinking water and ascorbic acid supplementation improved hemolytic anemia in experimental Wistar rats.  

PubMed

Anemia is a frequent problem in both the primary and secondary health care programs. In contrast, most areas of northeast India are vulnerable to iron toxicity. In the present study, we documented the effect of administration of iron rich water on hemolytic anemia in a Wistar rats' animal model. Hemolytic anemia was induced by phenyl hydrazine through intraperitoneal route and diagnosed by the lowering of blood hemoglobin. After inducing the hemolytic anemia, 24 Wistar rats (n?=?6 in four groups) were randomly assigned to 1?mg/l, 5?mg/l, and 10?mg/l ferric oxide iron along with 1?mg/ml ascorbic acid administered through drinking water; a control group was treated with iron-free water. The hematological and biochemical parameters, iron levels in liver, spleen, and kidney were estimated after 30?d of treatment. In the group treated with 5?mg/l iron and ascorbic acid, a significant increase of serum iron and ferritin, and a decrease of TIBC (total iron binding capacity) were observed without changes in other biochemical parameters and histopathological findings. However, in the group treated with 10?mg/l iron and ascorbic acid, hematological changes with significantly higher values for white blood cell count, serum glutamic phospho transaminase, serum glutamic oxaloacetic transaminase, alkaline phosphatase, glucose, splenic, and liver iron content, indicate potential toxicity at this supplementation level. Data suggest that the optimum concentration of iron (5?mg/l) and ascorbic acid solution may improve anemic conditions and may be therapeutically beneficial in the treatment of iron deficiency anemia without any negative impact, while 10?mg/l in drinking water seems to be the threshold for the initiation of toxicity. PMID:24896300

Chaturvedi, Richa; Chattopadhyay, Pronobesh; Banerjee, Saumen; Bhattacharjee, Chira R; Raul, Prasanta; Borah, Kusum; Singh, Lokendra; Veer, Vijay

2014-11-01

60

Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy  

PubMed Central

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

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

2014-01-01

61

Immune-mediated hemolytic anemia and thrombocytopenia in a foal.  

PubMed

A one-month-old Quarter Horse filly had unilateral epistaxis, hyphema, icterus, petechial hemorrhages in the oral, nasal, conjunctival, and vulvar mucous membranes, anemia, thrombocytopenia, negative antinuclear test result, and a positive direct Coombs' test result. Megakaryocytes or cell-associated IgG (fluorescent antibody and immunoperoxidase stains) were not found in bone marrow biopsy specimens. Treatment consisted of glucocorticoids, antibiotics, and a single whole blood transfusion. The foal responded well to treatment, did not develop relapses of the disease, and was clinically normal one year after treatment. PMID:3558071

Sockett, D C; Traub-Dargatz, J; Weiser, M G

1987-02-01

62

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

PubMed Central

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.

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

2014-01-01

63

Heinz-body hemolytic anemia associated with ingestion of methylene blue in a river otter.  

PubMed

Heinz-body hemolytic anemia and nephrosis associated with hemoglobinuria were diagnosed in a North American river otter. Fluids were administered, and the signs of renal failure improved immediately. Severe anemia developed, and the otter received a semisynthetic hemoglobin product to maintain the oxygen-carrying capacity of the blood until a blood transfusion could be given. Immediate clinical improvement was observed following hemoglobin administration, and adverse effects were not seen. Six days after admission, the otter began to produce its own RBC and recovered without complications. The Heinz-body anemia was determined to be caused by methylene blue that was in the water of minnows consumed by the otter the night before it became ill. Methylene blue is a common ingredient in products used to extend the life of bait fish. Bait fish kept in water treated with methylene blue should not be used as food for fish-eating animals. PMID:11829270

Narurkar, Neelesh S; Thomas, Jennifer S; Phalen, David N

2002-02-01

64

Experimental onion-induced hemolytic anemia in dogs.  

PubMed

Within one day following a single oral dose of dehydrated onions, dogs were found to have large numbers of Heinz bodies within erythrocytes. The percentage of erythrocytes that contained Heinz bodies increased slightly to a maximum on day 3 and then declined. The turbidity index increased more gradually with a maximal value on day 4. Erythrocytes with hemoglobin contracted to one side of the cell (eccentrocytes) also appeared after onion feeding. Eccentrocytes are believed to result from a direct injury to the erythrocyte membrane. As with Heinz body-containing cells, the percentages of eccentrocytes present declined as anemia developed. The packed cell volume began to decrease one day after onion administration. A mean decrease of 19 percentage points was reached by day 5. The most anemic dogs had evidence of intravascular hemolysis. Reticulocytosis was first observed five days after onion administration. A slight increase in methemoglobin content was measured four hours after onion administration. No significant changes in erythrocyte reduced glutathione concentration were measured. Transient neutrophilia occurred concomitant with the peak reticulocyte response. PMID:4035943

Harvey, J W; Rackear, D

1985-07-01

65

Penicillin-induced immune hemolytic anemia. Occurrence of massive intravascular hemolysis.  

PubMed

A patient with penicillin-induced immune hemolytic anemia had massive intravascular hemolysis with hemoglobinemia and hemoglobinuria. Substantial amounts of complement components C3 and C4 were detected on the patient's red blood cells (RBCs), in addition to the usual IgG antibody to penicillin. The patient's serum demonstrated a high titer of antibody to penicillin (8,000), which did not cause hemolysis in vitro, but did cause complement fixation when incubated with normal serum. The presence of complement components on the patient's RBCs, and the finding that the serum fixed complement in vitro suggests that penicillin-antipenicillin immune complexes may have been present in the serum. We attribute the severity of the hemolysis to participation of the complement system in the hemolytic process and to the high titer of antibody to pencillin. PMID:1173853

Ries, C A; Rosenbaum, T J; Garratty, G; Petz, L D; Fudenberg, H H

1975-08-01

66

Methylene blue can be used to treat methemoglobinemia in cats without inducing Heinz body hemolytic anemia.  

PubMed

Methylene blue (MB) is the drug of choice in the treatment of methemoglobinemia (MTHB) in humans and most domesticated animals, but is reported contraindicated in cats. Although prolonged treatment of cats for urologic syndromes with MB-containing antiseptics causes Heinz body (HB) hemolytic anemia, there is no evidence to suggest that single or repeated therapeutic doses of MB cause hemolytic anemia. We investigated the efficacy and safety of MB in reversing nitrite-induced MTHB in cats. Forty random-bred adult cats (20 males and 20 females) were divided as follows: Group 1, 1.5 mL saline/kg bw iv (control); Group 2, 1 dose of 1.5 mg MB/kg bw iv; Group 3, 2 doses of 1.5 mg MB/kg bw iv 4 h apart; Group 4 1 dose of 1.5 mg sodium nitrite/kg bw iv; Group 5, 1 dose of 1.5 mg sodium nitrite/kg bw iv followed by 1 dose of 1.5 mg MB/kg bw iv 1 h later; and Group 6, 1.5 mg sodium nitrite/kg bw iv followed in 2 h by 2 doses of 1.5 mg MB/kg iv 4 h apart. One iv dose of MB sufficiently and rapidly reversed MTHB in the cats without increasing circulating HB-containing red blood cells. Giving 2 iv doses of MB without or after sodium nitrite significantly increased the frequency of circulating HB-containing red blood cells. Pre-exposure to sodium nitrite potentiated the HB-inducing effect of 2 doses of MB. Hemolytic anemia was not observed or demonstrated in any of the cats groups. PMID:1509670

Rumbeiha, W K; Oehme, F W

1992-04-01

67

Ceftriaxone-induced Hemolytic Anemia: Case Report and Review of Literature.  

PubMed

Ceftriaxone is a frequently used empiric antibiotic in children. Acute hemolysis is a rare side effect of ceftriaxone therapy associated with a high mortality rate. A 14-year-old boy suffering from Crohn disease developed bacterial pneumonia that was treated with ceftriaxone. We report successful management of ceftriaxone-induced hemolytic anemia (CIHA) in this patient and review the CIHA literature in pediatric patients. Early recognition of CIHA with prompt discontinuation of ceftriaxone therapy may have a beneficial role in reduction of high mortality seen in these patients. PMID:24878619

Northrop, Michael S; Agarwal, Hemant S

2015-01-01

68

Hemolytic anemia after methylene blue therapy for aniline-induced methemoglobinemia.  

PubMed

Methylene blue is utilized as the main treatment of methemoglobinemia conventionally, but it may be ineffective in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency. We report a G6PD-deficient patient who suffered from aniline-induced methemoglobinemia with initial good response Heinz body but hemolytic anemia appeared later 3 d after methylene blue therapy. G6PD deficiency was identified. He recovered uneventfully with hydration, packed blood transfusion and adjuvant luvela-N(dl-alpha-tocopheryl nicotinate) medication. Caution should be taken in using methylene blue as antidote of acute methemoglobinemia, especially when a history of G6PD deficiency is obscure. PMID:11824767

Liao, Yao-Pan; Hung, Dong-Zong; Yang, Dar-Yu

2002-02-01

69

Laparoscopic splenectomy for autoimmune hemolytic anemia in patients with chronic lymphocytic leukemia: a case series and review of the literature.  

PubMed

The purpose of this study was to evaluate the safety and efficacy of laparoscopic splenectomy in patients with chronic lymphocytic leukemia (CLL) complicated by autoimmune hemolytic anemia. A series of nine such patients who underwent this procedure at our institution between August 1997 and September 2001 were retrospectively reviewed. Seven of 9 patients who underwent laparoscopic splenectomy for CLL and autoimmune hemolytic anemia achieved a complete response. One patient who initially responded relapsed 12 weeks postoperatively. Therefore, six of 9 patients showed sustained responses with a mean follow-up of 2 years, consistent with other published series. Two patients had no response, one of whom died within 3 weeks of surgery from transformed Hodgkin's disease. The only other postoperative complication occurred in a patient who developed pneumonia. We conclude that laparoscopic splenectomy is a safe and effective treatment for autoimmune hemolytic anemia in patients with CLL who fail medical therapy. PMID:14978692

Hill, John; Walsh, R Matthew; McHam, Scott; Brody, Fred; Kalaycio, Matt

2004-03-01

70

A Rare Case of Rosai-Dorfman Disease in an Adult Male Associated with Auto-Immune Hemolytic Anemia  

PubMed Central

Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder predominantly of the lymph nodes, which mostly occurs in children and young adults typically presenting with lymphadenopathy. Our case is of a 63 year-old African-American male who presented with subjective fever, weight loss, bilateral axillary and inguinal lymphadenopathy as well as auto-immune hemolytic anemia. The histological analysis showed emperipolesis and histiocytes that were positive for S-100 and CD-68 consistent with RDD. After steroid treatment and splenectomy, patient抯 symptoms and hemolytic anemia had resolved. Our case is the first case of RDD reported to be associated with auto-immune hemolytic anemia in an adult. PMID:23667720

Sachdeva, Mickey; Abdulhaq, Haifaa

2013-01-01

71

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

PubMed

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

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

2014-07-14

72

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

PubMed Central

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

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

2011-01-01

73

Dapsone-associated Heinz body hemolytic anemia in a Cambodian woman with hemoglobin E trait.  

PubMed

A Cambodian woman with hemoglobin E trait (AE) and leprosy developed a Heinz body hemolytic anemia while taking a dose of dapsone (50 mg/day) not usually associated with clinical hemolysis. Her red blood cells (RBCs) had increased incubated Heinz body formation, decreased reduced glutathione (GSH), and decreased GSH stability. The pentose phosphate shunt activity of the dapsone-exposed AE RBCs was increased compared to normal RBCs. Although the AE RBCs from an individual not taking dapsone had increased incubated Heinz body formation, the GSH content and GSH stability were normal. The pentose phosphate shunt activity of the non-dapsone-exposed AE RBCs was decreased compared to normal RBCs. Thus, AE RBCs appear to have an increased sensitivity to oxidant stress both in vitro and in vivo, since dapsone does not cause hemolytic anemia at this dose in hematologically normal individuals. Given the influx of Southeast Asians into the United States, oxidant medications should be used with caution, especially if an infection is present, in individuals of ethnic backgrounds that have an increased prevalence of hemoglobin E. PMID:3425586

Lachant, N A; Tanaka, K R

1987-11-01

74

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

PubMed

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., 2014. 2014 Wiley Periodicals, Inc. PMID:25382665

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

2014-11-10

75

Combination of two mutant alpha spectrin alleles underlies a severe spherocytic hemolytic anemia.  

PubMed Central

We studied a patient with a severe spherocytic hemolytic anemia without family history of spherocytosis. Analysis of patient's erythrocyte membrane proteins revealed spectrin deficiency and a truncated alpha spectrin protein. We determined that the patient is a compound heterozygote with two mutations in alpha spectrin gene. Mutation in the paternal allele, designated alpha spectrin(PRAGUE), is a transition A to G in the penultimate position of intron 36 that leads to skipping of exon 37, frameshift, and production of the truncated alpha spectrin protein. The maternal allele, designated alpha spectrin(LEPRA), contains transition C-->T in position -99 of intron 30. This mutation enhances an alternative acceptor splice site 70 nucleotides upstream from the regular site. The alternative splicing causes a frameshift and premature termination of translation leading to a significant decrease in alpha spectrin production. The alpha(LEPRA) mutation is linked to a spectrin alphaIIa marker that was found to be associated with recessive or nondominant spectrin-deficient hereditary spherocytosis in approximately 50% of studied families. We conclude that the alpha(LEPRA) mutation combined in trans with the alpha(PRAGUE) mutation underlie the severe hemolytic anemia in the proband. We suggest that allele alpha spectrin(LEPRA) may be frequently involved in pathogenesis of recessive or nondominant spectrin-deficient hereditary spherocytosis. PMID:8941647

Wichterle, H; Hanspal, M; Palek, J; Jarolim, P

1996-01-01

76

Anemia hemol韙ica em c鉫s e gatos  

Microsoft Academic Search

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

Rafael Almeida Fighera

77

Erythropoietin as Treatment for Late Hyporegenerative Anemia in Neonates with Rh Hemolytic Disease after in utero Exchange Transfusion  

Microsoft Academic Search

We report the effects of recombinant human erythropoietin (rHuEPO) in the treatment of late hyporegenerative anemia in 2 neonates with Rh hemolytic disease who had received several in utero exchange transfusions. In both cases anemia occurred at 6 weeks of age and we started therapy at approximately 70 days of age. We used rHuEPO at 250 U\\/kg three times a

Claire Nicaise; Catherine Gire; Paul Casha; Claude d扙rcole; C閏ile Chau; Christian Palix

2002-01-01

78

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

SciTech Connect

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.

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

1983-05-20

79

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

PubMed

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 accompanied 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. PMID:6844918

Leighton, F A; Peakall, D B; Butler, R G

1983-05-20

80

Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology  

PubMed Central

Background The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from a survey on the prevalence and characteristics of patients diagnosed with HHA in Korea from 2007 to 2011. Methods Information on patients diagnosed with HHA in Korea and their clinical and laboratory results were collected using a survey questionnaire. Globin gene and red blood cell (RBC) enzyme analyses were performed. In addition, we analyzed data collected by pediatricians. Results In total, 195 cases of HHA were identified. Etiologies identified for HHA were RBC membranopathies, hemoglobinopathies, and RBC enzymopathies, which accounted for 127 (64%), 39 (19.9%), and 26 (13.3%) cases, respectively. Of the 39 patients with hemoglobinopathies, 26 were confirmed by globin gene analysis, including 20 patients with ?-thalassemia minor, 5 patients with ?-thalassemia minor, and 1 patient with unstable hemoglobin disease. Conclusion The number of patients diagnosed with hemoglobinopathies and RBC enzymopathies has increased considerably since the previous survey on HHA in Korea, dated from 1997 to 2006. This is likely the result of improved diagnostic techniques. Nevertheless, there is still a need for more sensitive diagnostic tests utilizing flow cytometry and for better standardization of test results to improve the accuracy of diagnosis of RBC membranopathies in Korea. Additionally, more accurate assays for the identification of RBC enzymopathies are warranted. PMID:24086942

Park, Eun Sil; Jung, Hye Lim; Kim, Hee-Jin; Park, Sung Sup; Bae, Soon Hwan; Shin, Hee Young; Song, Sang Hoon; Koh, Kyung-Nam; Lyu, Chuhl Joo; Lim, Young Tak; Han, Dong Kyun

2013-01-01

81

Occupational inhalation of aniline fumes induced methemoglobinemea and hemolytic anemia precipitated days later  

PubMed Central

Methylene blue is utilized as the main treatment of methemoglobinemia. Here we report two cases, in which patient suffered from aniline-induced methemoglobinemia with initial good response but later developed haemolytic anemia due to methylene blue therapy. He was treated with hydration, high flow oxygen and steroid therapy. Caution should be exercised while using methylene blue as antidote of acute methemoglobinemia as both methemoglobinemia and its antidote methylene blue can itself precipitate fatal haemolytic anemia.

Ravi Kumar, YS; Manthappa; Kumar, Prasanna; Prasad, MC; Radhika, AR; Edara, Amarendra Chowdary

2014-01-01

82

Heinz body hemolytic anemia in newborns and failure of laboratory studies to implicate a phenolic disinfectant.  

PubMed

Two unrelated, white, female, premature infants in the same hospital nursery contemporaneously exhibited features of an acute, Heinz body hemolytic anemia: decreased levels of hemoglobin and hematocrit, anisocytosis, fragmented cells, hyperbilirubinemia, reticulocytosis, and red cell inclusion bodies. Physical examination and laboratory studies failed to reveal the etiology of this process. Epidemiologic studies indicated a possible association between the reaction and the improper use and inappropriately high concentration of a phenolic disinfectant. Such an association has been suggested previously between similar products and epidemics of hyperbilirubinemia. Despite extensive experimental efforts (four species, six routes of administration, newborn rats, splenectomized rats, direct incubation with age-matched human cord blood), the reaction could not be produced in the laboratory. It may be highly specific for the intact, human, premature infant. Perhaps the hyperbilirubinemia reported previously had an erythrocytic rather than hepatic origin. PMID:6828342

Vitkun, S A; Smith, R P; French, E E; Edwards, W H; Watkins, N

1983-03-01

83

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

PubMed Central

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

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

2013-01-01

84

Acute hemolytic anemia, methemoglobinemia, and heinz body formation associated with ingestion of red maple leaves by horses.  

PubMed

From June 1975 through June 1979, acute hemolytic anemia developed in 11 horses from 7 New York farms. Of the 7 horses that died, 6 had methemoglobinemia. In the 4 horses that recovered, methemoglobinemia was not observed. but Heinz body formation was seen in 3 of the 4. On 2 of the premises involved, frank methemoglobinemia was observed concurrently with Heinz body formation, suggesting a relationship between the pathogenesis of methemoglobinemia and Heinz body formation in the hemolytic process. In addition to the 11 cases described, 22 clinically similar cases were reported to us during the period of this investigation by practicing veterinarians from New York, Pennsylvania, and the New England states. All 33 cases of hemolytic anemia occurred between June and October of each year, and affected horses had access to outside paddocks or fields containing a variety of native grasses, weeds, and trees. On 2 farms, hemolytic anemia developed after the horses were observed browsing fallen branches of red maple trees (Acer rubrum). Red maple leaves and bark were obtained from 1 of these farms, and approximately 1 kg of a leaf and bark mixture was fed to each of 2 ponies. Within 48 hours, both ponies became ill. The syndrome was indistinguishable from that observed in clinical patients and was characterized by methemoglobinemia and intravascular hemolysis. The ponies died 5 and 6 days after which time the packed cell volumes were 6% and 7% respectively. It was concluded that many cases of hemolytic anemia in horses in northeastern states may be related to ingestion of leaves or bark from red maple trees. The studies did not, however, define the factors that predispose to poisoning and did not exclude the possibility that other environmental toxins may have been involved. PMID:7263466

Tennant, B; Dill, S G; Glickman, L T; Mirro, E J; King, J M; Polak, D M; Smith, M C; Kradel, D C

1981-07-15

85

Marrow transplantation in the treatment of a murine heritable hemolytic anemia  

SciTech Connect

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.

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

1989-05-15

86

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

PubMed Central

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

Masoodi, Ibrahim

2014-01-01

87

Co-infection with Nocardia asteroides complex and Strongyloides stercoralis in a patient with autoimmune hemolytic anemia.  

PubMed

We describe an unusual case of pulmonary nocardiosis co-existing with Strongyloides stercoralis hyperinfection syndrome in a patient with autoimmune hemolytic anemia who was being treated with corticosteroids. This case highlights the importance of being aware of the possibility that infections can co-exist in immunosuppressed patients. To the best of our knowledge, this is the first report of co-infection with Nocardia asteroides and S. stercoralis. PMID:23925638

Praharaj, I; Sujatha, S; Ashwini, M A; Parija, S C

2014-02-01

88

Megadose Methylprednisolone (MDMP) Treatment in a Patient with Autoimmune Hemolytic Anemia (AIHA) Resistant to Conventional Corticosteroid Administration: A Case Report  

PubMed Central

A female in the Netherlands with severe autoimmune hemolytic anemia (AIHA) was treated with conventional corticosteroid (2 mg/kg/d in divided doses) and blood transfusions for 18 months without improvement. The presented patient responded to megadose methylprednisolone (MDMP) 30 mg/kg/d for 3 d, followed by 20 mg/kg for 4 d, and subsequently 10, 5, 2, and 1 mg/kg/d each for 1 week. Conflict of interest:None declared. PMID:24385786

謟soylu, ?inasi; Berenschot, Henriette WA

2013-01-01

89

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

PubMed Central

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

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

90

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

SciTech Connect

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.

Datta, T.; Doermer, P.

1987-12-01

91

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

PubMed

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

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

2014-01-01

92

Heinz body hemolytic anemia associated with high plasma zinc concentration in a dog.  

PubMed

Acute zinc toxicosis from the ingestion of pennies was diagnosed in a dog with Heinz body hemolytic anemia (PCV = 14%), leukocytosis (51,000 cells/ml) with a left shift (3,060 band neutrophils; 37,740 segmented neutrophils) and monocytosis (4,080 cells/ml), azotemia (BUN = 60 mg/dl), bilirubinemia (total bilirubin = 5.3 mg/dl), hypokalemia (3.0 mEq/L), high serum alkaline phosphatase activity (691 U/L), high total plasma solids (8.1 g/dl), hemoglobinuria, and proteinuria. Despite aggressive medical treatment, renal failure ensued, and the dog died of cardiac arrest. The clinical signs, clinical course, and laboratory findings in this dog were similar to what has been reported in other cases of acute zinc toxicosis in dogs, with the exception of a history of generalized seizures and the findings of Heinz bodies. Although a causative relationship between plasma zinc values and Heinz body formation cannot be proven, their association suggests that oxidative damage to erythrocyte hemoglobin and cell membrane proteins may be involved in the pathogenesis of zinc-induced hemolysis. PMID:2266050

Luttgen, P J; Whitney, M S; Wolf, A M; Scruggs, D W

1990-11-15

93

Hemolytic anemia, thrombosis, and infarction in male and female F344 rats following gavage exposure to 2-butoxyethanol.  

PubMed

2-butoxyethanol (BE; ethylene glycol monobutyl ether) is used extensively in the manufacture of a wide range of domestic and industrial products which may result in human exposure and toxicity. BE causes severe hemolytic anemia in male and female rats and mice. In a recent report, female F344 rats exposed to 500 ppm BE by inhalation and sacrificed moribund on day 4 of treatment exhibited disseminated thrombosis associated with infarction in several organs. In contrast, no such lesions were observed in male rats similarly exposed to BE. Additional studies were therefore undertaken to compare the effects of BE in rats of both sexes. Rats received 250 mg BE/kg/day by gavage for 1, 2 or 3 days and were sacrificed 24 or 48 hr after the last dose. Control rats received 5 ml/kg water. Progressive time-dependent hemolytic anemia--macrocytic, hypochromic, and regenerative--was observed in both sexes of rats exposed to BE. Additionally, BE caused significant morphological changes in erythrocytes, first observed 24 hr after a single dose, including stomatocytosis, macrocytosis with moderate rouleaux formation, and spherocytosis. These morphological changes became progressively more severe as BE dosing continued and included the occasional occurrence of schistocytes and ghost cells, rouleaux formation in rats of both sexes, and an increased number of red blood cells with micronuclei in female rats. Overall, the progression of hemolytic anemia and morphological changes as a function of the number of days of exposure varied with gender and suggested a faster onset of hemolysis in female rats. The range of BE-related histopathological changes noted in both sexes was comparable; however, while these lesions were observed in female rats following a single dose, similar effects were first observed in males after 3 consecutive days of exposure to BE. Pathological changes involved disseminated thrombosis in the lungs, nasal submucosa, eyes, liver, heart, bones and teeth, with evidence of infarction in the heart, eyes, teeth and bones. Hemoglobinuric nephrosis and splenic extramedullary hematopoiesis were also noted. An apparent correlation between the severity of hemolytic anemia and subsequent disseminated thrombosis in BE-treated rats is proposed. Thrombosis may be related to intravascular hemolysis, which could be triggered by procoagulant release and/or alterations in erythrocyte morphology, as well as increased rigidity. PMID:11484844

Ghanayem, B I; Long, P H; Ward, S M; Chanas, B; Nyska, M; Nyska, A

2001-06-01

94

Heinz body hemolytic anemia with eccentrocytosis from ingestion of Chinese chive (Allium tuberosum) and garlic (Allium sativum) in a dog.  

PubMed

A 4-year-old, intact male miniature schnauzer was presented with anorexia. The dog had ingested some Chinese steamed dumplings 2 days before, which contained Chinese chive (Allium tuberosum) and garlic (Allium sativum). Hematological examinations revealed severe Heinz body hemolytic anemia with eccentrocytosis and an increased concentration of methemoglobin, which was thought to result from oxidative damage to erythrocytes by constituents in these Allium plants. In this case, eccentrocytosis was a hallmark finding and could be detected easily, suggesting that this hematological abnormality is useful in diagnosing Allium plant-induced hemolysis. PMID:15634869

Yamato, Osamu; Kasai, Ei; Katsura, Taro; Takahashi, Shinichi; Shiota, Takuji; Tajima, Motoshi; Yamasaki, Masahiro; Maede, Yoshimitsu

2005-01-01

95

Cold antibody autoimmune hemolytic anemia and lymphoproliferative disorders: a retrospective study of 20 patients including clinical, hematological, and molecular findings.  

PubMed

A total of 20 patients with cold antibody hemolytic anemia were evaluated in a retrospective study of them, 15 had a monoclonal gammopathy of unknown significance (MGUS): 14 with MGUS of immunoglobulin M (IgM) subtype and 1 with immunoglobulin G subtype. One patient had smoldering Waldenstr鰉's macroglobulinemia, but four patients had no monoclonal protein and no evidence of lymphoma. However, in three of these patients, we were able to demonstrate a (mono-)clonal rearrangement of their immunoglobulin heavy and/or light chains. Of the 20 patients, 5 had IgHV34 nucleotide sequence indicating that the antibody was directed against the "I" antigen. Two patients exhibited a progressive increase of IgM over time, however without increasing hemolytic activity. Moreover, in two patients with long-term follow-up, we were able to correlate recurrent hemolytic activity with low environmental temperatures. Among four patients treated with rituximab, all four responded to treatment. However, treatment effect was only transient in all of them. PMID:24842748

Arthold, Cathrin; Skrabs, Cathrin; Mitterbauer-Hohendanner, Gerlinde; Thalhammer, Renate; Simonitsch-Klupp, Ingrid; Panzer, Simon; Valent, Peter; Lechner, Klaus; J鋑er, Ulrich; Sillaber, Christian

2014-06-01

96

Hb Manukau [?67(E11)Val???Gly; HBB: c.203T>G]: the role of genetic testing in the diagnosis of idiopathic hemolytic anemia.  

PubMed

The increasing availability of DNA sequencing of globin genes has improved our ability to detect conditions that were presumed to be extremely rare. These conditions may remain undiagnosed due to unfamiliarity with clinical presentation, relative unavailability of advanced diagnostic alternatives, or may defy detection by being electrophoretically silent or extreme instability rendering their presence to be below detection level. Genetic studies were pursued in a mother and daughter with severe hemolytic anemia as initial testing failed to be diagnostic. DNA sequence analysis of the ?-globin gene identified Hb Manukau [?67(E11)Val???Gly; HBB: c.203T?>?G], an extremely unstable hemoglobin (Hb) variant. This is the second family described with this condition (first in the western hemisphere). An astute clinician may benefit from being persistent and pursuing additional testing including molecular genetic characterization where clinical suspicion remains high. PMID:24611675

Kumar, Mudra Kohli; Judd, Courtney; Hoyer, James D; Swanson, Kenneth C; Nelson, Linda; Oliveira, Jennifer L

2014-01-01

97

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

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

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

1997-01-01

98

Nrf2 and selenoproteins are essential for maintaining oxidative homeostasis in erythrocytes and protecting against hemolytic anemia.  

PubMed

Reactive oxygen species (ROS) are highly destructive toward cellular macromolecules. However, moderate levels of ROS can contribute to normal cellular processes including signaling. Herein we evaluate the consequence of a pro-oxidant environment on hematopoietic homeostasis. The NF-E2 related factor 2 (Nrf2) transcription factor regulates genes related to ROS scavenging and detoxification. Nrf2 responds to altered cellular redox status, such as occurs with loss of antioxidant selenoproteins after deletion of the selenocysteine-tRNA gene (Trsp). Conditional knockout of the Trsp gene using Mx1-inducible Cre-recombinase leads to selenoprotein deficiency and anemia on a wild-type background, whereas Trsp:Nrf2 double deficiency dramatically exacerbates the anemia and increases intracellular hydrogen peroxide levels in erythroblasts. Results indicate that Nrf2 compensates for defective ROS scavenging when selenoproteins are lost from erythroid cells. We also observed thymus atrophy in single Trsp-conditional knockout mice, suggesting a requirement for selenoprotein function in T-cell differentiation within the thymus. Surprisingly, no changes were observed in the myelomonocytic or megakaryocytic populations. Therefore, our results show that selenoprotein activity and the Nrf2 gene battery are particularly important for oxidative homeostasis in erythrocytes and for the prevention of hemolytic anemia. PMID:20978266

Kawatani, Yukie; Suzuki, Takafumi; Shimizu, Ritsuko; Kelly, Vincent P; Yamamoto, Masayuki

2011-01-20

99

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

PubMed Central

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

2014-01-01

100

[Hemolytic anemia after voluntary ingestion of henna (Lawsonia inermis) decoction by a young girl with G6PD deficiency].  

PubMed

Henna (Lawsonia inermis) is a shrub bearing leaves that are crushed and used for cosmetic purposes in Asia and Africa. In several countries, henna decoction is ingested as a traditional drug to induce abortion. One component of Henna, known as Lawsone, can induce hemolysis in G6PD-deficient patients after cutaneous exposure or ingestion. The purpose of this report is to describe a case of severe hemolytic anemia after voluntary ingestion of Henna decoction to induce abortion. This complication led to diagnosis of partial moderate G6PD-deficiency in the 17-year-old patient living in Mayotte in the Indian Ocean. This report emphasizes the life-threatening hazards associated with some plant extracts used as traditional medicines. PMID:21870562

Perinet, I; Lioson, E; Tichadou, L; Glaizal, M; de Haro, L

2011-06-01

101

Mutation in erythroid specific transcription factor KLF1 causes Hereditary Spherocytosis in the Nan hemolytic anemia mouse model.  

PubMed

KLF1 regulates definitive erythropoiesis of red blood cells by facilitating transcription through high affinity binding to CACCC elements within its erythroid specific target genes including those encoding erythrocyte membrane skeleton (EMS) proteins. Deficiencies of EMS proteins in humans lead to the hemolytic anemia Hereditary Spherocytosis (HS) which includes a subpopulation with no known genetic defect. Here we report that a mutation, E339D, in the second zinc finger domain of KLF1 is responsible for HS in the mouse model Nan. The causative nature of this mutation was verified with an allelic test cross between Nan/+ and heterozygous Klf1(+/-) knockout mice. Homology modeling predicted Nan KLF1 binds CACCC elements more tightly, suggesting that Nan KLF1 is a competitive inhibitor of wild-type KLF1. This is the first association of a KLF1 mutation with a disease state in adult mammals and also presents the possibility of being another causative gene for HS in humans. PMID:20691777

Heruth, Daniel P; Hawkins, Troy; Logsdon, Derek P; Gibson, Margaret I; Sokolovsky, Inna V; Nsumu, Ndona N; Major, Stephanie L; Fegley, Barbara; Woods, Gerald M; Lewing, Karen B; Neville, Kathleen A; Cornetta, Kenneth; Peterson, Kenneth R; White, Robert A

2010-11-01

102

Heinz bodies, methemoglobinemia, and hemolytic anemia induced in rats by 3-amino-1-[m-(trifluoromethyl)phenyl]-2-pyrazoline.  

PubMed

Sprague-Dawley CD strain rats were given 18, 35, 70, or 140 mg/kg/day of 3-amino-1-[m-(trifluoromethyl)phenyl]-2-pyrazoline by gavage for 2 weeks. Heinz bodies were seen in the erythrocytes of rats given 140 mg/kg/day. Dose-related increases in methemoglobin were found at 35 mg/kg/day or more. Hemolytic anemia was characterized by dose-related decreases in hematocrit, hemoglobin, and total erythrocyte count. Reticulocytosis, decreased myeloid:erythroid ratio, splenomegaly, extramedullary hematopoiesis, increased serum total bilirubin, and icterus were also observed. This compound was found to oxidize oxyhemoglobin to methemoglobin in vitro, suggesting that the parent compound is capable of causing the hematological changes observed in vivo without conversion to active metabolites. PMID:6427045

Fort, F L; Pratt, M C; Carter, G W; Lewkowski, J P; Heyman, I A; Cusick, P K; Kesterson, J W

1984-04-01

103

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

PubMed Central

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抯 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

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

2014-01-01

104

A possible mechanism of heinz body hemolytic anemia induced by DQ-2511, a new gastroprokinetic drug, in dogs.  

PubMed

A previous study revealed that DQ-2511, a new gastroprokinetic drug, induced hemolytic anemia together with increased Heinz body formation, preceded by a marked decrease in erythrocyte reduced glutathione (GSH) content, after 2 weeks of dosing onward in dogs. In this study, the effect of DQ-2511 on erythrocytes in the early period of dosing, in comparison with that of beta-acetylphenylhydrazine (APHZ), was investigated to confirm the difference between this drug and APHZ in the mechanism of increased Heinz body formation. DQ-2511 and APHZ were administered orally to beagle dogs for 1 week at dose levels of 600 and 4 mg/ kg, respectively. Dogs receiving APHZ showed anemia after dosing for 7 days, together with an increase in methemoglobin and Heinz body formation after 3 days of dosing. In contrast, blood GSH, glutathione reductase, and gamma-glutamylcysteine synthetase were only slightly decreased after dosing for 7 days. In dogs treated with DQ-2511, erythrocyte GSH began to decrease after 1 day of treatment and was about 25% of the control value after 7 days; however, no changes were seen in blood glutathione reductase, GSH peroxidase, or gamma-glutamylcysteine synthetase level. Hepatic GSH was decreased slightly. In another experiment, SD rats were administered DQ-2511 and APHZ orally for 1 week at dose levels of 1600 and 15 mg/kg, respectively. Rats receiving DQ-2511 showed no anemia or any changes in erythrocyte GSH and Heinz body formation. In contrast, rats treated with APHZ showed a marked anemia and increases in Heinz body formation and erythrocyte GSH. These results demonstrate that DQ-2511 causes a marked decrease in GSH in dogs, resulting in Heinz body anemia, whereas APHZ induces Heinz body formation after a significant increase in methemoglobin, and suggest that impairment of the GSH redox cycle and synthetases of GSH are not involved in the decreased GSH after DQ-2511 treatment. This difference in effects on GSH content may indicate the existence of a species difference in the anemia induced by DQ-2511. PMID:8921330

Ohno, H; Nomura, M; Watanabe, K

1996-08-01

105

Common variable immunodeficiency complicated with hemolytic uremic syndrome  

PubMed Central

Common variable immunodeficiency is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. Recurrent pyogenic infections of upper and lower respiratory tracts are the main clinical manifestations of common variable immunodeficiency. Hemolytic uremic syndrome is a multisystemic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia due to platelet aggregation in the arterial microvasculature. This is one of the rare cases of patients diagnosed with common variable immunodeficiency, which was complicated by hemolytic uremic syndrome. PMID:22059898

2012-01-01

106

Concurrent Infections with Vector-Borne Pathogens Associated with Fatal Hemolytic Anemia in a Cattle Herd in Switzerland  

PubMed Central

Bovine anaplasmosis is a vector-borne disease that results in substantial economic losses in other parts of the world but so far not in northern Europe. In August 2002, a fatal disease outbreak was reported in a large dairy herd in the Swiss canton of Grisons. Diseased animals experienced fever, anorexia, agalactia, and depression. Anemia, ectoparasite infestation, and, occasionally, hemoglobinuria were observed. To determine the roles of vector-borne pathogens and to characterize the disease, blood samples were collected from all 286 animals: 50% of the cows were anemic. Upon microscopic examination of red blood cells, Anaplasma marginale inclusion bodies were found in 47% of the cows. The infection was confirmed serologically and by molecular methods. Interestingly, we also found evidence of infections with Anaplasma phagocytophilum, large Babesia and Theileria spp., and Mycoplasma wenyonii. The last two species had not previously been described in Switzerland. Anemia was significantly associated with the presence of the infectious agents detected, with the exception of A. phagocytophilum. Remarkably, concurrent infections with up to five infectious vector-borne agents were detected in 90% of the ill animals tested by PCR. We concluded that A. marginale was the major cause of the hemolytic anemia, while coinfections with other agents exacerbated the disease. This was the first severe disease outbreak associated with concurrent infections with vector-borne pathogens in alpine Switzerland; it was presumably curtailed by culling of the entire herd. It remains to be seen whether similar disease outbreaks will have to be anticipated in northern Europe in the future. PMID:15297529

Hofmann-Lehmann, Regina; Meli, Marina L.; Dreher, Ute M.; G鰊czi, Enik; Deplazes, Peter; Braun, Ueli; Engels, Monika; Sch黳bach, J鰎g; J鰎ger, Kaspar; Thoma, Rudolf; Griot, Christian; St鋜k, Katharina D. C.; Willi, Barbara; Schmidt, Joseph; Kocan, Katherine M.; Lutz, Hans

2004-01-01

107

The prognostic significance of a positive direct antiglobulin test in chronic lymphocytic leukemia: a beneficial effect of the combination offludarabine and cyclophosphamide on the incidence of hemolytic anemia  

Microsoft Academic Search

Autoimmune hemolytic anemia (AHA) is a common complication in chronic lympho- cytic leukemia (CLL). The UK LRF CLL4 trial is the largest prospective trial in CLL to examine the prognostic impact of both a positive direct antiglobulin test (DAT) and AHA. Seven-hundred seventy-seven patients were randomized to receive chlorambucil or fludarabine, alone or with cyclophosphamide (FC). The incidence pretreatment of

Claire Dearden; Rachel Wade; Sue Richards; Don Milligan; Terry Hamblin; Daniel Catovsky

108

Diet-associated hepatic-failure and immune-mediated hemolytic anemia in a Weimaraner  

Microsoft Academic Search

Objective: To describe the medical and nutritional management of a 4-year-old Weimaraner with acute hepatic failure and immune-mediated haemolytic anemia (IMHA) associated with consuming a commercial dog food. Case summary: A 4-year-old male castrated Weimaraner developed signs of IMHA, hepatic failure, disseminated intravascular coagulopathy, and malnutrition after consuming a commercial dog food. During the course of hospitalization, medical management included

Ashley J. Smith; Katherine A. Stenske; Joseph W. Bartges; Claudia A. Kirk

2006-01-01

109

Acute Heinz-body anemia due to severe cresol poisoning: successful treatment with erythrocytapheresis.  

PubMed

A patient with massive intravascular Heinz-body hemolytic anemia associated with the presence of bizarre-looking erythrocytes following the oral ingestion of approximately 100 mL of "penetrating oil", a petroleum distillate containing 85% kerosene, 12% cresol and 2% surfactant, is described. He was treated successfully with immediate erythrocytapheresis and forced diuresis. PMID:6722696

C魌, M A; Lyonnais, J; Leblond, P F

1984-05-15

110

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

NSDL National Science Digital Library

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.

Robert A. Brodsky (Johns Hopkins Medicine)

2008-04-15

111

A Deep Intronic Mutation in the Ankyrin-1 Gene Causes Diminished Protein Expression Resulting in Hemolytic Anemia in Mice  

PubMed Central

Linkage between transmembrane proteins and the spectrin-based cytoskeleton is necessary for membrane elasticity of red blood cells. Mutations of the proteins that mediate this linkage result in various types of hemolytic anemia. Here we report a novel N-ethyl-N-nitrosourea?induced mutation of ankyrin-1, named hema6, which causes hereditary spherocytosis in mice through a mild reduction of protein expression. The causal mutation was traced to a single nucleotide transition located deep into intron 13 of gene Ank1. In vitro minigene splicing assay revealed two abnormally spliced transcripts containing cryptic exons from fragments of Ank1 intron 13. The inclusion of cryptic exons introduced a premature termination codon, which leads to nonsense-mediated decay of the mutant transcripts in vivo. Hence, in homozygous mice, only wild-type ankyrin-1 is expressed, albeit at 70% of the level in wild-type mice. Heterozygotes display a similar hereditary spherocytosis phenotype stemming from intermediate protein expression level, indicating the haploinsufficiency of the mutation. Weakened linkage between integral transmembrane protein, band 3, and underlying cytoskeleton was observed in mutant mice as the result of reduced high-affinity binding sites provided by ankyrin-1. Hema6 is the only known mouse mutant of Ank1 allelic series that expresses full-length canonical ankyrin-1 at a reduced level, a fact that makes it particularly useful to study the functional impact of ankyrin-1 quantitative deficiency. PMID:23934996

Huang, Hua; Zhao, PengXiang; Arimatsu, Kei; Tabeta, Koichi; Yamazaki, Kazuhisa; Krieg, Lara; Fu, Emily; Zhang, Tian; Du, Xin

2013-01-01

112

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

PubMed

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

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

113

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

PubMed

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

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; Fo, Robin; Neri, Antonino; Rodeghiero, Francesco; Cortelezzi, Agostino

2013-01-01

114

Absence of Mitochondrial Superoxide Dismutase Results in a Murine Hemolytic Anemia Responsive to Therapy with a Catalytic Antioxidant  

PubMed Central

Manganese superoxide dismutase 2 (SOD2) is a critical component of the mitochondrial pathway for detoxification of O2?, and targeted disruption of this locus leads to embryonic or neonatal lethality in mice. To follow the effects of SOD2 deficiency in cells over a longer time course, we created hematopoietic chimeras in which all blood cells are derived from fetal liver stem cells of Sod2 knockout, heterozygous, or wild-type littermates. Stem cells of each genotype efficiently rescued hematopoiesis and allowed long-term survival of lethally irradiated host animals. Peripheral blood analysis of leukocyte populations revealed no differences in reconstitution kinetics of T cells, B cells, or myeloid cells when comparing Sod2+/+, Sod2?/?, and Sod2+/? fetal liver recipients. However, animals receiving Sod2?/? cells were persistently anemic, with findings suggestive of a hemolytic process. Loss of SOD2 in erythroid progenitor cells results in enhanced protein oxidative damage, altered membrane deformation, and reduced survival of red cells. Treatment of anemic animals with Euk-8, a catalytic antioxidant with both SOD and catalase activities, significantly corrected this oxidative stress杋nduced condition. Such therapy may prove useful in treatment of human disorders such as sideroblastic anemia, which SOD2 deficiency most closely resembles. PMID:11304553

Friedman, Jeff S.; Rebel, Vivienne I.; Derby, Ryan; Bell, Kirsten; Huang, Ting-Ting; Kuypers, Frans A.; Epstein, Charles J.; Burakoff, Steven J.

2001-01-01

115

Hemolytic anemia in hereditary pyrimidine 5'-nucleotidase deficiency: nucleotide inhibition of G6PD and the pentose phosphate shunt.  

PubMed

We evaluated the erythrocytes of two patients with hereditary pyrimidine 5'-nucleotidase deficiency. Significant findings included an increased reduced glutathione content, increased incubated Heinz body formation, a positive ascorbate cyanide test, and decreased intraerythrocytic pH. The pentose phosphate shunt activity of the patients' red cells as measured by the release of 14CO2 from 14C-1-glucose was decreased compared to high reticulocyte controls. Glucose-6-phosphate dehydrogenase (G6PD) activity in hemolysates from control erythrocytes was inhibited 43% by 5.5 mM cytidine 5'-triphosphate (CTP) and 50% by 5.5 mM in uridine 5'-triphosphate (UTP) at pH 7.1. CTP was a competitive inhibitor for G6P (Ki = 1.7 mM) and a noncompetitive inhibitor for NADP+ (Ki = 7.8 mM). Glutathione peroxidase, glutathione reductase, and 6-phosphogluconate dehydrogenase were not affected by these compounds. Pentose phosphate shunt activity in control red cell hemolysate at pH 7.1 was inhibited to a similar degree by 5.5 mM CTP or UTP. Since the intracellular concentrations of G6P and NADP+ are below their KmS for G6PD, these data suggest that high concentrations of pyrimidine 5'-nucleotides depress pentose phosphate shunt activity in pyrimidin 5'-nucleotidase deficiency. Thus, this impairment of the pentose phosphate pathway appears to contribute to the pathogenesis of hemolysis in pyrimidine 5'-nucleotidase deficiency hemolytic anemia. PMID:6289944

Tomoda, A; Noble, N A; Lachant, N A; Tanaka, K R

1982-11-01

116

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

SciTech Connect

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.

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

1990-01-01

117

[Progression of the peripheral blood profile in phenylhydrazine-induced hemolytic anemia].  

PubMed

Male rabbits were made anemic by subcutaneous injections of 3 mg/Kg body weight of phenylhydrazine (PHZ) for the duration of three days. Blood samples were collected prior to the experiment, on the 2nd and 5th day from the end of the treatment. Hematocrit, reticulocyte count, MCV, osmotic resistance, RBC volume distribution curve and morphological analysis were performed on each sample in order to obtain a picture of the progressive changes of the parameters following the PHZ administration. Hematocrit values changed from 49 to 27%, whereas the reticulocytosis increased from 0.6 to 73%. A significant elevation of MCV was detected and the cell volume distribution curve, which presented the typical bell-shape profile in the normal animals became bimodal on the 5th day. The osmotic resistance of RBC of anemic animals, showed a marked deviation from the normal pattern. In fact initial haemolysis started at 0.75-0.70% NaCl concentration in the 5th day samples, while it was between 0.55-0.50% before the PHZ treatment. Finally, morphological analysis revealed a progressive increase of RBC with Heinz bodies, of macro-megalocytes and of immature erythroblasts thus indicating that the cell population, produced during recovery from PHZ induced anemia, is widely heterogeneous. PMID:2291827

Piacentini, G; Baronciani, L; Rapa, S; Benedetti, C; Ninfali, P

1990-08-01

118

Both hemolytic anemia and malaria parasite-specific factors increase susceptibility to Nontyphoidal Salmonella enterica serovar typhimurium infection in mice.  

PubMed

Severe pediatric malaria is an important risk factor for developing disseminated infections with nontyphoidal Salmonella serotypes (NTS). While recent animal studies on this subject are lacking, early work suggests that an increased risk for developing systemic NTS infection during malaria is caused by hemolytic anemia, which leads to reduced macrophage microbicidal activity. Here we established a model for oral Salmonella enterica serotype Typhimurium challenge in mice infected with Plasmodium yoelii nigeriensis. Initial characterization of this model showed that 5 days after coinoculation, P. yoelii nigeriensis infection increased the recovery of S. Typhimurium from liver and spleen by approximately 1,000-fold. The increased bacterial burden could be only partially recapitulated by antibody-mediated hemolysis, which increased the recovery of S. Typhimurium from liver and spleen by 10-fold. These data suggested that both hemolysis and P. yoelii nigeriensis-specific factors contributed to the increased susceptibility to S. Typhimurium. The mechanism by which hemolysis impaired resistance to S. Typhimurium was further investigated. In vitro, S. Typhimurium was recovered 24 h after infection of hemophagocytic macrophages in 2-fold-higher numbers than after infection of mock-treated macrophages, making it unlikely that reduced macrophage microbicidal activity was solely responsible for hemolysis-induced immunosuppression during malaria. Infection with P. yoelii nigeriensis, but not antibody-mediated hemolysis, reduced serum levels of interleukin-12p70 (IL-12p70) in response to S. Typhimurium challenge. Collectively, studies establishing a mouse model for this coinfection suggest that multiple distinct malaria-induced immune defects contribute to increased susceptibility to S. Typhimurium. PMID:20100860

Roux, Christelle M; Butler, Brian P; Chau, Jennifer Y; Paixao, Tatiane A; Cheung, Kong Wai; Santos, Renato L; Luckhart, Shirley; Tsolis, Ren閑 M

2010-04-01

119

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

PubMed Central

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

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

1981-01-01

120

Rapid, sensitive diagnosis of hemolytic anemia using antihemoglobin antibody in hypotonic solution.  

PubMed

We have developed a new and simple flow cytometric method to detect damaged red blood cells (RBCs) using anti-Hb in hypotonic solution. We studied a total of 200 patients, including 62 patients with schistocytosis, 8 postsplenectomy patients, and 108 healthy controls. Peripheral blood (2 microl) was stained with phycoerythrin-conjugated (PE) antihemoglobin antibody (anti-Hb) in 0.6% (w/v) NaCl solution, and analyzed by flow cytometry omitting the washing step. The proportion of RBCs stained by anti-Hb was 0.55% (SD +/-0.23%) in normal controls and was significantly higher in patients with schistocytosis (2.95+/-2.95%, p <0.001). Six of 108 blood samples from normal controls and 60 of 62 samples from schistocytosis patients showed > or =1.01% stained RBCs (ie, values > mean+2SD of normal controls). The number of schistocytes counted by microscopic examination correlated with the proportion of RBCs stained by anti-Hb (r = 0.637, p <0.001). The proportions of stained RBCs in blood samples with malaria, spherocytosis, and elliptocytosis were also significantly higher than in normal controls. However, the results in postsplenectomy and iron-deficiency anemia (IDA) patients were not significantly different from the normal controls; the number of schistocytes in postsplenectomy patients was not related to the proportion of RBCs stained by anti-Hb. Based on these findings, flow cytometry of damaged RBCs using anti-Hb in hypotonic solution is a simple, sensitive, and accurate method to detect active hemolysis. PMID:11848616

Lee, Wonbae; Kim, Yonggoo; Lim, Jihyang; Kim, Myungshin; Lee, Eun Jung; Lee, Ahwon; Lee, Kyo Young; Kang, Chang Suk; Kim, So-Young; Han, Kyungja; Pai, Soo Hwan

2002-01-01

121

Lenoir et al., 1 Iron deficiency anemia due to matriptase-2 inactivation is dependent upon  

E-print Network

Lenoir et al., 1 1 Iron deficiency anemia due to matriptase-2 inactivation is dependent upon, hepcidin, anemia, Bmp6, hemojuvelin inserm-00552073,version1-5Jan2011 Author manuscript, published by increased hepcidin levels and anemia) and Bmp6-/- mice (exhibiting severe iron overload due to hepcidin

Paris-Sud XI, Universit茅 de

122

Drugs that have been shown to cause drug-induced immune hemolytic anemia or positive direct antiglobulin tests: some interesting findings since 2007.  

PubMed

This review updates new findings in drug-induced immune- hemolytic anemia (DIIHA) since the 2007 review in Immunohematology by these authors. Twelve additional drugs have been added to the three tables listing drugs associated with drug-dependent antibodies, drugs associated with drug-independent antibodies, and drugs associated with nonimmunologic protein adsorption. Other updated findings include (1) piperacillin is currently the most commonly encountered cause of DIIHA, (2) new data on blood group specificity of drug-dependent antibodies, (3) drug-dependent antibodies detected in healthy donors, (4) DIIHA associated with transplantation, and(5) DIIHA associated with chemotherapeutic drugs. PMID:25247621

Garratty, George; Arndt, Patricia A

2014-01-01

123

Early decline of hemoglobin correlates with progression of ribavirin-induced hemolytic anemia during interferon plus ribavirin combination therapy in patients with chronic hepatitis C  

Microsoft Academic Search

Background牋The aim of this study was to examine the factors correlated with the progression of ribavirin-induced hemolytic anemia in\\u000a patients with chronic hepatitis C treated by interferon and ribavirin combination therapy.\\u000a \\u000a \\u000a \\u000a Methods牋This study was conducted on 505 patients by the Osaka Liver Disease Study Group. A decline of hemoglobin (Hb) concentration\\u000a by 2?g\\/dl at the end of 2 weeks from

Tsugiko Oze; Naoki Hiramatsu; Nao Kurashige; Natsuko Tsuda; Takayuki Yakushijin; Tatsuya Kanto; Tetsuo Takehara; Akinori Kasahara; Michio Kato; Harumasa Yoshihara; Kazuhiro Katayama; Shinji Kubota; Taizo Hijioka; Kazunobu Ishibashi; Masahide Oshita; Hideki Hagiwara; Yoshimichi Haruna; Eiji Mita; Shinji Tamura; Norio Hayashi

2006-01-01

124

Characterization of a phosphoglycerate kinase deficiency variants not associated with hemolytic anemia.  

PubMed Central

The properties of a variant phosphoglycerate kinase (PGK) found in a large German clan were examined. The normal and variant enzymes, isolated by affinity chromatography, have the same molecular weight, specific activity, substrate affinity, and nearly identical pH-optima. Using immunoinactivation and immunodiffusion, the same specific activity for both forms was again determined. Since the enzymatic activity in older and younger erythrocytes varied only slightly, and since the specific activity of the variant was normal, the variant seems to be stable in vivo. This suggests that the decreased enzyme content is due to a decreased synthesis rate. The variant PGK described here is distinctly different from the known PGK variants and has been designated as "PGK M黱chen." PMID:6770677

Krietsch, W K; Eber, S W; Haas, B; Ruppelt, W; Kuntz, G W

1980-01-01

125

Model mice for Presbyterian hemoglobinopathy (Asn(beta108)-->Lys) confer hemolytic anemia with altered oxygen affinity and instability of Hb.  

PubMed

Hb Presbyterian is a variant hemoglobin that carries Lys at Asn-108 of beta-globin. This variant Lys(beta108) residue enhances the stability of Hb in the deoxy-state, conferring the low affinity for oxygen-binding in vitro. In the present study, we generated mutant mice carrying the Presbyterian mutation (Asn(beta108)-->Lys) at the beta-globin locus by a targeted knock-in strategy. Heterozygous mice showed the expression of Hb Presbyterian in 27.7% of total peripheral blood without any hematological abnormalities, which well mimicked human cases. On the other hand, homozygous mice exclusively expressed Hb Presbyterian in 100% of peripheral blood associated with hemolytic anemia, Heinz body formation, and splenomegaly. Hb Presbyterian showed instability in an in vitro precipitation assay. Erythrocytes from homozygous mice showed a shortened life span when transfused into wild-type mice, confirming that the knocked-in mutation of Lys(beta108) caused hemolysis in homozygous mice. This is the first report on the hemolytic anemia of unstable hemoglobin in an animal model. These results confirm the notion that the higher ratio of an unstable variant beta-globin chain in erythrocytes triggers the pathological precipitation and induces hemolysis in abnormal hemoglobinopathies. PMID:12127975

Suzuki, Yo-ichi; Shimizu, Takahiko; Sakai, Hiromi; Tamaki, Masakatsu; Koizumi, Ken ichi; Kuriyama, Takayuki; Tsuchida, Eishun; Koseki, Haruhiko; Shirasawa, Takuji

2002-07-26

126

Types of Hemolytic Anemia  

MedlinePLUS

... affect people of Southeast Asian, Indian, Chinese, Filipino, Mediterranean, or African origin or descent. Hereditary Spherocytosis In ... G6PD deficiency mostly affects males of African or Mediterranean descent. In the United States, the condition is ...

127

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 ...

128

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 ...

129

Anemias  

Microsoft Academic Search

Anemias can be secondary to a primary blood disorder (e.g., stem cell failure in aplastic anemia, malignant transformation\\u000a of stem cells in acute leukemias), but more often, anemias are secondary to other diseases or conditions (e.g., acute blood\\u000a loss after trauma, chronic blood loss in menstruating women, inadequate nutrition, chronic autoimmune conditions, chronic\\u000a infections, and many other disorders). An anemia

Reinhold Munker

130

Hb Youngstown [?101(G3)Glu???Ala; HBB: c.305A > C]: An unstable hemoglobin variant causing severe hemolytic anemia.  

PubMed

Hb Youngstown is a rare hemoglobin (Hb) variant caused by substitution of glutamic acid with alanine at amino acid residue 101 of the ?-globin chain as a result of an A > C transversion on the ?-globin gene nucleotide sequences [?101(G3)Glu???Ala; HBB: c.305A > C]. We now report three patients from two different families, one from South Africa and the other from Costa Rica, who are heterozygous for this Hb variant. All three carriers had marked hemolysis, consistent with Hb Youngstown being a highly unstable variant. The substitution of glutamic acid, a large and negatively charged amino acid, with alanine, a small and non polar amino acid, in the interface of the ?1- and ?2-globin subunits might interfere with the transition between the oxy- and deoxyHb, and lead to Hb instability and hemolytic anemia. PMID:25347256

Edward, Heather L; Pisani, Louis Almero Du; Rodriguez-Romero, Walter E; Chaves-Villalobos, Jorge; Garcia-Quesada, Jonielle; Harris, Neil S; Luo, Hong-Yuan; Steinberg, Martin H; Forget, Bernard G; Chui, David H K

2014-01-01

131

Graves' disease causing pancytopenia and autoimmune hemolytic anemia at different time intervals: a case report and a review of the literature.  

PubMed

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

Naji, Peyman; Kumar, Geetika; Dewani, Shabana; Diedrich, William A; Gupta, Ankur

2013-01-01

132

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 ...

133

Zinc-induced hemolytic anemia in a dog caused by ingestion of a game-playing die  

PubMed Central

A 16-month-old spayed female mixed breed dog was presented with a 1-week history of anorexia, lethargy, diarrhea, vomiting, and difficulty rising. Hematologic evaluation indicated a marked macrocytic hypo-chromic, markedly regenerative anemia. A metallic foreign object in the gastrointestinal tract was identified on abdominal radiographs. Serum zinc concentration was markedly increased. PMID:23024383

Clancey, Noel P.; Murphy, Megan C.

2012-01-01

134

Anemia  

MedlinePLUS

... 8, 2015, Orlando, FL CME/Certificate of Attendance Satellite Symposia programs View all meetings 2015 Highlights of ... Americans. Jump To: The Role of Red Blood Cells in Anemia Red blood cells carry hemoglobin, an ...

135

A novel mutation in the glucose-6-phosphate dehydrogenase gene in a subject with chronic nonspherocytic hemolytic anemia梒haracterization of enzyme using yeast expression system and molecular modeling  

Microsoft Academic Search

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzymopathy. Human G6PD gene is highly polymorphic, with over 130 mutations identified, many of which cause hemolytic anemia. We studied a novel point mutation in the G6PD gene 1226 C?G, predicting the proline 409 to arginine substitution (G6PD Suwalki). We expressed the human wild-type and mutated G6PD gene in yeast Saccharomyces

Dorota Grabowska; Ewa Jablonska-Skwiecinska; Danuta Plochocka; Anna Chelstowska; Irmina Lewandowska; Iwona Witos; Zofia Majewska; Roma Rokicka-Milewska; Beata Burzynska

2004-01-01

136

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

Microsoft Academic Search

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

1982-01-01

137

Molecular basis of inherited microcytic anemia due to defects in iron acquisition or heme synthesis  

PubMed Central

Microcytic anemia is the most commonly encountered anemia in general medical practice. Nutritional iron deficiency and ? thalassemia trait are the primary causes in pediatrics, whereas bleeding disorders and anemia of chronic disease are common in adulthood. Microcytic hypochromic anemia can result from a defect in globin genes, in heme synthesis, in iron availability or in iron acquisition by the erythroid precursors. These microcytic anemia can be sideroblastic or not, a trait which reflects the implications of different gene abnormalities. Iron is a trace element that may act as a redox component and therefore is integral to vital biological processes that require the transfer of electrons as in oxygen transport, oxidative phosphorylation, DNA biosynthesis and xenobiotic metabolism. However, it can also be pro-oxidant and to avoid its toxicity, iron metabolism is strictly controlled and failure of these control systems could induce iron overload or iron deficient anemia. During the past few years, several new discoveries mostly arising from human patients or mouse models have highlighted the implication of iron metabolism components in hereditary microcytic anemia, from intestinal absorption to its final inclusion into heme. In this paper we will review the new information available on the iron acquisition pathway by developing erythrocytes and its regulation, and we will consider only inherited microcytosis due to heme synthesis or to iron metabolism defects. This information could be useful in the diagnosis and classification of these microcytic anemias. PMID:19181781

Iolascon, Achille; De Falco, Luigia; Beaumont, Carole

2009-01-01

138

Reduced glutathione accelerates the oxidative damage produced by sodium n-propylthiosulfate, one of the causative agents of onion-induced hemolytic anemia in dogs.  

PubMed

The oxidative effects of sodium n-propylthiosulfate, one of the causative agents of onion-induced hemolytic anemia in dogs, were investigated in vitro using three types of canine erythrocytes, which are differentiated by the concentration of reduced glutathione and the composition of intracellular cations. After incubation with sodium n-propylthiosulfate, the methemoglobin concentration and Heinz body count in all three types of erythrocytes increased and a decrease in the erythrocyte reduced glutathione concentration was then observed. The erythrocytes containing high concentrations of potassium and reduced glutathione (approximately five times the normal values) were more susceptible to oxidative damage by sodium n-propylthiosulfate than were the normal canine erythrocytes. The susceptibility of the erythrocytes containing high potassium and normal reduced glutathione concentrations was intermediate between those of erythrocytes containing high concentrations of potassium and reduced glutathione and normal canine erythrocytes. In addition, the depletion of erythrocyte reduced glutathione by 1-chloro-2, 4-dinitrobenzene resulted in a marked decrease in the oxidative injury induced by sodium n-propylthiosulfate in erythrocytes containing high concentrations of potassium and reduced glutathione. The generation of superoxide in erythrocytes containing high concentrations of potassium and reduced glutathione was 4.1 times higher than that in normal canine erythrocytes when the cells were incubated with sodium n-propylthiosulfate. These observations indicate that erythrocyte reduced glutathione, which is known as an antioxidant, accelerates the oxidative damage produced by sodium n-propylthiosulfate. PMID:10216234

Yamato, O; Hayashi, M; Kasai, E; Tajima, M; Yamasaki, M; Maede, Y

1999-04-19

139

Aplastic anemia and red cell aplasia due to pentachlorophenol  

SciTech Connect

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.

Roberts, H.J.

1983-01-01

140

Hemolytic activity of Fusobacterium necrophorum culture supernatants due to presence of phospholipase A and lysophospholipase.  

PubMed

Culture supernatants of Fusobacterium necrophorum demonstrated hemolytic activity. The hemolysin(s), which was partially purified by ammonium sulfate precipitation, was temperature-dependent and heat labile. The spectrum of hemolytic activity against various erythrocytes included rabbit, human, and dog erythrocytes. Goats, sheep, and bovine erythrocytes showed only trace hemolysis. According to results of thin-layer chromatography, the hemolysin hydrolyzed rabbit erythrocyte phosphatidyl choline, phosphatidyl ethanolamine, lysophosphatidyl choline, and bovine phosphatidyl choline. Hydrolysis of egg yolk phosphatidyl choline, bovine phosphatidyl ethanolamine, cholesterol, 1,2-dipalmitin, 1,3-dipalmitin, sphingomyelin, or triolein was not detected by thin layer chromatography. A more sensitive procedure utilizing gas-liquid chromatography revealed that, of the substrates tested, the following were bein hydrolyzed: bovine and egg yolk phosphatidyl choline, lysophosphatidyl choline, alpha-palmito-beta-eleoyl-L-alpha lecithin and alpha-oleoyl-betal-palmitoyl-L-alpha lecithin. Substrates which were weakly hydrolyzed were bovine phosphatidyl ethanolamine, DL-alpha-hosphatidyl ethanolamine dipalmitoyl, 1,2-dipalmitin, 1,3-dipalmitin, and triolein. PMID:453690

Abe, P M; Kendall, C J; Stauffer, L R; Holland, J W

1979-01-01

141

Atypical Hemolytic Uremic Syndrome  

PubMed Central

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

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

2013-01-01

142

Donor deferral due to anemia: A tertiary care center-based study  

PubMed Central

Background: The minimum hemoglobin cutoff for blood donation in India is 12.5 gm% for both male and female donors and the minimum donation interval is 3 months. Donation of one unit of blood results in decrease in hemoglobin by 1 gm% and loss of 200250 mg of iron. Donor deferral due to anemia is one of the major reasons of temporary rejection of blood donors. In the absence of further workup or advise, it results in loss of valuable donor base. Aim and Objective: To provide baseline information regarding the prevalence and spectrum of anemia in prospective blood donors to help plan a future strategy for donor management. Materials and Methods: Hemoglobin testing of donors was performed using Hemocue and Copper sulfate specific gravity method. Ethylene diamine tetraacetic acid sample of all the donors who failed either or both the screening tests was tested on automated analyzer for evaluation of hemoglobin and red blood cell indices. Results: Of all the donors, 15.5% were deferred due to anemia. Prevalence of anemia in prospective blood donors was 1.8%. It was significantly higher in female donors compared with male donors (34.2% vs 1.2%). The most common type of anemia was normocytic normochromic. PMID:21572718

Bahadur, Shalini; Pujani, Meenu; Jain, Manjula

2011-01-01

143

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

E-print Network

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

Bandettini, Peter A.

144

Hemolytic uremic syndrome associated with Clostridium difficile infection.  

PubMed

We report 3 cases of Clostridium difficile-associated hemolytic uremic syndrome (HUS) with biopsy proven renal thrombotic microangiopathy. Two patients with acute renal failure were kidney transplants recipients whereas the third patient developed renal failure in the native kidneys. The presentation was preceded by acute diarrhea and stool. Clostridium difficile toxin was detected in all the 3 patients. Stool studies were negative for Escherichia coli, Shigella dysenteriae and other enteric pathogens. The diagnosis of Clostridium difficile-associated hemolytic uremic syndrome was suspected due to presence of thrombocytopenia, microangiopathic hemolytic anemia and biopsy proven renal thrombotic microangiopathy without another clinically apparent cause. This case series suggest that Clostridium difficile infection may cause renal failure due to thrombotic microangiopathy (TMA) and should be considered in the differential diagnosis of diarrhea-associated HUS. PMID:23320969

Alvarado, Anthony S; Brodsky, Sergey V; Nadasdy, Tibor; Singh, Neeraj

2014-04-01

145

Genetics Home Reference: Atypical hemolytic-uremic syndrome  

MedlinePLUS

... Hemolytic anemia occurs when red blood cells break down (undergo hemolysis) prematurely. In atypical hemolytic-uremic syndrome, red blood cells can break apart as they squeeze past clots within small blood vessels. Anemia results if these cells are destroyed faster ...

146

Atypical hemolytic uremic syndrome  

PubMed Central

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

2011-01-01

147

Laboratory Evaluation of Anemia  

PubMed Central

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

Wallerstein, Ralph O.

1987-01-01

148

Folate-deficiency anemia  

MedlinePLUS

Folate-deficiency anemia is a decrease in red blood cells ( anemia ) due to a lack of folate. Folate is a type ... B vitamin. It is also called folic acid. Anemia is a condition in which the body does ...

149

[Anemias due to disorder of folate, vitamin B12 and transcobalamin metabolism].  

PubMed

Macrocytic megaloblastic anemia is the most typical but the latest sign of a cobalamin (vitamin B12) and/or folic acid deficiency or of a congenital abnormality of cobalamin and folate metabolism. Macrocytosis in blood and megaloblastosis in bone marrow are the morphological features of a disturbance in cell division related to a defect in DNA biosynthesis. Macrocytosis without anemia, normocytic normochronic anemia with a low reticulocyte cell count or microcytic hypochromic anemia in case of associated iron deficiency do not exclude a vitamin deficiency. Neurological or psychiatric disorders and immune abnormalities have been reported in patients with vitamin B12 or folate deficiencies or in children with congenital abnormalities of these 2 vitamins; such manifestations may even occur without anemia. PMID:8235383

Zittoun, J

1993-06-01

150

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

SciTech Connect

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.

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

1988-09-01

151

[A case of hemolytic uremic syndrome after adjuvant chemotherapy with gemcitabine in a patient with pancreatic cancer].  

PubMed

A 63-year-old man with Stage IVa pancreas tail cancer was admitted for a distal pancreatectomy and splenectomy; adjuvant chemotherapy with gemcitabine was also administered. The chemotherapy was terminated after 16 courses due to hemolytic anemia, thrombocytopenia and renal dysfunction. Plasma exchange was performed; however the patient's renal function was diminished, requiring chronic hemodialysis. Physicians should be cautious of hemolytic uremic syndrome as a possible adverse reaction to gemcitabine and be aware that tests are needed for its early detection. PMID:20938119

Wato, Masaki; Inaba, Tomoki; Ishikawa, Hisashi; Ishikawa, Shigenao; Baba, Nobuyuki; Miyoshi, Masatsugu; Senoh, Tomonori; Nagano, Takuya; Takaguchi, Koichi; Watanabe, Seishiro; Kawai, Kozo

2010-10-01

152

Anemia Causes Hypoglycemia in ICU Patients Due to Error in Single-Channel Glucometers: Methods of Reducing Patient Risk  

PubMed Central

OBJECTIVE Intensive insulin therapy (IIT) in the critically ill reduces mortality but carries the risk of increased hypoglycemia. Point-of-care (POC) blood glucose analysis is standard; however anemia causes falsely high values and potentially masks hypoglycemia. Permissive anemia is routinely practiced in most intensive care units (ICUs). We hypothesized that POC glucometer error due to anemia is prevalent, can be mathematically corrected, and correction uncovers occult hypoglycemia during IIT. DESIGN The study has both retrospective and prospective phases. We reviewed data to verify the presence of systematic error, determine the source of error, and establish the prevalence of anemia. We confirmed our findings by reproducing the error in an in-vitro model. Prospective data was used to develop a correction formula validated by the Monte Carlo method. Correction was implemented in a burn ICU and results evaluated after nine months. SETTING Burn and trauma ICUs at a single research institution. PATIENTS/SUBJECTS Samples for in-vitro studies were taken from healthy volunteers. Samples for formula development were from critically ill patients on IIT. INTERVENTIONS Insulin doses were calculated based on predicted serum glucose values from corrected POC glucometer measurements. MEASUREMENTS Time-matched POC glucose, laboratory glucose, and hematocrit values. MAIN RESULTS We previously found that anemia (HCT<34%) produces systematic error in glucometer measurements. The error was correctible with a mathematical formula developed and validated using prospectively collected data. Error of uncorrected POC glucose ranged from 19% to 29% (p<0.001), improving to ?5% after mathematical correction of prospective data. Comparison of data pairs before and after correction formula implementation demonstrated a 78% decrease in the incidence of hypoglycemia in critically ill and anemic patients treated with insulin and tight glucose control (p<0.001). CONCLUSIONS A mathematical formula that corrects erroneous POC glucose values due to anemia in ICU patients reduces the incidence of hypoglycemia during IIT. PMID:19789438

Pidcoke, Heather F.; Wade, Charles E.; Mann, Elizabeth A.; Salinas, Jose; Cohee, Brian M.; Holcomb, John B.; Wolf, Steven E.

2014-01-01

153

Severe hemolytic disease of the newborn due to anti-Vw and detection of glycophorin A antigens on the Miltenberger I sialoglycoprotein by Western blotting.  

PubMed

Anti-Vw detecting an antigen on Miltenberger I (Mi I) variant glycophorin A (GPA) has rarely been reported as a cause of hemolytic disease of the newborn (HDN). We report an infant with severe HDN due to anti-Vw. Examination of the Vw+ erythrocytes of the father and paternal grandmother by sodium dodecylsulphate polyacrylamide gel electrophoresis showed an extra trypsin-sensitive, periodic-acid-Schiff staining band, consistent with Mi I variant GPA. Staining of Western blots by monoclonal antibodies showed that normal paternal GPA expressed blood group M, while Mi I variant GPA expressed blood group N. Mi I variant GPA expressed the trypsin-sensitive antigenic determinant detected by MoAb 10F7, indicating that the alterations known to occur in the trypsin-sensitive fragment of Mi I variant GPA do not affect expression of the antigen detected by 10F7. PMID:2442890

Rearden, A; Frandson, S; Carry, J B

1987-01-01

154

Inborn anemias in mice  

SciTech Connect

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.

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

1981-06-01

155

Iron deficiency anemia due to lymphocytic gastritis with Helicobacter pylori infection in childhood: case report.  

PubMed

Lymphocytic gastritis (LG) is a chronic inflammatory process of poorly understood pathogenesis. We report the case of a 12-year-old premenstrual girl with refractory iron deficiency anemia in which the oral iron absorption challenge suggested iron malabsorption. Laboratory studies ruled out celiac disease and autoimmune gastritis, and carbon-13 urea breath test for Helicobacter pylori was also negative. Upper endoscopy with gastric body and antral mucosa biopsies revealed a LG with focal intestinal metaplasia and H. pylori infection. H. pylori eradication was undertaken with success and 3 months later her hematologic parameters normalized. Histologic reevaluation showed disappearance of LG. This case shows that investigation of malabsorption disease in the presence of refractory iron deficiency anemia can lead to the diagnosis of important gastric diseases, even in the absence of gastrointestinal symptoms. This nonceliac child was diagnosed with a severe histopathologic pattern of LG, with potential risk of malignant transformation, which was completely reverted with adequate H. pylori eradication treatment. PMID:23528908

Santalha, Marta F F; Costa, Em韑ia; Miguel, Natalina; Vizca韓o, Ram髇; Barbot, Jos; Pereira, Fernando

2013-05-01

156

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

SciTech Connect

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.

Bernstein, S.E.

1982-07-19

157

Laparoscopic-assisted partial ileectomy for crohn's disease associated with chronic anemia due to frequent hemorrhage.  

PubMed

Crohn's disease can involve any part of the gastrointestinal tract. Although good conservative treatment is given as soon as possible, most patients with this disease will eventually require surgery. We encountered a case of Crohn's disease associated with anemia which we treated with laparoscopic-assisted ileectomy. The postoperative course was satisfactory. The most important characteristic of Crohn's disease, fat wrapping and extending over the serosal surface toward the antimesenteric border, was observed in the ileum, distinguishing the disease and pinpointing the lesion accurately. This surgical method has an advantage over open surgery in that the recovery time is shorter and incisions are smaller, allowing easier surgery in the future, shortening the patient's hospital stay, and improving the patient's quality of life. PMID:15966212

Hirayama, Isao; Ide, Munenori; Shoji, Hisanori; Nakamura, Jun-Ichi; Fujita, Kin-Ichi; Iizuka, Haruhisa; Oshimoto, Koichi; Hisada, Takeshi; Sato, Tetsuro; Mori, Masatomo; Asao, Takayuki; Kuwano, Hiroyuki

2005-01-01

158

Optimal management of iron deficiency anemia due to poor dietary intake  

PubMed Central

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抯 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

Aspuru, Kattalin; Villa, Carlos; Bermejo, Fernando; Herrero, Pilar; L髉ez, Santiago Garc韆

2011-01-01

159

Aplastic Anemia  

MedlinePLUS

... from the NHLBI on Twitter. What Is Aplastic Anemia? Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is ... heart, heart failure , infections, and bleeding. Severe aplastic anemia can even cause death. Overview Aplastic anemia is ...

160

Chronic anemia due to mitomycin C is drug dose-dependent, normocytic, progressive, related to erythropoietin levels and quantitatively predictable: implications for radiochemotherapy.  

PubMed

Mitomycin C (MC) is used as therapy against solid tumors, also combined with other chemotherapeutic agents or radiotherapy. It may cause acute, subacute, or chronic anemia capable of modifying the results of chemo- and radiotherapy. Erythropoietin may be lowered by cancer itself or because of chemoradiotherapy. There are few studies investigating the relationship between erythropoietin and chronic anemia.We prospectively analyzed the chronic anemia and erythropoietin in 38 patients with solid cancer. Patients were 40 to 82 years of age. MC was randomly given every 3 weeks as a single drug at 10 or 20 mg/m. When myelotoxicity occurred the next therapy cycle was delayed until recovery. RBC indices, hemolysis, erythropoietin, liver and kidney function were studied. MC cycles were 136 (3.6 1.4 per pt), 32 being delayed because of myelotoxicity.Hematocrit, hemoglobin and RBC were inversely related to the cumulative dose (r = 0.70 to 0.86; p 0.03 to 0.01) of MC. Other tests remained stable. Anemia occurred almost twofold earlier in the 20 mg/m group (p=0.049). basal erythropoietin, already lower than in age and sex watched 81 non cancerous subjects (p<0.001), decreased during MC therapy (p<0.01). For each given MC mg/m a 0.0372 Hb mg/dl reduction occurred. Chronic anemia due to MC is accompanied by erythropoietin reduction. These results can help in designing chemoradiotherapy. PMID:22233822

Cartei, G; Colombrino, E; Sanzari, M C; Plebani, M; Micucci, M; Fiorica, F; Giraldi, T; Zustovich, F; Cartei, F

2011-12-01

161

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

Microsoft Academic Search

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

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

2004-01-01

162

DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome  

Microsoft Academic Search

DEAP-HUS [Deficiency of CFHR (complement factor H-related) plasma proteins and Autoantibody Positive form of Hemolytic Uremic Syndrome] represents a novel subtype of hemolytic uremic syndrome (HUS) with unique characteristics. It affects children and\\u000a requires special clinical attention in terms of diagnosis and therapy. DEAP-HUS and other atypical forms of HUS share common\\u000a features, such as microangiopathic hemolytic anemia, acute renal

Peter F. Zipfel; Christoph Mache; Dominik M黮ler; Christoph Licht; Marianne Wigger; Christine Skerka

2010-01-01

163

Natural history of premacular hemorrhage due to severe acute anemia: clinical and anatomical features in two untreated patients.  

PubMed

Premacular retrohyaloid hemorrhage is a rare complication of acute severe anemia. The authors report two cases of premacular hemorrhage in which no treatment other than clinical and spectral-domain optical coherence tomography observation was performed. The natural history of this condition reveals that complete clinical resolution is not accompanied by full anatomical restoration. [Ophthalmic Surg Lasers Imaging Retina. 2014;45:E5-E7.]. PMID:24496165

Turco, Claudia Del; La Spina, Carlo; Mantovani, Elena; Gagliardi, Marco; Lattanzio, Rosangela; Pierro, Luisa

2014-01-01

164

Hemolytic uremic syndrome complicated with IgA nephropathy: a case report and literature review.  

PubMed

A previously healthy young female, presenting with nausea, vomiting, diarrhea, anemia, thrombocytopenia, and acute renal failure, was admitted to our hospital. Her clinical and histological features were consistent with both hemolytic uremic syndrome and IgA nephropathy, and she responded to steroid treatment, plasma transfusion, and gamma globulin therapy and did not need hemodialysis. In the following months, she achieved clinical remission except for low complement C3. Since hemolytic uremic syndrome is rarely associated with IgA nephropathy, we present this case and discuss potential connection between hemolytic uremic syndrome and IgA nephropathy. PMID:24290408

Wang, Rong; Zhang, Yiyan; Li, Shijun; Chen, Hao; Zeng, Caihong; Chen, Huiping; Tang, Zheng; Liu, Zhihong

2015-01-01

165

Aplastic Anemia  

MedlinePLUS

Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... cause is unknown. Your doctor will diagnose aplastic anemia based on your medical and family histories, a ...

166

Aplastic anemia  

MedlinePLUS

Aplastic anemia is a condition in which the bone marrow does not make enough new blood cells. Bone marrow ... Aplastic anemia results from injury to the blood stem cells. These are immature cells in the bone marrow that ...

167

How to approach chronic anemia.  

PubMed

We present herein an approach to diagnosing the cause of chronic anemia based on a patient's history and complete blood cell count (CBC). Four patterns that are encountered frequently in CBCs associated with chronic anemias are considered: (1) anemia with abnormal platelet and/or leukocyte counts, (2) anemia with increased reticulocyte counts, (3) life-long history of chronic anemia, and (4) anemia with inappropriately low reticulocytes. The pathophysiologic bases for some chronic anemias with low reticulocyte production are reviewed in terms of the bone marrow (BM) events that reduce normal rates of erythropoiesis. These events include: apoptosis of erythroid progenitor and precursor cells by intrinsic and extrinsic factors, development of macrocytosis when erythroblast DNA replication is impaired, and development of microcytosis due to heme-regulated eIF2? kinase inhibition of protein synthesis in iron-deficient or thalassemic erythroblasts. PMID:23233579

Koury, Mark J; Rhodes, Melissa

2012-01-01

168

An In vivo Drug Screening Model Using Glucose-6-Phosphate Dehydrogenase Deficient Mice to Predict the Hemolytic Toxicity of 8-Aminoquinolines  

PubMed Central

Anti-malarial 8-aminoquinolines drugs cause acute hemolytic anemia in individuals with glucose-6-phosphate dehydrogenase deficiency (G6PDD). Efforts to develop non-hemolytic 8-aminoquinolines have been severely limited caused by the lack of a predictive in vivo animal model of hemolytic potential that would allow screening of candidate compounds. This report describes a G6PDD mouse model with a phenotype closely resembling the G6PDD phenotype found in the African A-type G6PDD human. These G6PDD mice, given different doses of primaquine, which used as a reference hemolytic drug, display a full array of hemolytic anemia parameters, consistently and reproducibly. The hemolytic and therapeutic indexes were generated for evaluation of hemotoxicity of drugs. This model demonstrated a complete hemolytic toxicity response to another known hemolytic antimalarial drug, pamaquine, but no response to non-hemolytic drugs, chloroquine and mefloquine. These results suggest that this model is suitable for evaluation of selected 8-AQ type candidate antimalarial drugs for their hemolytic potential. PMID:23530079

Zhang, Peng; Gao, Xiugong; Ishida, Hiroshi; Amnuaysirikul, Jack; Weina, Peter J.; Grogl, Max; O'Neil, Michael T.; Li, Qigui; Caridha, Diana; Ohrt, Colin; Hickman, Mark; Magill, Alan J.; Ray, Prabhati

2013-01-01

169

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

PubMed Central

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

Kuo, Elizabeth

2014-01-01

170

Delayed hemolytic transfusion reaction in children with sickle cell disease  

PubMed Central

Background Transfusion is a cornerstone of the management of sickle cell disease but carries a high risk of hemolytic transfusion reaction, probably because of differences in erythrocyte antigens between blood donors of European descent and patients of African descent. Patients may experience hemolytic transfusion reactions that are delayed by from a few days to two weeks and manifest as acute hemolysis (hemoglobinuria, jaundice, and pallor), symptoms suggesting severe vaso-occlusive crisis (pain, fever, and acute chest syndrome), and profound anemia, often with reticulocytopenia. This case-series study aims to describe the main characteristics of this syndrome, to discuss its pathophysiology, and to propose a management strategy. Design and Methods We identified 8 pediatric cases of delayed hemolytic transfusion reactions between 2006 and 2009 in the database of the Necker Hospital, France. All patients had received cross-matched red cell units compatible in the ABO, RH, and KEL systems. We reviewed the medical charts in the computerized blood transfusion databases. All patients were admitted to the intensive care unit. We progressively adopted the following strategy: intravenous immunoglobulins, and darbopoietin alpha when the reticulocyte count was below 150109/L, without further blood transfusion during the acute episode unless absolutely necessary. Results The median time between the transfusion and the diagnosis of delayed hemolytic transfusion reaction was six days. All patients had severe bone pain; all but one had a high-grade fever. Five patients had hemoglobin levels less than than 4g/dL and 3 had reticulocytopenia. In 5 patients, no new antibody was found; one patient had weakly reactive antibodies. Only 2 patients had new allo-antibodies possibly responsible for the delayed hemolytic reaction. Conclusions The initial symptoms of delayed hemolytic transfusion reaction were complex and mimicked other complications of sickle cell disease. In most of our cases, no new antibody was identified, which underlines the complexity of the pathophysiology of this syndrome. PMID:21330322

de Montalembert, Mariane; Dumont, Marie-Dominique; Heilbronner, Claire; Brousse, Valentine; Charrara, Oussama; Pellegrino, B閍trice; Piguet, Christophe; Soussan, Val閞ie; Noizat-Pirenne, France

2011-01-01

171

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

PubMed Central

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

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

172

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

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

P閞ez-Oliva, Jorge F; Casanova-Gonz醠ez, Martha; Garc韆-Garc韆, Idrian; Porrero-Mart韓, Pedro J; Valenzuela-Silva, Carmen M; Hern醤dez-Montero, Tair; Lagarde-Ampudia, Marcia; Casanova-Kutsareva, Yuri; 羦ila-Albuerne, Yisel; Vargas-Batista, Alicia; Bobillo-L髉ez, Hailen; Herrera-Vald閟, Ra鷏; L髉ez-Saura, Pedro A

2005-01-01

173

Management of hemolytic uremic syndrome  

PubMed Central

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.

Kavanagh, David; Raman, Shreya

2014-01-01

174

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

PubMed

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

Gelbart, David

2014-04-01

175

Hemolytic Anemia in Wild Seaducks Caused by Marine Oil Pollution  

Microsoft Academic Search

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

Osamu Yamato; Yoshimitsu Macdc

176

Genetics Home Reference: Anemia  

MedlinePLUS

... Home Conditions Genes Chromosomes Handbook Glossary Resources Conditions > Anemia Related topics on Genetics Home Reference: acute promyelocytic ... syndrome beta thalassemia Coats plus syndrome congenital dyserythropoietic anemia Diamond-Blackfan anemia Fanconi anemia Ghosal hematodiaphyseal dysplasia ...

177

Anemia and Fatigue  

MedlinePLUS

Anemia And Fatigue Anemia And Fatigue htmAnemiaAndFatigue Left untreated, anemia can lead to a lack of energy and, more seriously, strokes, heart attacks ... InteliHealth Medical Content 2014-12-08 What Is Anemia? Anemia means that your hemoglobin level is below ...

178

Hemolytic Uremic Syndrome: Toxins, Vessels, and Inflammation  

PubMed Central

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

Cheung, Victoria; Trachtman, Howard

2014-01-01

179

Anemia of chronic disease  

MedlinePLUS

Anemia of inflammation; AOCD; ACD ... Anemia is a lower-than-normal number of red blood cells in the blood. Some conditions can lead to anemia of chronic disease include: Autoimmune disorders , such as ...

180

Living with Anemia  

MedlinePLUS

... page from the NHLBI on Twitter. Living With Anemia Often, you can treat and control anemia. If ... by an inherited or chronic disease or trauma. Anemia and Children/Teens Infants and young children have ...

181

Sickle Cell Anemia  

MedlinePLUS

... have the disease itself. What Is Sickle Cell Anemia? Sickle cell anemia is a blood disorder that affects hemoglobin (pronounced: ... helps carry oxygen throughout the body. Sickle cell anemia occurs when a person inherits two abnormal genes ( ...

182

How Is Anemia Treated?  

MedlinePLUS

... page from the NHLBI on Twitter. How Is Anemia Treated? Treatment for anemia depends on the type, cause, and severity of ... is to treat the underlying cause of the anemia. Dietary Changes and Supplements Low levels of vitamins ...

183

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. ...

184

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 ...

185

SIGNIFICANCE OF THE HEMOSIDEROSIS OF PERNICIOUS ANEMIA  

PubMed Central

The selective deposition of hemosiderin in the liver parenchyma during pernicious anemia does not constitute evidence that there is a hemolytic cause for the disease located in the portal region. The repeated introduction of small amounts of free hemoglobin into the general circulation, by the subcutaneous route, leads, as we have shown, to an identical siderosis. Larger amounts of hemoglobin cause a renal pigmentation equalling or exceeding the hepatic, a fact that is in keeping with what is known of the physiology of hemoglobin excretion and of the findings in human beings afteroutspoken hemolysis. PMID:19868626

McMaster, Philip D.; Rous, Peyton; Larimore, Louise C.

1922-01-01

186

Chickens treated with a nitric oxide inhibitor became more resistant to Plasmodium gallinaceum infection due to reduced anemia, thrombocytopenia and inflammation  

PubMed Central

Malaria is a serious infectious disease caused by parasites of the Plasmodium genus that affect different vertebrate hosts. Severe malaria leads to host death and involves different pathophysiological phenomena such as anemia, thrombocytopenia and inflammation. Nitric oxide (NO) is an important effector molecule in this disease, but little is known about its role in avian malaria models. Plasmodium gallinaceum- infected chickens were treated with aminoguanidine (AG), an inhibitor of inducible nitric oxide synthase, to observe the role of NO in the pathogenesis of this avian model. AG increased the survival of chickens, but also induced higher parasitemia. Treated chickens demonstrated reduced anemia and thrombocytopenia. Moreover, erythrocytes at different stages of maturation, heterophils, monocytes and thrombocytes were infected by Plasmodium gallinaceum and animals presented a generalized leucopenia. Activated leukocytes and thrombocytes with elongated double nuclei were observed in chickens with higher parasitemia; however, eosinophils were not involved in the infection. AG reduced levels of hemozoin in the spleen and liver, indicating lower inflammation. Taken together, the results suggest that AG reduced anemia, thrombocytopenia and inflammation, explaining the greater survival rate of the treated chickens. PMID:23398940

2013-01-01

187

Comparative effects of mono-, di-, tri-, and tetrasulfides derived from plants of the Allium family: redox cycling in vitro and hemolytic activity and Phase 2 enzyme induction in vivo.  

PubMed

Epidemiological evidence indicates that a high dietary intake of plants of the Allium family, such as garlic and onions, decreases the risk of cancer in humans. It has been suggested that this effect is due to the ability of the aliphatic mono-, di-, tri-, and tetrasulfides derived from these vegetables to increase tissue activities of Phase 2 detoxification enzymes. In contrast, toxic effects have been recorded in domestic and farm animals after the consumption of garlic or onions, involving oxidative damage to erythrocytes and consequent hemolytic anemia. This effect again has been attributed to the aliphatic sulfides. In the present study, the ability of sulfides derived from garlic and onions to generate "active oxygen" species and cause oxidative damage to erythrocytes in vitro has been compared, together with their ability to cause hemolytic anemia and increase the activity of the Phase 2 enzymes quinone reductase (QR) and glutathione S-transferase (GST) in rats. Monosulfides were without significant effect on any parameter. Di-, tri-, and tetrasulfides generated hydrogen peroxide in the presence of GSH and hemoglobin and caused oxidative damage to erythrocytes in vitro. The activity decreased in the order of tetra- > tri- > disulfide, with the allyl compounds being more potent than the propyl. In vivo, both allyl and propyl tri- and tetrasulfides were powerful hemolytic agents. In contrast, only the allyl sulfides increased the activities of QR and GST; the propyl derivatives were completely without effect. Allyl and propyl tri- and tetrasulfides, thus, may contribute to the toxic effects of Allium vegetables, while only the allyl derivatives are effective in increasing tissue activities of cancer-protective enzymes. PMID:12706500

Munday, Rex; Munday, John S; Munday, Christine M

2003-05-01

188

Sexuality and sickle cell anemia  

PubMed Central

Background Sickle cell disease, the most common hereditary blood disease in the world, is the result of an atypical hemoglobin called S (Hb S) which, when homozygous (Hb SS) is the cause of sickle cell anemia. Changes of puberty, correlated with a delayed growth spurt, begin late in both male and female sickle cell anemia individuals with repercussions on sexuality and reproduction. The objectives of this exploratory and descriptive study were to characterize the development of sexuality in adults with sickle cell anemia by investigating the patient's perception of their sex life, as well as the information they had and needed on this subject. Methods Twenty male and female sickle cell anemia patients treated at the Hemocentro Regional de Uberaba (UFTM) with ages between 19 and 47 years old were enrolled. A socioeconomic questionnaire and a semi-structured interview on sexuality, reproduction and genetic counseling were applied. Results This study shows that the sickle cell anemia patients lacked information on sexuality especially about the risks of pregnancy and the possible inheritance of the disease by their children. Moreover, the sexual life of the patients was impaired due to pain as well as discrimination and negative feelings experienced in close relationships. Conclusion The health care of sickle cell anemia patients should take into account not only the clinical aspects of the disease, but also psychosocial aspects by providing counseling on sexuality, reproduction and genetics, in order to give this population the possibility of a better quality of life. PMID:23741184

C鬮o, Viviane de Almeida; Chapadeiro, Cibele Alves; Ribeiro, Jo鉶 Batista; Moraes-Souza, Helio; Martins, Paulo Roberto Juliano

2013-01-01

189

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

PubMed Central

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

Tyner, Harmony L; Schlievert, M; Baddour

2011-01-01

190

Nitric Oxide and Arginine Dysregulation: A Novel Pathway to Pulmonary Hypertension in Hemolytic Disorders  

PubMed Central

Secondary pulmonary hypertension (PH) is emerging as one of the leading causes of mortality and morbidity in patients with hemolytic anemias such as sickle cell disease (SCD) and thalassemia. Impaired nitric oxide (NO) bioavailability represents the central feature of endothelial dysfunction, and is a major factor in the pathophysiology of PH. Inactivation of NO correlates with hemolytic rate and is associated with the erythrocyte release of cell-free hemoglobin, which consumes NO directly, and the simultaneous release of the arginine-metabolizing enzyme arginase, which limits bioavailability of the NO synthase substrate arginine during the process of intravascular hemolysis. Rapid consumption of NO is accelerated by oxygen radicals that exists in both SCD and thalassemia. A dysregulation of arginine metabolism contributes to endothelial dysfunction and PH in SCD, and is strongly associated with prospective patient mortality. The central mechanism responsible for this metabolic disorder is enhanced arginine turnover, occurring secondary to enhanced plasma arginase activity. This is consistent with a growing appreciation of the role of excessive arginase activity in human diseases, including asthma and pulmonary arterial hypertension. New treatments aimed at improving arginine and NO bioavailability through arginase inhibition, suppression of hemolytic rate, oral arginine supplementation, or use of NO donors represent potential therapeutic strategies for this common pulmonary complication of hemolytic disorders. PMID:18991648

Gladwin, Mark T.; Kato, Gregory J.

2011-01-01

191

Humanized mouse model of glucose 6-phosphate dehydrogenase deficiency for in vivo assessment of hemolytic toxicity  

PubMed Central

Individuals with glucose 6-phosphate dehydrogenase (G6PD) deficiency are at risk for the development of hemolytic anemia when given 8-aminoquinolines (8-AQs), an important class of antimalarial/antiinfective therapeutics. However, there is no suitable animal model that can predict the clinical hemolytic potential of drugs. We developed and validated a human (hu)RBC-SCID mouse model by giving nonobese diabetic/SCID mice daily transfusions of huRBCs from G6PD-deficient donors. Treatment of SCID mice engrafted with G6PD-deficient huRBCs with primaquine, an 8-AQ, resulted in a dose-dependent selective loss of huRBCs. To validate the specificity of this model, we tested known nonhemolytic antimalarial drugs: mefloquine, chloroquine, doxycycline, and pyrimethamine. No significant loss of G6PD-deficient huRBCs was observed. Treatment with drugs known to cause hemolytic toxicity (pamaquine, sitamaquine, tafenoquine, and dapsone) resulted in loss of G6PD-deficient huRBCs comparable to primaquine. This mouse model provides an important tool to test drugs for their potential to cause hemolytic toxicity in G6PD-deficient populations. PMID:24101478

Rochford, Rosemary; Ohrt, Colin; Baresel, Paul C.; Campo, Brice; Sampath, Aruna; Magill, Alan J.; Tekwani, Babu L.; Walker, Larry A.

2013-01-01

192

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

SciTech Connect

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.

Bernstein, S.E.

1983-09-09

193

Blood . Author manuscript Iron-deficiency anemia from matriptase-2 inactivation is dependent on the  

E-print Network

Blood . Author manuscript Page /1 6 Iron-deficiency anemia from matriptase-2 inactivation (characterized by increased hepcidin levels and anemia) and mice (exhibitingTmprss6 /- - Bmp6 /- - severe iron deficiency anemia are due to excess signaling through the Bmp6/Hjv pathway. MESH Keywords Anemia, Iron

Boyer, Edmond

194

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

PubMed

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

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

195

Imaging Diagnosis of Neonatal Anemia: Report of Two Unusual Etiologies  

PubMed Central

Anemia in neonatal period is rare, with the common causes being Rh and ABO blood group incompatibility, hemorrhagic disease of newborn, congenital hemolytic anemia, hemoglobinopathies, and TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes virus) infections. Congenital leukemia and infantile osteopetrosis (OP) are among the rare causes of neonatal anemia. A review of the literature shows approximately 200 reported cases of congenital leukemia. Articles describing the imaging features of congenital leukemia are still rarer. Infantile OP, another rare disorder with a reported incidence of 1 in 250,000 has characteristic imaging features, which are diagnostic of the disease. We report a case each, of two rare diseases: Congenital leukemia and infantile osteopetrosis. Additionally, our report highlights the radiological and imaging features of congenital leukemia and infantile OP and their crucial role in arriving at an early diagnosis. PMID:24605254

Grover, Shabnam Bhandari; Preethi, G Rajalakshmi; Saluja, Sumita; Bhargava, Ankit

2013-01-01

196

Living with Fanconi Anemia  

MedlinePLUS

... from the NHLBI on Twitter. Living With Fanconi Anemia Improvements in blood and marrow stem cell transplants ... Rate This Content: Next >> November 1, 2011 Fanconi Anemia Clinical Trials Clinical trials are research studies that ...

197

Sickle cell anemia  

MedlinePLUS

Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease ... Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells ...

198

The Anemias of Athletes.  

ERIC Educational Resources Information Center

Diagnosing anemia in athletes is complicated because athletes normally have a pseudoanemia that needs no treatment. Athletes, however, can develop anemia from iron deficiency or footstrike hemolysis, which require diagnosis and treatment. (Author/MT)

Eichner, Edward R.

1986-01-01

199

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 ...

200

Sickle Cell Anemia  

MedlinePLUS

... from the NHLBI on Twitter. What Is Sickle Cell Anemia? Espa駉l Sickle cell anemia (uh-NEE-me- ... raise the risk for infection. Normal Red Blood Cells and Sickle Cells Figure A shows normal red ...

201

Nitrite-induced anemia in channel catfish, Ictalurus punctatus Rafinesque  

SciTech Connect

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.

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

202

Lipid peroxidation in the hemolytic uremic syndrome.  

PubMed

Based on recent evidence of a genetic influence on prognosis (1) and the existence of red cell membrane phospholipid depletion with low or absent serum alpha-tocopherol (2) levels in three children with the Hemolytic Uremic Syndrome (H.U.S.), we wish to suggest the existence of an inborn error of antioxident capacity as the basic pathogenetic mechanism in the development of the hemolytic uremic syndrome (H.U.S.). PMID:713892

O'Regan, S; Fong, J S

1978-01-01

203

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

PubMed

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

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

2014-01-01

204

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

SciTech Connect

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.

Bernstein, S.E.

1981-07-02

205

IRON METABOLISM IN EXPERIMENTAL ANEMIA  

PubMed Central

In experimental anemia in dogs due to blood loss the term "available iron" as determined by the dipyridyl test has no physiological significance. Iron salts (100 per cent available by dipyridyl) given in optimum dose (560 mg. per 2 weeks) will cause a net production of 50 to 55 gm. hemoglobin above the control base line in anemic dogs. This means that an iron salt which is rated as 100 per cent available by the dipyridyl test is only 35 per cent physiologically available. The term "available iron (dipyridyl)" simmers down to iron not in the form of hematin compounds. The absorption of this "available iron" is conditioned by a great variety of factors, many unknown at this time. Iron is indeed an elusive sprite whose "availability" or comings and goings cannot be determined in dogs by dipyridyl梡erhaps only in part by studies of absorption and excretion. Liver contains "available iron (dipyridyl)" but also organic factors influencing hemoglobin regeneration in anemia as liver ash contains only about 50 per cent the potency of the whole liver. One can readily dissociate the iron from other potent factors in various tissues. Fractions of heart, liver, spleen, and kidney may contain very little iron yet cause much hemoglobin regeneration in anemic dogs. No investigator has reported any condition of copper deficiency in man or dog. In fact, in anemias copper is usually above normal concentration in the liver. It is unlikely, therefore, that in experimental anemia in dogs and in the various anemias of man, any significance attaches to the intake of copper. PMID:19870718

Hahn, P. F.; Whipple, G. H.

1938-01-01

206

How Is Pernicious Anemia Treated?  

MedlinePLUS

... from the NHLBI on Twitter. How Is Pernicious Anemia Treated? Doctors treat pernicious anemia by replacing the missing vitamin B12 in the body. People who have pernicious anemia may need lifelong treatment. The goals of treating ...

207

Managing Chemotherapy Side Effects: Anemia  

MedlinePLUS

... National Institutes of Health Managing Chemotherapy Side Effects Anemia Call your doctor or nurse if you feel: ? ... tired ? Your heart beating very fast What is anemia? Anemia is when your body doesn抰 have ...

208

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

PubMed Central

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

Mijatovic, Dino; Blagaic, Ana; Zupan, Zeljko

2014-01-01

209

Group A ?-hemolytic streptococcal pharyngotonsillitis outbreak  

PubMed Central

The aim was to describe an outbreak of group A ?-hemolytic streptococcal pharyngotonsillitis in health care professionals. This is a cross-sectional descriptive study of 17 clients who dined at the same table in a restaurant in Barcelona in July 2012. The frequency, timing and severity of symptoms were analyzed, as were demographic variables and others concerning the food ingested. The attack rate was 58.8%. Six of the 10 clients were positive for group A ?-hemolytic streptococcal. Six of the 13 individuals who handled the food involved in the dinner had symptoms. No association was identified with the food consumed. There is epidemiological evidence of foodborne group A ?-hemolytic streptococcal transmission, but respiratory transmission could not be ruled out. PMID:24897054

Culqui, Dante R; Manzanares-Laya, Sandra; Van Der Sluis, Sarah Lafuente; Fanlo, Albert Anton; Comas, Rosa Bartolom; Rossi, Marcello; Cayl, Jo醤 A

2014-01-01

210

Selenium deficiency in cattle associated with Heinz bodies and anemia.  

PubMed

Cattle grazing St. Augustine grass growing on peaty muck soils in the Florida Everglades developed anemia associated with the presence of Heinz bodies and suboptimal concentrations of selenium in blood. Selenium supplementation corrected the anemia, prevented Heinz body formation, increased the body weight of cows and calves, and elevated blood selenium. This may be the first recorded example of widespread anemia in a population due to selenium deficiency. PMID:6691160

Morris, J G; Cripe, W S; Chapman, H L; Walker, D F; Armstrong, J B; Alexander, J D; Miranda, R; Sanchez, A; Sanchez, B; Blair-West, J R

1984-02-01

211

Cyclosporine therapy during pregnancy in a patient with ?-thalassemia major and autoimmune haemolytic anemia: a case report and review of the literature.  

PubMed

Recent advances in the management of hemoglobinopathies offer an improved potential for safe pregnancy with favourable outcome in patients with ?-thalassemia major. Autoimmune diseases that are common in women at reproductive age might be fulminant and hardly manageable in pregnant women with thalassemia. Thus immunosuppressant drugs like cyclosporine A could be necessary in order to maintain good maternal and foetal health. We present a case report of a 35-year-old woman with ?-thalassemia major, splenectomy, autoimmune hemolytic anemia and insulin treated diabetes mellitus who was treated with cyclosporine A during her pregnancy, and delivered a healthy male infant. First line therapy with steroids was ineffective, due to deregulation of diabetes mellitus. PMID:23935353

Agapidou, A; Vlachaki, E; Theodoridis, T; Economou, M; Perifanis, V

2013-01-01

212

Fifth Cooley's anemia symposium  

SciTech Connect

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.

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

1985-01-01

213

Intestinal HIF2? promotes tissue-iron accumulation in disorders of iron overload with anemia  

PubMed Central

Several distinct congenital disorders can lead to tissue-iron overload with anemia. Repeated blood transfusions are one of the major causes of iron overload in several of these disorders, including ?-thalassemia major, which is characterized by a defective ?-globin gene. In this state, hyperabsorption of iron is also observed and can significantly contribute to iron overload. In ?-thalassemia intermedia, which does not require blood transfusion for survival, hyperabsorption of iron is the leading cause of iron overload. The mechanism of increased iron absorption in ?-thalassemia is unclear. We definitively demonstrate, using genetic mouse models, that intestinal hypoxia-inducible factor-2? (HIF2?) and divalent metal transporter-1 (DMT1) are activated early in the pathogenesis of ?-thalassemia and are essential for excess iron accumulation in mouse models of ?-thalassemia. Moreover, thalassemic mice with established iron overload had significant improvement in tissue-iron levels and anemia following disruption of intestinal HIF2?. In addition to repeated blood transfusions and increased iron absorption, chronic hemolysis is the major cause of tissue-iron accumulation in anemic iron-overload disorders caused by hemolytic anemia. Mechanistic studies in a hemolytic anemia mouse model demonstrated that loss of intestinal HIF2?/DMT1 signaling led to decreased tissue-iron accumulation in the liver without worsening the anemia. These data demonstrate that dysregulation of intestinal hypoxia and HIF2? signaling is critical for progressive iron overload in ?-thalassemia and may be a novel therapeutic target in several anemic iron-overload disorders. PMID:24282296

Anderson, Erik R.; Taylor, Matthew; Xue, Xiang; Ramakrishnan, Sadeesh K.; Martin, Angelical; Xie, Liwei; Bredell, Bryce X.; Gardenghi, Sara; Rivella, Stefano; Shah, Yatrik M.

2013-01-01

214

Intestinal HIF2? promotes tissue-iron accumulation in disorders of iron overload with anemia.  

PubMed

Several distinct congenital disorders can lead to tissue-iron overload with anemia. Repeated blood transfusions are one of the major causes of iron overload in several of these disorders, including ?-thalassemia major, which is characterized by a defective ?-globin gene. In this state, hyperabsorption of iron is also observed and can significantly contribute to iron overload. In ?-thalassemia intermedia, which does not require blood transfusion for survival, hyperabsorption of iron is the leading cause of iron overload. The mechanism of increased iron absorption in ?-thalassemia is unclear. We definitively demonstrate, using genetic mouse models, that intestinal hypoxia-inducible factor-2? (HIF2?) and divalent metal transporter-1 (DMT1) are activated early in the pathogenesis of ?-thalassemia and are essential for excess iron accumulation in mouse models of ?-thalassemia. Moreover, thalassemic mice with established iron overload had significant improvement in tissue-iron levels and anemia following disruption of intestinal HIF2?. In addition to repeated blood transfusions and increased iron absorption, chronic hemolysis is the major cause of tissue-iron accumulation in anemic iron-overload disorders caused by hemolytic anemia. Mechanistic studies in a hemolytic anemia mouse model demonstrated that loss of intestinal HIF2?/DMT1 signaling led to decreased tissue-iron accumulation in the liver without worsening the anemia. These data demonstrate that dysregulation of intestinal hypoxia and HIF2? signaling is critical for progressive iron overload in ?-thalassemia and may be a novel therapeutic target in several anemic iron-overload disorders. PMID:24282296

Anderson, Erik R; Taylor, Matthew; Xue, Xiang; Ramakrishnan, Sadeesh K; Martin, Angelical; Xie, Liwei; Bredell, Bryce X; Gardenghi, Sara; Rivella, Stefano; Shah, Yatrik M

2013-12-10

215

Characterization of hemolytic Escherichia coli strains in ferrets: recognition of candidate virulence factor CNF1.  

PubMed

Diseases associated with Escherichia coli infection are the subject of renewed interest due to emerging conditions such as hemolytic uremia syndrome. A collection of 15 strains of beta-hemolytic E. coli was isolated from diarrheic feces and diseased tissues of ferrets. All 15 strains were positive in specific PCR assays for the presence of hlyA, pap1, and cnf1. Seven of the cnf1-positive isolates were tested and shown to have a cytopathic effect on HeLa cell monolayers. The pathogenesis of these strains warrants future study. PMID:15583337

Marini, Robert P; Taylor, Nancy S; Liang, Alvin Y; Knox, Kimberly A; Pe馻, Jeremy Andrew; Schauer, David B; Fox, James G

2004-12-01

216

Characterization of Hemolytic Escherichia coli Strains in Ferrets: Recognition of Candidate Virulence Factor CNF1  

PubMed Central

Diseases associated with Escherichia coli infection are the subject of renewed interest due to emerging conditions such as hemolytic uremia syndrome. A collection of 15 strains of beta-hemolytic E. coli was isolated from diarrheic feces and diseased tissues of ferrets. All 15 strains were positive in specific PCR assays for the presence of hlyA, pap1, and cnf1. Seven of the cnf1-positive isolates were tested and shown to have a cytopathic effect on HeLa cell monolayers. The pathogenesis of these strains warrants future study. PMID:15583337

Marini, Robert P.; Taylor, Nancy S.; Liang, Alvin Y.; Knox, Kimberly A.; Pe馻, Jeremy Andrew; Schauer, David B.; Fox, James G.

2004-01-01

217

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

PubMed

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

Wang, Xiaoyan; Wang, Lixin; Liu, Sijin

2014-12-01

218

Group A Hemolytic Streptococcal Osteomyelitis in Children  

Microsoft Academic Search

Objective Little attention has been given to acute hematogenous osteomyelitis (AHO) caused by group A -hemolytic Streptococcus (GABHS), although up to 10% of cases are caused by this microorganism. The objective of this study was to define the clinical and laboratory characteristics of AHO caused by GABHS. Methods. Between January 1983 and June 1999, 29 patients were treated at Children's

Ekopimo O. Ibia; Menfo Imoisili; Andreas Pikis

2010-01-01

219

Reassessment of the microcytic anemia of lead poisoning  

SciTech Connect

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.

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

1981-06-01

220

MEGALOBLASTIC AND OTHER MACROCYTIC ANEMIA  

E-print Network

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

221

Anemia in Frailty  

PubMed Central

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

Roy, Cindy N.

2010-01-01

222

Anemia as a risk factor for chronic kidney disease  

Microsoft Academic Search

Chronic kidney disease (CKD) is an important and leading cause of end-stage renal disease (ESRD) and moreover, plays a role in the morbidity and mortality due to cardiovascular disease, infection, and cancer. Anemia develops during the early stages of CKD and is common in patients with ESRD. Anemia is an important cause of left ventricular hypertrophy and congestive heart failure.

K Iseki; K Kohagura

2007-01-01

223

Refractory anemia with ring sideroblasts.  

PubMed

Refractory anemia with ring sideroblasts (RARS) is a subtype of myelodysplastic syndrome (MDS) characterized by 15% or more ring sideroblasts in the bone marrow according to the WHO classification. After Perls staining, ring sideroblasts are defined as erythroblasts in which there are 5 or more siderotic granules covering at least a third of the nuclear circumference. The iron deposited in perinuclear mitochondria of ring sideroblasts is present in the form of mitochondrial ferritin. The molecular basis of MDS with ring sideroblasts has remained unknown until recently. In 2011, whole exome sequencing studies revealed somatic mutations of SF3B1, a gene encoding a core component of RNA splicing machinery, in myelodysplasia with ring sideroblasts. The close relationship between SF3B1 mutation and ring sideroblasts is consistent with a causal relationship, and makes SF3B1 the first gene to be associated with a specific morphological feature in MDS. RARS is mainly characterized by isolated anemia due to ineffective erythropoiesis, and its clinical course is generally benign, although there is a tendency to worsening of anemia in most patients over time. By contrast, refractory cytopenia with multilineage dysplasia and ring sideroblasts (RCMD-RS) is characterized by pancytopenia and dysplasia in two or more myeloid cell lineages. More importantly, patients with RCMD-RS have a higher risk of developing bone marrow failure or progressing to acute myeloid leukemia (AML). Refractory anemia with ring sideroblasts (RARS-T) associated with marked thrombocytosis is a myelodysplastic/myeloproliferative neoplasm associated with both SF3B1 and JAK2 or MPL mutations. RARS-T may develop from an SF3B1 mutated RARS through the acquisition of a JAK2 or MPL mutations in a subclone of hematopoietic cells. PMID:24507814

Malcovati, Luca; Cazzola, Mario

2013-12-01

224

Current issues of managing anemia in patients with advanced heart failure: Is there a new pathway?  

Microsoft Academic Search

Anemia is a common co-morbid found in patients with chronic heart failure with incidence widely ranging from 4% to 70% due to lack of a consistent definition of anemia in HF setting. This prevalence is higher in heart failure (HF) patients with NYHA class III朓V or refractory HF, and some trials have showed that persistence of anemia in this population

Leonardo Paskah Suciadi; Bambang Budi Siswanto

225

Anemia in Georgia centenarians and octogenarians.  

E-print Network

??This secondary data analysis examined the prevalence and proportion of several classifications of anemia in Georgia centenarians and octogenarians: anemia of chronic disease, nutritional anemia, (more)

Haslam, Alyson

2010-01-01

226

Anemia (For Parents)  

MedlinePLUS

... it can affect those of Middle Eastern and Mediterranean descent, as well as others. In this condition, ... of anemia. Thalassemia , which usually affects people of Mediterranean, African, and Southeast Asian descent, is marked by ...

227

Your Guide to Anemia  

MedlinePLUS

... feel tired or short of breath. Conserve your energy and don抰 overdo physical activity. Because your ... control anemia. These actions can give you greater energy, improve your quality of life, and help you ...

228

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- ...

229

How Is Anemia Diagnosed?  

MedlinePLUS

... to diagnose anemia is a complete blood count (CBC). The CBC measures many parts of your blood. The test ... can explain your test results to you. The CBC also checks the number of red blood cells, ...

230

An etiologic profile of anemia in 405 geriatric patients.  

PubMed

Background. Anemia is a common condition in the elderly and a significant risk factor for increased morbidity and mortality, reducing not only functional capacity and mobility but also quality of life. Currently, few data are available regarding anemia in hospitalized geriatric patients. Our retrospective study investigated epidemiology and causes of anemia in 405 hospitalized geriatric patients. Methods. Data analysis was performed using laboratory parameters determined during routine hospital admission procedures (hemoglobin, ferritin, transferrin saturation, C-reactive protein, vitamin B12, folic acid, and creatinine) in addition to medical history and demographics. Results. Anemia affected approximately two-thirds of subjects. Of 386 patients with recorded hemoglobin values, 66.3% were anemic according to WHO criteria, mostly (85.1%) in a mild form. Anemia was primarily due to iron deficiency (65%), frequently due to underlying chronic infection (62.1%), or of mixed etiology involving a combination of chronic disease and iron deficiency, with absolute iron deficiency playing a comparatively minor role. Conclusion. Greater awareness of anemia in the elderly is warranted due to its high prevalence and negative effect on outcomes, hospitalization duration, and mortality. Geriatric patients should be routinely screened for anemia and etiological causes of anemia individually assessed to allow timely initiation of appropriate therapy. PMID:24707396

Geisel, Tabea; Martin, Julia; Schulze, Bettina; Schaefer, Roland; Bach, Matthias; Virgin, Garth; Stein, J黵gen

2014-01-01

231

Postpartum anemia II: prevention and treatment.  

PubMed

This review focuses on the prevention and treatment of anemia in women who have just given childbirth (postpartum anemia). The problem of anemia both prepartum and postpartum is far more prevalent in developing countries than in the Western societies. The conditions for mother and child in the postpartum, nursing, and lactation period should be as favorable as possible. Many young mothers have a troublesome life due to iron deficiency and iron deficiency anemia (IDA) causing a plethora of symptoms including fatigue, physical disability, cognitive problems, and psychiatric disorders. Routine screening for postpartum anemia should be considered as part of the national maternal health programs. Major causes of postpartum anemia are prepartum iron deficiency and IDA in combination with excessive blood losses at delivery. Postpartum anemia should be defined as a hemoglobin level of <110爂/l at 1爓eek postpartum and <120爂/l at 8爓eeks postpartum. Bleeding exceeding normal blood losses of approximately 300爉l may lead to rapid depletion of body iron reserves and may, unless treated, elicit long-standing iron deficiency and IDA in the postpartum period. The prophylaxis of postpartum anemia should begin already in early pregnancy in order to ensure a good iron status prior to delivery. The most reliable way to obtain this goal is to give prophylactic oral ferrous iron supplements 30-50爉g daily from early pregnancy and take obstetric precautions in pregnancies at risk for complications. In the treatment of slight-to-moderate postpartum IDA, the first choice should be oral ferrous iron 100 to 200爉g daily; it is essential to analyze hemoglobin after approximately 2爓eeks in order to check whether treatment works. In severe IDA, intravenous ferric iron in doses ranging from 800 to 1,500爉g should be considered as first choice. In a few women with severe anemia and blunted erythropoiesis due to infection and/or inflammation, additional recombinant human erythropoietin may be considered. Blood transfusion should be restricted to women who develop circulatory instability due to postpartum hemorrhage. National health authorities should establish guidelines to combat iron deficiency in pregnancy and postpartum in order to facilitate a prosperous future for both mothers and children in a continuing globalized world. PMID:22160256

Milman, Nils

2012-02-01

232

Serotypes of beta-hemolytic Treponema hyodysenteriae.  

PubMed Central

Cultures form 13 isolates of pathogenic, beta-hemolytic Treponema hyodysenteriae from 11 geographically separate outbreaks and 2 experimentally induced cases of swine dysentery were lyophilized and extracted with hot phenol-water. The resulting water phases were examined serologically with antisera produced in rabbits against whole-cell bacterins of the 13 isolates for evidence of antigenic classes within the species. Water-phase antigens gave precipitin reactions with homologous antisera. Results from cross-testing of each water phase with each antiserum showed four serologically distinct groups among the isolants examined. Based on precipitin reactions in agarose gel, four serotypes of pathogenic, beta-hemolytic T. hyodysenteriae are proposed. Images PMID:115788

Baum, D H; Joens, L A

1979-01-01

233

Assessment of Hemolytic and Bactericidal Complement Activities in Normal and Mastitic Bovine Milk  

Microsoft Academic Search

Bactericidal and hemolytic comple- ment activities were investigated in 51 quarter milk samples of 13 cows in late lactation. Hemolytic activity was in all of the samples but one, after accounting for whey inhibitory activity. Mean hemolytic activity and inhibitory activities were .18 and .34 complement hemolytic units. Inflammation, in relation to infection status, increased hemolytic titers and heat-labile bactericidal

P. Rainard; B. Poutrel; J. P. Caffin

1984-01-01

234

Erythrogram and red cell distribution width of Equidae with experimentally induced anemia.  

PubMed

The erythrogram (erythrocyte histogram) and red cell distribution width (RDW) were evaluated in 5 purebred horses and 1 pony of mixed breeding with experimentally induced anemia. Four horses were studied for 6 weeks after 20% of their estimated blood volume was removed on each of 2 consecutive days (40% total blood loss; acute blood-loss group). Two horses were given acetylphenyl hydrazine IV daily, until acute Heinz body hemolytic anemia was induced; the 2 horses were then evaluated for 6 weeks. One horse and the pony had 20% of their estimated blood volume removed via phlebotomy once each week for 8 weeks to induce iron-deficiency anemia (chronic blood-loss group); the horse had been partially depleted of iron before the study began. Weekly blood samples were examined for changes in the erythrogram, RDW, mean cell volume (MCV), and erythrocyte glucose-6-phosphate dehydrogenase activity. Fourteen days after acute blood loss, mild increases were seen in the MCV, which persisted to day 42. The RDW was increased at day 14 and remained increased until day 42; however, the percentage increase was double that of the MCV at days 14, 21, and 28. Erythrograms had mild extensions of the right slope at days 14 to 28. Mean erythrocyte glucose-6-phosphate dehydrogenase activity increased in all 3 groups, but individual concentrations were erratic. In the 2 horses with acute hemolytic anemia, modest increases of similar magnitude were seen in RDW and MCV.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3000231

Easley, J R

1985-11-01

235

Initiation and Regulation of Complement during Hemolytic Transfusion Reactions  

PubMed Central

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

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

236

Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation  

PubMed Central

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

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

2014-01-01

237

Familial Atypical Hemolytic Uremic Syndrome: A Review of Its Genetic and Clinical Aspects  

PubMed Central

Atypical hemolytic uremic syndrome (aHUS) is a rare renal disease (two per one million in the USA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Both sporadic (80% of cases) and familial (20% of cases) forms are recognized. The study of familial aHUS has implicated genetic variation in multiple genes in the complement system in disease pathogenesis, helping to define the mechanism whereby complement dysregulation at the cell surface level leads to both sporadic and familial disease. This understanding has culminated in the use of Eculizumab as first-line therapy in disease treatment, significantly changing the care and prognosis of affected patients. However, even with this bright outlook, major challenges remain to understand the complexity of aHUS at the genetic level. It is possible that a more detailed picture of aHUS can be translated to an improved understanding of disease penetrance, which is highly variable, and response to therapy, both in the short and long terms. PMID:23251215

Bu, Fengxiao; Borsa, Nicolo; Gianluigi, Ardissino; Smith, Richard J. H.

2012-01-01

238

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 ...

239

Selection of peptides for serological detection of equine infectious anemia.  

PubMed

Equine infectious anemia caused by equine infectious anemia virus is an important disease due to its high severity and incidence in animals. We used a phage display library to isolate peptides that can be considered potential markers for equine infectious anemia diagnosis. We selected peptides using IgG purified from a pool comprised of 20 sera from animals naturally infected with equine infectious anemia virus. The diagnostic potential of these peptides was investigated by ELISA, Western blot and dot blot with purified IgG and serum samples. Based on the results, we chose a peptide mimetic for glycoprotein gp45 epitopes of equine infectious anemia virus, with potential for use as an antigen in indirect diagnostic assays. Synthesis of this peptide has possible applications for the development of new diagnostic tools for this disease. PMID:22653674

Santos, E M; Cardoso, R; Souza, G R L; Goulart, L R; Heinemann, M B; Leite, R C; Reis, J K P

2012-01-01

240

How Is Fanconi Anemia Treated?  

MedlinePLUS

... from the NHLBI on Twitter. How Is Fanconi Anemia Treated? Doctors decide how to treat Fanconi anemia (FA) based on a person's age and how ... Long-term treatments for FA can: Cure the anemia. Damaged bone marrow cells are replaced with healthy ...

241

[Equine Infectious Anemia (EIA)].  

PubMed

Equine Infectious Anemia (EIA) is a reportable, eradicable epizootic disease caused by the equine lentivirus of the retrovirus family which affects equids only and occurs worldwide. The virus is transmitted by blood, mainly by sanguivorous insects. The main symptoms of the disease are pyrexia, apathy, loss of body condition and weight, anemia, edema and petechia. However, infected horses can also be inapparent carriers without any overt signs. The disease is diagnosed by serological tests like the Coggins test and ELISA tests. Presently, Switzerland is offi cially free from EIA. However, Switzerland is permanently at risk of introducing the virus as cases of EIA have recently been reported in different European countries. PMID:19333901

Kaiser, A; Meier, H P; Straub, R; Gerber, V

2009-04-01

242

Hemolytik: a database of experimentally determined hemolytic and non-hemolytic peptides  

PubMed Central

Hemolytik (http://crdd.osdd.net/raghava/hemolytik/) is a manually curated database of experimentally determined hemolytic and non-hemolytic peptides. Data were compiled from a large number of published research articles and various databases like Antimicrobial Peptide Database, Collection of Anti-microbial Peptides, Dragon Antimicrobial Peptide Database and Swiss-Prot. The current release of Hemolytik database contains ?3000 entries that include ?2000 unique peptides whose hemolytic activities were evaluated on erythrocytes isolated from as many as 17 different sources. Each entry in Hemolytik provides comprehensive information about a peptide, like its name, sequence, origin, reported function, property such as chirality, types (linear and cyclic), end modifications as well as details pertaining to its hemolytic activity. In addition, tertiary structure of each peptide has been predicted, and secondary structure states have been assigned. To facilitate the scientific community, a user-friendly interface has been developed with various tools for data searching and analysis. We hope, Hemolytik will be useful for researchers working in the field of designing therapeutic peptides. PMID:24174543

Gautam, Ankur; Chaudhary, Kumardeep; Singh, Sandeep; Joshi, Anshika; Anand, Priya; Tuknait, Abhishek; Mathur, Deepika; Varshney, Grish C.; Raghava, Gajendra P. S.

2014-01-01

243

Anemia and inflammatory bowel diseases  

Microsoft Academic Search

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,

Fernando Gomoll髇; Javier P Gisbert

2009-01-01

244

Congenital spherocytic anemia  

MedlinePLUS

This outcome is usually good with treatment. After the spleen is removed, the life span of the red blood cell returns to normal. ... Gallstones Much lower red blood cell production (aplastic crisis) caused by a viral infection, which can make anemia worse

245

Anemia and School Participation  

ERIC Educational Resources Information Center

Anemia is among the most widespread health problems for children in developing countries. This paper evaluates the impact of a randomized health intervention delivering iron supplementation and deworming drugs to Indian preschool children. At baseline, 69 percent were anemic and 30 percent had intestinal worm infections. Weight increased among

Bobonis, Gustavo J.; Miguel, Edward; Puri-Sharma, Charu

2006-01-01

246

Sickle Cell Anemia Bibliography.  

ERIC Educational Resources Information Center

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.

Christy, Steven C.

247

Hepcidin and sports anemia  

PubMed Central

Iron is an important mineral element used by the body in a variety of metabolic and physiologic processes. These processes are highly active when the body is undergoing physical exercises. Prevalence of exercise-induced iron deficiency anemia (also known as sports anemia) is notably high in athletic populations, particularly those with heavy training loads. The pathogenesis of sports anemia is closely related to disorders of iron metabolism, and a more comprehensive understanding of the mechanism of iron metabolism in the course of physical exercises could expand ways of treatment and prevention of sports anemia. In recent years, there have been remarkable research advances regarding the molecular mechanisms underlying changes of iron metabolism in response to physical exercises. This review has covered these advances, including effects of exercise on duodenum iron absorption, serum iron status, iron distribution in organs, erythropoiesis, and hepcidin抯 function and its regulation. New methods for the treatment of exercise-induced iron deficiency are also discussed. PMID:24731443

2014-01-01

248

Cooley's Anemia Foundation  

MedlinePLUS

... Chapter Donate We're Leaders in World Class Medical Research You Can Help Donate Now Julia抯 Story Born ... The Cooley抯 Anemia Foundation is accepting applications for medical research grants and fellowships in areas related to thalassemia. ...

249

Hemolytic activity of five different calcium silicates.  

PubMed Central

Mineral characteristics and the in vitro hemolytic activity of three synthetic and two natural calcium silicates (CaSi) are compared. Hemolysis is higher for the synthetic compounds than for the natural ones. The difference is accentuated by weak ultrasonication of the minerals. No variation was observed within the two groups, including both acicular and fibrous forms. Calcium was released from the minerals during storage in Tris-buffered saline. At the same time, hemolysis decreased, and crystallographic alterations occurred in the leached minerals. Treatment of the CaSi with calcium chelators (EGTA and EDTA) did not change hemolytic activity. An increase was observed when 30 mM calcium was added. Hemolysis is related to specific surface areas and the crystalline structure of the minerals. Calcium may also be a contributing factor. Images FIGURE 1. a FIGURE 1. b FIGURE 1. c FIGURE 1. d FIGURE 1. e FIGURE 1. f FIGURE 7. a FIGURE 7. b FIGURE 7. c FIGURE 7. d FIGURE 7. e FIGURE 7. f PMID:6315361

Skaug, V; Gylseth, B

1983-01-01

250

Anemia and inflammatory bowel diseases  

PubMed Central

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

Gomoll髇, Fernando; Gisbert, Javier P

2009-01-01

251

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

SciTech Connect

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.

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

1982-07-01

252

Myopathy with altered mitochondria due to a triosephosphate isomerase (TPI) deficiency  

Microsoft Academic Search

Morphological changes are shown in the muscle biopsy specimens of an 8-year-old girl who suffered from a triosephosphate isomerase (TPI) deficiency, resulting in a chronic, nonspherocytic, hemolytic anemia, mental retardation and neuromuscular impairment. The newly introduced enzyme histochemical reaction for TPI demonstrated a total lack of histochemically detectable enzyme activity, whereas biochemical analysis of muscle tissue revealed less than 10%

A. Bardosi; S. W. Eber; M. Hendrys; A. Pekrum

1990-01-01

253

Heinz body anemia and methemoglobinemia in ponies given red maple (acer rubrum L.) leaves.  

PubMed

Ponies given dried red maple (Acer rubrum L.) leaves at a dose of 3.0 gm/kg body weight became ill and died one to five days after administration of the leaves. Two clinical patterns of disease were seen. Ponies given dried leaves collected after September 15 died by 18 hours, while ponies given dried leaves collected before September 15 became ill with a hemolytic syndrome and died by three to five days. Freshly harvested leaves administered immediately after collection did not produce disease in ponies, but when dried, they became toxic and remained so for at least 30 days. Overnight freezing did not alter the toxicity of the leaves. Leaves were toxic when administered at doses of 1.5 gm/kg of body weight. The clinical signs of ponies with the hemolytic syndrome included polypnea, tachycardia, icterus, cyanosis, scleral petechiation, and brownish discoloration of the urine and blood. Blood changes of ponies with the hemolytic syndrome included anemia, hemoglobinemia, Heinz bodies, depletion of erythrocyte reduced glutathione, increased erythrocyte fragility, and increased serum levels of aspartate amino transferase, sorbitol dehydrogenase, plasma protein, and bilirubin. Lesions of ponies that died from the hemolytic syndrome included icterus, centrilobular hepatic degeneration, hemoglobinemic nephrosis, and erythrophagocytosis by splenic, adrenal, and hepatic phagocytes. Only brownish discoloration of the blood and mild centrilobular hepatic degeneration were observed in the four ponies that died peracutely. PMID:7147611

George, L W; Divers, T J; Mahaffey, E A; Suarez, M J

1982-09-01

254

Acute hemolytic anemia induced by oral administration of indole in ponies.  

PubMed

Eight ponies were allotted to 2 groups of 4. Group-1 ponies (1-4) were given 0.2 g of indole/kg of body weight orally and group-2 ponies (5 to 8) were given 0.1 g of indole/kg. Various physical, hematologic, and physiologic measurements were obtained after administration of indole. Intravascular hemolysis and hemoglobinuria were detected in both groups within 24 hours of dosing. Hemolysis was reflected by decreases in PCV, hemoglobin concentration, and RBC count, and an increase in indirect bilirubin. Erythrocyte fragility appeared to increase in both groups at 8 hours after dosing and peaked at 16 hours after dosing. At 72 hours after dosing, the RBC fragility value was less than predose measurements. Heinz body formation was noticed in group-2 ponies, but not in group 1. Plasma indole concentrations increased in both groups from the nondetectable predose concentrations. Group-1 values were 203% of group-2 values. In group 2, plasma indole was nondetectable by 12 hours, whereas low concentrations could still be measured in the group-1 ponies at 24 hours. Ponies in group 1 died or were euthanatized between 24 and 72 hours after dosing, whereas group-2 ponies were euthanatized between 48 and 120 hours. At necropsy, all body fat, mucous membranes, and elastic tissue were stained yellow. Hemoglobinuric nephrosis was the most prominent microscopic lesion. Results of this study indicated that indole, a metabolite of the amino acid tryptophan, causes acute intravascular hemolysis in ponies. PMID:1854101

Paradis, M R; Breeze, R G; Laegreid, W W; Bayly, W M; Counts, D F

1991-05-01

255

[An aplastic anemia patient died of severe anemia who refused transfusion].  

PubMed

A 41-year-old woman who had been given a diagnosis of aplastic anemia 14 years before was admitted because of recurrence of the disease. Despite therapy, the anemia progressed gradually. The patient refused blood product transfusions for religious reasons. Angina pectoris-like chest pain without ischemic changes on electrocardiograms appeared at a hemoglobin concentration (Hb) of 1.6 g/dl. The patient died of heart failure at Hb 1.5 g/dl. Autopsy showed enlargement of the heart, fatty changes in the myocardium and liver due to chronic hypoxia, and no changes in coronary arteries. PMID:10695402

Iki, S; Ohbayashi, Y; Urabe, A

2000-01-01

256

Hb Trento: an elongated C-terminal beta chain due to a new frameshift mutation [beta144 (-A)].  

PubMed

An elongated C-terminal hemoglobin variant, due to the deletion of nucleotide A in codon 144 (nucleotide 63600 GenBank entry UO1317) was found in a 31-year-old woman from Trento (northeastern Italy). This deletion led to the replacement of lysine at beta144 by a serine residue, the disappearance of the stop codon at position 147, and the presence of 12 additional residues, identical to those observed in Hbs Saveme, Tak and Cranston, which result from a similar mechanism. Hb Trento, amounting to 29% of the total hemoglobin, was unstable and had, as the other variants of this group, an increased oxygen affinity. It led to a mild compensated hemolytic anemia with red cell inclusion bodies. Functional studies of the isolated abnormal hemoglobin were difficult to perform because of autoxidation, precipitation, and formation of hybrids with Hb A. PMID:12603089

Ivaldi, Giovanni; David, Onorata; Baffico, Maria; Leone, Daniela; Baldi, Maurizia; Parodi, Maria Isola; Scim-Degani, Valeria; Piga, Antonio; Scagni, Paola; Rabino-Massa, Emma; Ricco, Giuseppe

2003-02-01

257

Hemolytic Uremic Syndrome following Infection with O111 Shiga Toxin-Producing Escherichia coli Revealed through Molecular Diagnostics  

PubMed Central

We report a case of hemolytic uremic syndrome in a 69-year-old woman due to Shiga toxin-producing Escherichia coli, possibly serotype O111, to illustrate the potentially deleterious implications of a Campylobacter enzyme immunoassay (EIA) result and the increasing importance of molecular testing when conventional methods are limited. PMID:24371241

Operario, Darwin J.; Moonah, Shannon

2014-01-01

258

Management of anemia induced by triple therapy in patients with chronic hepatitis C: challenges, opportunities and recommendations.  

PubMed

The addition of protease inhibitors, boceprevir or telaprevir, to peginterferon+ribavirin (PegIFN/RBV) increases the frequency as well as the severity, and hence, clinical relevance of anemia, which has now become one of the major complications associated with triple therapy. Most significant factors associated with anemia in patients receiving triple therapy include older age, lower body mass index (BMI), advanced fibrosis, and lower baseline hemoglobin. The variability in inosine triphosphate pyrophosphatase (ITPA) gene, which encodes a protein that hydrolyses inosine triphosphate (ITP), has been identified as an essential genetic factor for anemia both in dual and triple therapy. The correct management of anemia is based on anticipation, characterization and therapeutic management. Basically, anemia can be characterized in 3 types: ferropenic (mostly in fertile women), thalassemic type hemolytic anemia, and anemia from chronic processes. Functional deficit of iron should also be excluded in patients with normal ferritin and lower saturation of transferrin. Ribavirin dose reduction and epoetin, sequentially, are indicated in the management of anemia. Epoetin non-response can be caused by lack of time, type of anemia, functional iron deficit or erythropoietin resistance. In the transplantation setting, adding a protease inhibitor to PegIFN/RBV results in a significant increase in the incidence and severity of anemia and, as a consequence, a greater need for epoetin, transfusions, and ribavirin dose reductions. Packed red cell transfusions are utilized when hemoglobin decreases to less than 7.5g/dl and/or there are clinical symptoms and/or there is no response to other therapeutic measures. PMID:23867320

Romero-G髆ez, Manuel; Berenguer, Marina; Molina, Esther; Calleja, Jos Luis

2013-12-01

259

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

SciTech Connect

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.

Bernstein, S.E.

1984-09-01

260

Anemia and Cancer  

Microsoft Academic Search

This chapter explores the management of anemia in older cancer patients. Cancer is a disease of aging: more than 50% of all\\u000a malignancies currently occur in the 12% of the population aged 65 and over; by the year 2030 older individuals are expected\\u000a to account for 20% of the population and 70% of all cancer cases (1). Though not unique

Kaaron Benson; Lodovico Balducci; Matti Aapro

261

In Vitro Prostacyclin Production in the Hemolytic-Uremic Syndrome  

PubMed Central

Reports from Europe suggest that the hemolytic-uremic syndrome is associated with an impaired ability to produce prostacyclin (prostaglandin [PG] I2), a potent inhibitor of platelet aggregation and thrombus formation. In comparing the production of PGI2 by cultured endothelial cells using serum obtained from 22 children with the hemolytic-uremic syndrome with values obtained using serum from 22 normal children, we found that cultured endothelial cells produced less PGF1? (the stable metabolite of PGI2) when incubated with affected serum. The relationship of this observation to the pathogenesis of the hemolytic-uremic syndrome is unclear. PMID:3754077

Siegler, Richard L.; Smith, Jean B.; Lynch, Michael B.; Mohammad, S. F.

1986-01-01

262

Who Is at Risk for Anemia?  

MedlinePLUS

... inherited anemia, such as sickle cell anemia or thalassemia Rate This Content: Next >> May 18, 2012 Anemia Clinical Trials Clinical trials are research studies that explore whether a medical strategy, treatment, ...

263

Genetics Home Reference: Dyserythropoietic anemia and thrombocytopenia  

MedlinePLUS

... Recent literature OMIM Genetic disorder catalog Conditions > Dyserythropoietic anemia and thrombocytopenia On this page: Description Genetic changes ... Glossary definitions Reviewed October 2014 What is dyserythropoietic anemia and thrombocytopenia? Dyserythropoietic anemia and thrombocytopenia is a ...

264

Genetics Home Reference: Diamond-Blackfan anemia  

MedlinePLUS

... literature OMIM Genetic disorder catalog Conditions > Diamond-Blackfan anemia On this page: Description Genetic changes Inheritance Diagnosis ... definitions Reviewed February 2012 What is Diamond-Blackfan anemia? Diamond-Blackfan anemia is a disorder of the ...

265

Iron-Deficiency Anemia (For Parents)  

MedlinePLUS

... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ... red blood cells. This is a condition called anemia . When someone has anemia, less oxygen reaches the ...

266

[Role of Helicobacter pylori infection in iron deficiency anemia].  

PubMed

Anemia induced by digestive diseases refers to anemia due to iron deficiency. Conventional gastrointestinal diagnostic workup fails to establish the cause of iron deficiency in about one third of patients. Abnormal iron absorption is increasingly recognized as an important cause of unexplained iron deficiency. The importance of coeliac disease as a possible cause of iron deficiency anemia refractory to oral iron treatment, without other manifestations of malabsorption syndrome, is increasingly being recognized. In addition, Helicobacter pylori (HP) has been implicated in several recent studies as a cause of iron deficiency anemia (IDA) refractory to oral iron treatment. Cure of previously refractory IDA by HP eradication provides strong evidence supporting a cause-and-effect relationship. In order to establish a cause-and-effect relationship between HP gastritis and IDA, prospective randomized studies comparing the effects of iron administration with or without H. pylorieradication are highly recommended. PMID:21196096

Chaabane, Nabil Ben; Mansour, Imed Ben; Hellara, Olfa; Loghmeri, Hichem; Bdioui, Fethia; Safer, Leila; Saffar, Hamouda

2011-03-01

267

Anemia associated with chronic heart failure: current concepts  

PubMed Central

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

Shah, Ravish; Agarwal, Anil K

2013-01-01

268

Modification of sticholysin II hemolytic activity by free radicals  

Microsoft Academic Search

Sticholysin II is a highly hemolytic toxin present in the caribbean sea anemone Stichodactyla helianthus. Pre-incubation of St II with 2,2?-azobis(2-amidinopropane), a source of peroxyl radicals in air saturated solution, readily reduces its hemolytic activity. Analysis of the amino acids present in the protein after its modification shows that only tryptophan groups are significantly modified by the free radicals. According

Isabel F. Pazos; Carlos Alvarez; Maria E. Lanio; Diana Martinez; Vivian Morera; Eduardo A. Lissi; Ana M. Campos

1998-01-01

269

Sickle Cell Anemia  

NSDL National Science Digital Library

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.

Debra L. Stamper

2000-01-01

270

The Anemia of Heart Failure  

Microsoft Academic Search

Anemia is common in congestive heart failure (CHF) and is associated with an increased mortality and morbidity. The most likely causes of anemia are chronic kidney disease (CKD) and excessive cytokine production, both of which can cause depression of erythropoietin (EPO) production and bone marrow activity. The cytokines also induce iron deficiency by both reducing gastrointestinal iron absorption and iron

Donald S. Silverberg; Dov Wexler; Alberto Palazzuoli; Adrian Iaina; Doron Schwartz

2009-01-01

271

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

SciTech Connect

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.

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

1991-03-01

272

STUDIES ON BACTERIOPHAGES OF HEMOLYTIC STREPTOCOCCI  

PubMed Central

The host ranges of bacteriophages for group A, types 1, 6, 12, and 25 and group C streptococci have been determined. The findings indicate that the susceptibility to these phages is primarily a group-specific phenomenon, although it is modified by several factors such as the hyaluronic acid capsule, lysogeny, and possibly the presence of surface proteins. Phage antibody studies indicate that while the group A phages are antigenically related, they are distinct from the group C phage. This is in agreement with the observation that group A phages are not specific for their homologous streptococcal types. The purified group C carbohydrate inactivates group C phage but not the group A phages, thus suggesting that the carbohydrate, a component of the cell wall, may serve as the phage receptor site. It has not been possible to inactivate the group A phages with group A carbohydrate. Phage lysis of groups A and C streptococci is accompanied by fragmentation of the cell wall since the C carbohydrate has been identified serologically and chemically in the supernate of centrifuged lysates. The immediate lysis of groups A and C hemolytic streptococci and their isolated cell walls by an accesory heat-labile lytic factor in fresh group C lysates is also described. PMID:13463248

Krause, Richard M.

1957-01-01

273

Mitochondrial Iron Metabolism and Sideroblastic Anemia  

Microsoft Academic Search

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

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

2009-01-01

274

Managing Anemia of Chronic Kidney Disease  

Microsoft Academic Search

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

Susan A. Krikorian

2009-01-01

275

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: ...

276

Anemia after traumatic spinal cord injury.  

PubMed

The incidence and natural history of anemia in patients with spinal cord injuries (SCI) were investigated in a prospective study of 68 patients consecutively admitted to a regional acute SCI unit. Fifty had SCI and 18 had spine injuries (SI) without neurologic deficit. Thirty-six of 41 males (88%) and six of nine females (67%) with SCI were anemic on at least one occasion. In the first two weeks after injury, in females and in males, there was no significant difference in mean hemoglobin level between SI and SCI patients. At six weeks, no male with SI was anemic, and males with SCI had significantly lower mean hemoglobin levels than those with SI (121.6 g/L vs 145.4 g/L, p less than .001). Identified early causes of anemia were blood loss due to bony soft tissue or visceral injury, gastrointestinal bleeding, and surgery. In the postacute phase (more than six weeks after injury), anemia occurred in 25 of 41 male and three of nine female SCI patients, and its occurrence was associated with the presence of an identified chronic disease, especially urinary tract infection. PMID:1998453

Hirsch, G H; Menard, M R; Anton, H A

1991-03-01

277

Current treatment of atypical hemolytic uremic syndrome  

PubMed Central

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

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

2014-01-01

278

Heinz-body anemia: "bite cell" variant--a light and electron microscopic study.  

PubMed

Light and scanning electron microscopic studies of blood from five patients with drug-induced oxidant hemolysis are presented. None of the patients had a previous history of hemolytic disease and laboratory studies indicated no evidence of either glucose-6-phosphate dehydrogenase (G6PD) deficiency or unstable hemoglobinopathy. Although the red cell deformities in our patients overlapped to some extent with those reported in patients with microangiopathic hemolytic anemia (MAHA) and in patients with G6PD deficiency undergoing oxidant hemolysis, striking differences were also observed. Cell fragments, commonly found in patients with MAHA, and eccentrocytes, frequently found in patients with G6PD deficiency undergoing oxidant hemolysis, were seldom found in blood samples from the five patients in this study. Bite cells were extremely common in our patients. They are rare in patients with either of the above disorders. An awareness of the morphologic abnormalities detailed in this report may help characterize the nature of a hemolytic process so that appropriate therapy can be initiated. PMID:6613984

Ward, P C; Schwartz, B S; White, J G

1983-09-01

279

Living with Sickle Cell Anemia  

MedlinePLUS

... and trans fatty acids. Refined grains come from processing whole grains, which results in a loss of ... stresses related to sickle cell anemia, including: Body-image problems caused by delayed sexual maturity. Coping with ...

280

Anemia in People with Cancer  

MedlinePLUS

... cause of your anemia. These could include: Blood chemistry tests to check organ function and levels of ... risks. The most common problem is a transfusion reaction. This happens when a patient抯 immune system attacks ...

281

Facts about Diamond Blackfan Anemia  

MedlinePLUS

... DBA Diamond Blackfan anemia (DBA) is a rare blood disorder that is also associated with birth defects or ... in the United States and Canada. Related Pages Blood Disorders Homepage CDC抯 National Center on Birth Defects and ...

282

Importance of Sideropenic Anemia in the Diagnosis of Gastrointestinal Tract Tumors  

PubMed Central

Introduction: Sideropenic anemia is a hypochromic, microcytic anemia caused by insufficient iron level in the body. This is the most common anemia. In a large percentage it is the symptom of gastrointestinal tract cancer. Anemia was defined by hemoglobin level <119 g/dl, hematocrit <0.356 for women or hemoglobin level <138 g/dL and hematocrit <0415 for men. Gastric cancer after lung cancer is the second most common malignant tumor in the world. Frequent localization is the antrum, and less frequently in the cardia and fundus. Definite factors in the development of gastric cancer are chronic atrophic gastritis, H. pylori, intestinal metaplasia, and epithelial dysplasia as a precancerous lesion. Strong link between sideropenic anemia and gastrointestinal tract cancers recommend that patients with sideropenic anemia without a clear indication underwent same gastroscopic and colonoscopy examination. The goals were to prove sideropenic anemia, diagnose and histologically confirm tumors, tumors location and correlates anemia with tumor anemia or show the dependence of anemia on tumor Results: The study included 100 subjects (50 from counseling center for hematology that came due to sideropenic anemia and 50 patients from the Clinic for Gastroenterology who had gastrointestinal tract cancer). Respondents had regular laboratory tests and endoscopic examinations, ultrasound of the abdomen, CT of the abdomen and tumor markers. In the group of patients from Counseling center for hematology with sideropenic anemia was found 11 cancerous processes, mostly in form of gastric and colon cancer. In the group of patients hospitalized at the Clinic for Gastroenterology most cancer process were localized in the stomach and colorectum. Conclusion: Tumors of the gastrointestinal tract are the most common cause of sideropenic anemia, due to which the patients often first contact Counseling center for hematology. Sideropenic anemia is more common in men as also the number of digestive tract cancers in men. Sideropenic anemia has a significant place in the diagnosis of gastrointestinal tract tumors. Sideropenic anemia is most common in men after 50 years of age. The most common tumors of the gastrointestinal tube were gastric and colon cancer. PMID:23922517

Sahovic, Sanela; Vukobrat-Bijedic, Zora; Sahovic, Vahidin

2012-01-01

283

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

Federal Register 2010, 2011, 2012, 2013, 2014

...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...

2013-12-30

284

Hemolytic Activities of the Candida Species in Liquid Medium  

PubMed Central

Objective The aim of this study was to evaluate the in vitro hemolytic activities of 107 Candida strains isolated from different clinical samples in liquid medium, and to examine the impact of glucose on this activity. Materials and Methods A total of 107 Candida isolates representing seven species (C. albicans, n=28; C. glabrata, n=23; C. tropicalis, n=17; C. parapsilosis, n=16; C. kefyr, n=14; C. krusei, n=5; C. guilliermondii, n=4) were included in the study. The hemolytic activities of the strains were tested on two different Sabouraud dextrose liquid media (SDB) containing 7% defibrinated human blood, one of which is supplemented with 3% glucose and the other without glucose. Cultures were evaluated at the end of a 48-hour incubation. The hemolysis in the media was detected spectrophotometrically by measuring the amount of released hemoglobin and compared with a standard hemolysate which was prepared prior to testing. The degree of hemolysis (percentage value) by an individual strain was calculated according to the following formula below: (Absorbance of supernatant media at 540 nm / Absorbance of standard hemolysate at 540 nm X 100). Results In the liquid medium without glucose, strains generally produced hemolysis at low levels. The degree of hemolysis produced by all species increased noticeably in the liquid medium with glucose. Strains of C. albicans and C.kefyr had demonstrated significant hemolytic activity, whereas others had lower activity. C. parapsilosis exerted very little hemolytic activity in the medium with glucose and showed no activity in the medium without glucose. Conclusion The hemolytic activities of most Candida species was found to be higher in the human blood-enriched SDB medium containing 3% additive glucose than in the one free from additives. This result indicates that increased blood glucose concentration may contribute to increased hemolytic activity in Candida species, and it suggests a parallel with possible pathogenesis of Candida in patients with diabetes mellitus.

Malcok, Hilal Kuzucu; Aktas, Esin; Ayyildiz, Ahmet; Yigit, Nimet; Yazgi, Halil

2009-01-01

285

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

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

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

2012-01-01

286

Persistent pulmonary hypertension of the newborn associated with severe congenital anemia of various etiologies.  

PubMed

Among the many associated features of persistent pulmonary hypertension of the neonate (PPHN), severe congenital anemia has been described only occasionally and is not included in the list of conditions that may cause PPHN in the neonate. We describe the clinical course of a group of 12 full-term neonates with PPHN and congenital anemia due to congenital dyserythropoietic anemia (7/12), ? thalasemia (1/12), Diamond-Blackfan (1/12), and epsilon gamma delta beta thalassemia (3/12). The association of congenital anemia and PPHN is more common than previously thought; it can exist with various etiologies and severity of anemia. Congenital anemia has not been described until now as a cause or risk factor for PPHN; it should be considered as such alone or in combination with other known causes to be recognized early and treated appropriately to improve outcome. In families with known cases of congenital anemia due to the above-mentioned diagnosis, closer prenatal follow-up should be offered to anticipate possible fetal distress and/or fetal anemia and PPHN after birth. PMID:24309603

Landau, Danielle; Kapelushnik, Josef; Harush, Miri B; Marks, Kyla; Shalev, Hanna

2015-01-01

287

What Are the Signs and Symptoms of Anemia?  

MedlinePLUS

... Twitter. What Are the Signs and Symptoms of Anemia? The most common symptom of anemia is fatigue ( ... mild symptoms or none at all. Complications of Anemia Some people who have anemia may have arrhythmias ( ...

288

LEUKEMIA, MULTIPLE MYELOMA, AND APLASTIC ANEMIA IN AMERICAN RADIOLOGISTS  

Microsoft Academic Search

A survey of 425 death certificates of radiologists dying between the ; ages of 35 and 74 during the years 1948 to 1961 revealed a statistically highly ; significant excess of deaths from leukemia, multiple myeloma, and aplastic anemia. ; That this excess is due to radiation exposure (or to some factor acting in a ; similar manner), rather than

E. B. Lewis

1963-01-01

289

Novel Human Erythrovirus Associated with Transient Aplastic Anemia  

Microsoft Academic Search

Erythrovirus (formerly parvovirus) B19 causes a wide range of diseases in humans, including anemia due to aplastic crisis. Diagnosis of B19 infection relies on serology and the detection of viral DNA by PCR. These techniques are usually thought to detect all erythrovirus field isolates, since the B19 genome is known to undergo few genetic variations. We have detected an erythrovirus

QUANG TRI NGUYEN; CHRISTOPHE SIFER; VERONIQUE SCHNEIDER; XAVIER ALLAUME; ANNABELLE SERVANT; FRANCOISE BERNAUDIN; VERONIQUE AUGUSTE; ANTOINE GARBARG-CHENON; UFR Saint-Antoine

1999-01-01

290

Cationic amphiphilic non-hemolytic polyacrylates with superior antibacterial activity.  

PubMed

Acrylic copolymers with appropriate compositions of counits having cationic charge with 2-carbon and 6-carbon spacer arms can show superior antibacterial activities with concomitant very low hemolytic effect. These amphiphilic copolymers represent one of the most promising synthetic polymer antibacterial systems reported. PMID:24854366

Punia, Ashish; He, Edward; Lee, Kevin; Banerjee, Probal; Yang, Nan-Loh

2014-07-01

291

Identification of a hemolytic activity elaborated by Haemophilus ducreyi.  

PubMed Central

Haemophilus ducreyi is the causative agent of the sexually transmitted disease chancroid. We have identified a hemolytic activity expressed by H. ducreyi. This activity is most readily detected when horse erythrocytes are used as a target; however, low levels of activity can be detected with sheep, human, or rabbit erythrocyte targets. The activity is heat labile and protease sensitive. PMID:8005696

Palmer, K L; Grass, S; Munson, R S

1994-01-01

292

Classification of anemia for gastroenterologists  

PubMed Central

Most anemia is related to the digestive system by dietary deficiency, malabsorption, or chronic bleeding. We review the World Health Organization definition of anemia, its morphological classification (microcytic, macrocytic and normocytic) and pathogenic classification (regenerative and hypo regenerative), and integration of these classifications. Interpretation of laboratory tests is included, from the simplest (blood count, routine biochemistry) to the more specific (iron metabolism, vitamin B12, folic acid, reticulocytes, erythropoietin, bone marrow examination and Schilling test). In the text and various algorithms, we propose a hierarchical and logical way to reach a diagnosis as quickly as possible, by properly managing the medical interview, physical examination, appropriate laboratory tests, bone marrow examination, and other complementary tests. The prevalence is emphasized in all sections so that the gastroenterologist can direct the diagnosis to the most common diseases, although the tables also include rare diseases. Digestive diseases potentially causing anemia have been studied in preference, but other causes of anemia have been included in the text and tables. Primitive hematological diseases that cause anemia are only listed, but are not discussed in depth. The last section is dedicated to simplifying all items discussed above, using practical rules to guide diagnosis and medical care with the greatest economy of resources and time. PMID:19787825

Moreno Chulilla, Jose Antonio; Romero Col醩, Maria Soledad; Guti閞rez Mart韓, Mart韓

2009-01-01

293

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

PubMed Central

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

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

2013-01-01

294

Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997-2003.  

PubMed

Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in childhood. In its typical presentation, it is preceded by an episode of diarrhea mostly due to Shiga-toxin-producing Escherichia coli. There is important geographical variation of many aspects of this syndrome. Nationwide data on childhood HUS in Switzerland have not been available so far. In a prospective national study through the Swiss Pediatric Surveillance Unit 114 cases (median age 21 months, 50% boys) were reported between April 1997 and March 2003 by 38 pediatric units (annual incidence 1.42 per 10(5) children < or =16 years). Shiga-toxin-producing E. coli were isolated in 32 (60%) of tested stool samples, serotype O157:H7 in eight. Sixteen children presented with only minimal renal involvement, including three with underlying urinary tract infection. Six patients presented with atypical hemolytic-uremic syndrome, and six with HUS due to invasive Streptococcus pneumoniae infection. Mortality was 5.3%, including two out of six children with S. pneumoniae infection. The severity of thrombocytopenia and the presence of central nervous system involvement significantly correlated with mortality. In conclusion, childhood HUS is not rare in Switzerland. Contrasting other countries, E. coli O157:H7 play only a minor role in the etiology. Incomplete manifestation is not uncommon. PMID:19830454

Schifferli, Alexandra; von Vigier, Rodo O; Fontana, Matteo; Spart, Giuseppina; Schmid, Hans; Bianchetti, Mario G; Rudin, Christoph

2010-05-01

295

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 ...

296

How Is Sickle Cell Anemia Treated?  

MedlinePLUS

... from the NHLBI on Twitter. How Is Sickle Cell Anemia Treated? Sickle cell anemia has no widely ... severity of the disease. Blood and Marrow Stem Cell Transplant A blood and marrow stem cell transplant ...

297

[Immune pathophysiology of refractory anemias].  

PubMed

Among different immune pathophysiologies of anemia, those of bone marrow failure syndromes such as aplastic anemia and myelodysplastic syndrome are most difficult to understand. An increase in the proportion of glycosylphosphatidyl-inositol anchored protein-deficient cells has been identified as the best marker for the presence of immune pathophysiology in this elusive syndrome. The significance of detecting small populations of such paroxysmal nocturnal hemoglobinuria (PNH)-type cells was substantiated by a recent observation that PNH-type cells arose from a donor-derived hematopoietic stem cell with a PIG-A mutation in an aplastic anemia patient with late graft failure which responded well to immunosuppressive therapy. Identification of auto-antigens capable of inducing cytotoxic T cells against hematopoietic stem cells is necessary to prove the escape of PIG-A mutant clone from the immune system attack using animal models. PMID:18326316

Nakao, Shinji

2008-03-01

298

Anemia in children with chronic kidney disease  

PubMed Central

Anemia is a common feature of chronic kidney disease, but the management of anemia in children is complex. Erythropoietin and supplemental iron are used to maintain hemoglobin levels. The National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) clinical practice guidelines for the management of anemia specifically in children were recently published. Pediatric nephrologists are encouraged to use current clinical practice guidelines and best evidence in conjunction with their clinical experience to optimally manage patients with anemia. PMID:17245602

Koshy, Susan M.

2007-01-01

299

(Inborn anemias of mice): Terminal progress report  

SciTech Connect

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.

Bernstein, S.E.

1987-01-01

300

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

PubMed

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

Guagnozzi, Danila; Lucendo, Alfredo J

2014-04-01

301

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

PubMed Central

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

Guagnozzi, Danila; Lucendo, Alfredo J

2014-01-01

302

Delayed Anemia after Treatment with Injectable Artesunate in the Democratic Republic of the Congo: A Manageable Issue  

PubMed Central

Cases of delayed hemolytic anemia have been described after treatment with injectable artesunate, the current World Health Organization (WHO)杛ecommended first-line drug for the treatment of severe malaria. A total of 350 patients (215 [61.4%] < 5 years of age and 135 [38.6%] ? 5 years of age) were followed-up after treatment with injectable artesunate for severe malaria in hospitals and health centers of the Democratic Republic of the Congo. Complete series of hemoglobin (Hb) measurements were available for 201 patients. A decrease in Hb levels between 2 and 5 g/dL was detected in 23 (11.4%) patients during the follow-up period. For five patients, Hb levels decreased below 5 g/dL during at least one follow-up visit. All cases of delayed anemia were clinically manageable and resolved within one month. PMID:25071004

Burri, Christian; Ferrari, Giovanfrancesco; Ntuku, Henry Maggi; Kitoto, Antoinette Tshefu; Duparc, Stephan; Hugo, Pierre; Mitembo, Didier Kalemwa; Lengeler, Christian

2014-01-01

303

Delayed anemia after treatment with injectable artesunate in the Democratic Republic of the Congo: a manageable issue.  

PubMed

Cases of delayed hemolytic anemia have been described after treatment with injectable artesunate, the current World Health Organization (WHO)-recommended first-line drug for the treatment of severe malaria. A total of 350 patients (215 [61.4%] < 5 years of age and 135 [38.6%] ? 5 years of age) were followed-up after treatment with injectable artesunate for severe malaria in hospitals and health centers of the Democratic Republic of the Congo. Complete series of hemoglobin (Hb) measurements were available for 201 patients. A decrease in Hb levels between 2 and 5 g/dL was detected in 23 (11.4%) patients during the follow-up period. For five patients, Hb levels decreased below 5 g/dL during at least one follow-up visit. All cases of delayed anemia were clinically manageable and resolved within one month. PMID:25071004

Burri, Christian; Ferrari, Giovanfrancesco; Ntuku, Henry Maggi; Kitoto, Antoinette Tshefu; Duparc, Stephan; Hugo, Pierre; Mitembo, Didier Kalemwa; Lengeler, Christian

2014-10-01

304

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

PubMed Central

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

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

2014-01-01

305

Perioperative anemia management in colorectal cancer patients: A pragmatic approach  

PubMed Central

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

Mu駉z, Manuel; G髆ez-Ram韗ez, Susana; Mart韓-Monta馿z, Elisa; Auerbach, Michael

2014-01-01

306

Cooley's Anemia: A Psychosocial Directory.  

ERIC Educational Resources Information Center

The directory is intended to aid patients and their families who are coping with the genetic disorder of Cooley's anemia. A brief review of the disease covers background, genetics, symptoms, effect on the patient, treatment, and current research. The next section looks at psychosocial needs at various times (time of diagnosis, infancy and toddler

National Center for Education in Maternal and Child Health, Washington, DC.

307

Harderoporphyria due to homozygosity for coproporphyrinogen oxidase missense mutation H327R.  

PubMed

Hereditary coproporphyria (HCP) is an autosomal dominant acute hepatic porphyria due to the half-normal activity of the heme biosynthetic enzyme, coproporphyrinogen oxidase (CPOX). The enzyme catalyzes the step-wise oxidative decarboxylation of the heme precursor, coproporphyrinogen III, to protoporphyrinogen IX via a tricarboxylic intermediate, harderoporphyrinogen. In autosomal dominant HCP, the deficient enzymatic activity results primarily in the accumulation of coproporphyrin III. To date, only a few homozygous HCP patients have been described, most having Harderoporphyria, a rare variant due to specific CPOX mutations that alter enzyme residues D400-K404, most patients described to date having at least one K404E allele. Here, we describe a Turkish male infant, the product of a consanguineous union, who presented with the Harderoporphyria phenotype including neonatal hyperbilirubinemia, hemolytic anemia, hepatosplenomegaly, and skin lesions when exposed to UV light. He was homoallelic for the CPOX missense mutation, c.980A>G (p.H327R), and had massively increased urinary uroporphyrins I and III (9,250 and 2,910 ?M, respectively) and coproporphyrins I and III (895 and 19,400 ?M, respectively). The patient expired at 5 months of age from an apparent acute neurologic porphyric attack. Structural studies predicted that p.H327R interacts with residue W399 in the CPOX active site, thereby accounting for the Harderoporphyria phenotype. PMID:21103937

Hasanoglu, Alev; Balwani, Manisha; Kasapkara, Ci?dem S; Ezg, Fatih S; Okur, Ilyas; T黰er, Leyla; Cakmak, Alpay; Nazarenko, Irina; Yu, Chunli; Clavero, Sonia; Bishop, David F; Desnick, Robert J

2011-02-01

308

HARDEROPORPHYRIA DUE TO HOMOZYGOSITY FOR COPROPORPHYRINOGEN OXIDASE MISSENSE MUTATION H327R  

PubMed Central

Summary Hereditary coproporphyria (HCP) is an autosomal dominant acute hepatic porphyria due to the half-normal activity of the heme biosynthetic enzyme, coproporphyrinogen oxidase (CPOX). The enzyme catalyzes the step-wise oxidative decarboxylation of the heme precursor, coproporphyrinogen III to protoporphyrinogen IX via a tricarboxylic intermediate, harderoporphyrinogen. In autosomal dominant HCP, the deficient enzymatic activity results primarily in the accumulation of coproporphyrin III. To date, only a few homozygous HCP patients have been described, most having Harderoporphyria, a rare variant due to specific CPOX mutations that alter enzyme residues D400-K404, most patients described to date having at least one K404E allele. Here, we describe a Turkish male infant, the product of a consanguineous union, who presented with the Harderoporphyria phenotype including neonatal hyperbilirubinemia, hemolytic anemia, hepatosplenomegaly, and skin lesions when exposed to UV light. He was homoallelic for the CPOX missense mutation, c.980A>G (p.H327R), and had massively increased urinary uroporphyrins I and III (9250 and 2910 ?M, respectively) and coproporphyrins I and III (895 and 19,400 ?M, respectively). The patient expired at five months of age from an apparent acute neurologic porphyric attack. Structural studies predicted that p.H327R interacts with residue W399 in the CPOX active site, thereby accounting for the Harderoporphyria phenotype. PMID:21103937

Hasanoglu, A; Balwani, M; Kasapkara, 荢; Ezg, FS; Okur, I; T黰er, L; 莂kmak, A; Nazarenko, I; Yu, C; Clavero, S; Bishop, DF; Desnick, RJ

2011-01-01

309

[Anemia as a surgical risk factor].  

PubMed

Perioperative anemia is common in patients undergoing surgery and is associated with increased morbidity and mortality and a decreased quality of life. The main causes of anemia in the perioperative context are iron deficiency and chronic inflammation. Anemia can be aggravated by blood loss during surgery, and is most commonly treated with allogeneic transfusion. Moreover, blood transfusions are not without risks, once again increasing patient morbidity and mortality. Given these concerns, we propose to review the pathophysiology of anemia in the surgical environment, as well as its treatment through the consumption of iron-rich foods and by oral or intravenous iron therapy (iron sucrose and iron carboxymaltose). In chronic inflammatory anemia, we use erythropoiesis-stimulating agents (erythropoietin alpha) and, in cases of mixed anemia, the combination of both treatments. The objective is always to reduce the need for perioperative transfusions and speed the recovery from postoperative anemia, as well as decrease the patient morbidity and mortality rate. PMID:24314568

Moral Garc韆, Victoria; 羘geles Gil de Bernab Sala, M; Nadia Diana, Kinast; Pericas, Bartolom Cantallops; Nebot, Alexia Galindo

2013-07-01

310

Effect of toluene on the hemolytic resistance of rat erythrocytes.  

PubMed

The effect of toluene on rat erythrocyte hemolytic resistance was studied both in vitro and in vivo. In vitro, toluene at concentrations up to 1000 ppm showed a marked antihemolytic effect, the maximum being at 300 ppm. Above 1000 ppm, an increase in the hypotonic hemolysis was seen. The antihemolytic effect of toluene was temperature-dependent. Elevation of temperature diminished the ability of toluene to protect erythrocytes. In the in vivo experiments, when the rats breathed 2000 ppm of toluene in an inhalation chamber for 7 days and for 21 days (6 h/day), the antihemolytic effect of toluene was evident. Our results demonstrate that toluene, at moderate concentrations, increases the hemolytic resistance of rat erythrocytes in hypotonic media both in vitro and in inhalation exposures in vivo. PMID:6623518

Korpela, M; Vapaatalo, H; T鋒ti, H

1983-07-01

311

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

SciTech Connect

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.

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

1984-10-01

312

Antibacterial and Hemolytic Activities of Quaternary Pyridinium  

E-print Network

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

313

Hematological parameters and prevalence of anemia among free-living elderly in south Brazil  

PubMed Central

Objective The aims of this study were to analyze the hematological parameters, the prevalence of anemia and the association between anemia and socioeconomic conditions in an elderly community-based population. Methods A population-based study was performed as part of the Multidimensional Study of the Elderly in Porto Alegre, Brazil (EMIPOA). An initial total of 1058 community residents aged 60 years and older were interviewed. Of these, 392 agreed to have a physical evaluation and a blood sample was taken from each. The hematological parameters analyzed in the blood samples included the hemoglobin concentration, mean cell volume (MCV), mean corpuscular hemoglobin concentration (MCHC) and red cell distribution width (RDW). The association between the variables and the diagnosis of anemia was assessed using the chi-squared test and a multiple logistic regression model. Results The overall prevalence of anemia was 12.8%. Anemia was present in 13.7% of women and in 10.4% of men. Normocytic normochromic anemia without anisocytosis was the most common type of anemia (46%). The assessment of erythrocyte morphology showed significant differences between anemic and non-anemic individuals (microcytosis = 12% vs. 1.5%, hypochromia = 40% vs. 8.8%, and anisocytosis = 26% vs. 7%). In the analysis of socioeconomic conditions, significant differences were found in respect to age and race. Conclusion The prevalence of anemia increases with age and is associated with race, microcytosis, hypochromia and anisocytosis. Anemia is not a condition that should be associated only with the aging process, as it may be due to pathological conditions that occur most frequently in this age group. As a result, a diagnosis of anemia warrants adequate clinical attention. PMID:23741189

Sgnaolin, Vanessa; Engroff, Paula; Ely, Lu韘a Scheer; Schneider, Rodolfo Herberto; Schwanke, Carla Helena Augustin; Gomes, Irenio; Morrone, Fernanda Bueno; de Carli, Geraldo Attilio

2013-01-01

314

Hemolytic venoms from marine cnidarian jellyfish - an overview.  

PubMed

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

Mariottini, Gian Luigi

2014-01-01

315

Cloning and characterization of hemolytic genes from Helicobacter pylori.  

PubMed Central

Strains of Helicobacter pylori, the bacterium associated with gastritis, peptic ulcer disease, and gastric cancer in humans, express different degrees of hemolysis on agar containing erythrocytes (RBC). Here we report the isolation and characterization of six recombinant clones from a genomic library of H. pylori ATCC 49503 that confer on Escherichia coli the ability to lyse sheep RBC. DNA hybridizations indicated no sequence homology among these hemolytic clones. Hybridization mapping of them to an ordered H. pylori cosmid library identified their separate chromosomal locations. One clone hybridized to two regions separated by approximately 200 kb. The specificities of the hemolytic activities of these clones were tested with RBC from humans, monkeys, cattle, horses, guinea pigs, rabbits, and chickens as well as with RBC from sheep. One clone conferred the ability to lyse RBC from five species, a second clone allowed the lysis of RBC from four of these species, three other clones allowed the lysis of RBC from three of these species, and the sixth clone allowed the lysis of RBC from just two species. We propose that some or all of the genes that confer these various hemolytic activities contribute to pathogen-host tissue interactions and that the different specificities seen here are important for H. pylori infections of humans of different genotypes or disease states. PMID:7591069

Drazek, E S; Dubois, A; Holmes, R K; Kersulyte, D; Akopyants, N S; Berg, D E; Warren, R L

1995-01-01

316

Candida Species Exhibit Differential In Vitro Hemolytic Activities  

PubMed Central

A total of 80 Candida isolates representing 14 species were examined for their respective responses to an in vitro hemolytic test. A modification of a previously described plate assay system where the yeasts are incubated on glucose (3%)-enriched sheep blood agar in a carbon dioxide (5%)-rich environment for 48 h was used to evaluate the hemolytic activity. A group of eight Candida species which included Candida albicans (15 isolates), C. dubliniensis (2), C. kefyr (2), C. krusei (4), C. zeylanoides (1), C. glabrata (34), C. tropicalis (5), and C. lusitaniae (2) demonstrated both alpha and beta hemolysis at 48 h postinoculation. Only alpha hemolysis was detectable in four Candida species, viz., C. famata (3), C. guilliermondii (4), C. rugosa (1), and C. utilis (1), while C. parapsilosis (5) and C. pelliculosa (1) failed to demonstrate any hemolytic activity after incubation for 48 h or longer. This is the first study to demonstrate the variable expression profiles of hemolysins by different Candida species. PMID:11474025

Luo, Gang; Samaranayake, Lakshman P.; Yau, Joyce Y. Y.

2001-01-01

317

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

PubMed Central

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 12 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

Alasfar, Sami; Alachkar, Nada

2014-01-01

318

Individualized treatment for iron deficiency anemia in adults  

PubMed Central

Iron deficiency is one of the most common disorders affecting mankind, and iron deficiency anemia continues to represent a major public health problem worldwide. It is especially common among women of childbearing age due to pregnancy and menstrual blood loss. Additional patient groups include those with other sources of blood loss, malnutrition or gut malabsorption. Iron deficiency anemia remains quite prevalent despite the widespread ability to diagnose the disease and availability of medicinal iron preparations. Therefore, new approaches are needed to effectively manage these patient populations. In this review, the diagnosis and treatment of iron deficiency anemia are discussed with emphasis placed upon consideration of patient specific features. It is proposed that all patients participate in their own care by helping their physician to identify a tolerable daily iron dose, formulation, and schedule. Dosing cycles are recommended for iron replacement based upon the tolerated daily dose and the total iron deficit. Each cycle consists of 5000mg of oral elemental iron ingested over at least one month with appropriate follow-up. This approach should assist physicians and their patients with the implementation of individualized treatment strategies for patients with iron deficiency anemia. PMID:18954837

Alleyne, Michael; Horne, McDonald K.; Miller, Jeffery L.

2008-01-01

319

Decreased hematocrit-to-viscosity ratio and increased lactate dehydrogenase level in patients with sickle cell anemia and recurrent leg ulcers.  

PubMed

Leg ulcer is a disabling complication in patients with sickle cell anemia (SCA) but the exact pathophysiological mechanisms are unknown. The aim of this study was to identify the hematological and hemorheological alterations associated with recurrent leg ulcers. Sixty-two SCA patients who never experienced leg ulcers (ULC-) and 13 SCA patients with a positive history of recurrent leg ulcers (ULC+)--with no leg ulcers at the time of the study--were recruited. All patients were in steady state condition. Blood was sampled to perform hematological, biochemical (hemolytic markers) and hemorheological analyses (blood viscosity, red blood cell deformability and aggregation properties). The hematocrit-to-viscosity ratio (HVR), which reflects the red blood cell oxygen transport efficiency, was calculated for each subject. Patients from the ULC+ group were older than patients from the ULC- group. Anemia (red blood cell count, hematocrit and hemoglobin levels) was more pronounced in the ULC+ group. Lactate dehydrogenase level was higher in the ULC+ group than in the ULC- group. Neither blood viscosity, nor RBC aggregation properties differed between the two groups. HVR was lower and RBC deformability tended to be reduced in the ULC+ group. Our study confirmed increased hemolytic rate and anemia in SCA patients with leg ulcers recurrence. Furthermore, our data suggest that although systemic blood viscosity is not a major factor involved in the pathophysiology of this complication, decreased red blood cell oxygen transport efficiency (i.e., low hematocrit/viscosity ratio) may play a role. PMID:24223994

Connes, Philippe; Lamarre, Yann; Hardy-Dessources, Marie-Dominique; Lemonne, Nathalie; Waltz, Xavier; Mougenel, Dani鑜e; Mukisi-Mukaza, Martin; Lalanne-Mistrih, Marie-Laure; Tarer, Vanessa; Tressi鑢es, Benoit; Etienne-Julan, Maryse; Romana, Marc

2013-01-01

320

Decreased Hematocrit-To-Viscosity Ratio and Increased Lactate Dehydrogenase Level in Patients with Sickle Cell Anemia and Recurrent Leg Ulcers  

PubMed Central

Leg ulcer is a disabling complication in patients with sickle cell anemia (SCA) but the exact pathophysiological mechanisms are unknown. The aim of this study was to identify the hematological and hemorheological alterations associated with recurrent leg ulcers. Sixty-two SCA patients who never experienced leg ulcers (ULC-) and 13 SCA patients with a positive history of recurrent leg ulcers (ULC+) - but with no leg ulcers at the time of the study were recruited. All patients were in steady state condition. Blood was sampled to perform hematological, biochemical (hemolytic markers) and hemorheological analyses (blood viscosity, red blood cell deformability and aggregation properties). The hematocrit-to-viscosity ratio (HVR), which reflects the red blood cell oxygen transport efficiency, was calculated for each subject. Patients from the ULC+ group were older than patients from the ULC- group. Anemia (red blood cell count, hematocrit and hemoglobin levels) was more pronounced in the ULC+ group. Lactate dehydrogenase level was higher in the ULC+ group than in the ULC- group. Neither blood viscosity, nor RBC aggregation properties differed between the two groups. HVR was lower and RBC deformability tended to be reduced in the ULC+ group. Our study confirmed increased hemolytic rate and anemia in SCA patients with leg ulcers recurrence. Furthermore, our data suggest that although systemic blood viscosity is not a major factor involved in the pathophysiology of this complication, decreased red blood cell oxygen transport efficiency (i.e., low hematocrit/viscosity ratio) may play a role. PMID:24223994

Connes, Philippe; Lamarre, Yann; Hardy-Dessources, Marie-Dominique; Lemonne, Nathalie; Waltz, Xavier; Mougenel, Dani鑜e; Mukisi-Mukaza, Martin; Lalanne-Mistrih, Marie-Laure; Tarer, Vanessa; Tressi鑢es, Benoit; Etienne-Julan, Maryse; Romana, Marc

2013-01-01

321

Incidence and risk factors of aplastic anemia in Latin American countries: the LATIN case-control study  

PubMed Central

Background Associations between aplastic anemia and numerous drugs, pesticides and chemicals have been reported. However, at least 50% of the etiology of aplastic anemia remains unexplained. Design and Methods This was a case-control, multicenter, multinational study, designed to identify risk factors for agranulocytosis and aplastic anemia. The cases were patients with diagnosis of aplastic anemia confirmed through biopsy or bone marrow aspiration, selected through an active search of clinical laboratories, hematology clinics and medical records. The controls did not have either aplastic anemia or chronic diseases. A total of 224 patients with aplastic anemia were included in the study, each case was paired with four controls, according to sex, age group, and hospital where the case was first seen. Information was collected on demographic data, medical history, laboratory tests, medications, and other potential risk factors prior to diagnosis. Results The incidence of aplastic anemia was 1.6 cases per million per year. Higher rates of benzene exposure (?30 exposures per year) were associated with a greater risk of aplastic anemia (odds ratio, OR: 4.2; 95% confidence interval, CI: 1.829.82). Individuals exposed to chloramphenicol in the previous year had an adjusted OR for aplastic anemia of 8.7 (CI: 0.8787.93) and those exposed to azithromycin had an adjusted OR of 11.02 (CI 1.14108.02). Conclusions The incidence of aplastic anemia in Latin America countries is low. Although the research study centers had a high coverage of health services, the underreporting of cases of aplastic anemia in selected regions can be discussed. Frequent exposure to benzene-based products increases the risk for aplastic anemia. Few associations with specific drugs were found, and it is likely that some of these were due to chance alone. PMID:19734415

Maluf, Eliane; Hamerschlak, Nelson; Cavalcanti, Alexandre Biasi; J鷑ior, 羖varo Avezum; Eluf-Neto, Jos; Falc鉶, Roberto Passetto; Lorand-Metze, Irene G.; Goldenberg, Daniel; Santana, C閦ar Leite; de Oliveira Werneck Rodrigues, Daniela; da Motta Passos, Leny Nascimento; Rosenfeld, Luis Gast鉶 Mange; Pitta, Marimilia; Loggetto, Sandra; Feitosa Ribeiro, Andreza A.; Velloso, Elvira Deolinda; Kondo, Andrea Tiemi; de Miranda Coelho, Erika Oliveira; Pint鉶, Maria Carolina Tostes; de Souza, H閘io Moraes; Borbolla, Jos Rafael; Pasquini, Ricardo

2009-01-01

322

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

PubMed

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爕ears 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

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

2014-12-01

323

Pagophagia in iron deficiency anemia.  

PubMed

The relationship between pagophagia (ice pica) and iron deficiency anemia was studied. All 81 patients with iron deficiency anemia defined as hemoglobin <12.0 g/dl and ferritin level <12 ng/ml were interviewed about their habits of eating ice or other non-food substances. Pagophagia was defined as compulsive and repeated ingestion of at least one tray of ice or ice eating which was relieved after iron administration. Pagophagia was present in 13 patients (16.0%). All patients who received oral iron were periodically assessed employing a questionnaire on pagophagia and laboratory data. Iron therapy can cure the pagophagia earlier than hemoglobin recovery and repair of tissue iron deficiency. Although the pathogenesis of pagophagia is unclear, a biochemical approach involving the central nervous system might elucidate the mechanism underlying these abnormal behaviors. PMID:24850454

Uchida, Tatsumi; Kawati, Yasunori

2014-04-01

324

Fanconi anemia - learning from children  

PubMed Central

Fanconi Anemia (FA) is a rare autosomic recessive and X-linked disease with chromosomal instability after exposure to crosslinking agents as the hallmark. Clinical features of FA are somatic malformations, progressive bone marrow failure and cancer proneness, however there is wide clinical heterogeneity. The symptom most frequently and early associated with morbidity and mortality is progressive pancytopenia in the first decade of life although acute myelogenous leukemia (AML) or myelodysplastic syndrome (MDS) can appear before aplastic anemia. Squamous cell carcinoma (SCC) of the head-neck, intestinal or genital tract has a very high incidence in FA and can appear at young age. This paper will focus on treatment of bone marrow failure in FA. PMID:22053284

Svahn, Johanna; Dufour, Carlo

2011-01-01

325

Anemia Among Hospitalized Children at a Multispecialty Hospital, Bangalore (Karnataka), India  

PubMed Central

Background: Due to the limited availability of data related to anemia in hospitalized children, this research was conducted to study the occurrence, morphological patterns, distribution in different age groups, sex, and severity of anemia among children aged 6 months-12 years. Setting: Inpatients in department of pediatrics at a multispecialty hospital, Bangalore. Study Design: Descriptive cross sectional study from Oct, 2011 to Sep, 2012. Materials and Methods: Ethical clearance was obtained from the ethical committee of the hospital as per 1964 Declaration of Helsinki. Unrestricted random sampling method was used to select the study group consisting of 882 children between the age of 6 months and 12 years. After obtaining the consent, data were obtained and statistically analyzed using statistical tools like mean, median, standard deviation, and Chi-square test. Results: Out of 882 children selected, 642 (72.79%) were anemic, out of which a majority of 629 (98%) children suffered from nonhemoglobinopathies and a meagre 13 (2%) suffered from hemoglobinopathies. Children in the age group of 6 months-1 year were most affected with nonhemoglobinopathies (33%). Moderate degree of anemia (hemoglobin = 7-9.9 g/dL) was the commonest grade of anemia (80%), while microcytic hypochromic anemia was commonest morphological type of anemia (48%). Among hemoglobinopathies, thalassemia major was the most common (69%, that is 9 out of 13 patients). Conclusion: The occurrence of anemia among children aged between 6 months and 12 years is high and nonhemoglobinopathies predominate over the hemoglobinopathies. PMID:24791237

Saba, Firdos; Poornima, Siddaraju; Balaji, Pishey Ashwathnarayan Rao; Varne, Smitha Ranoji Rao; Jayashree, Krishnamurthy

2014-01-01

326

MCPIP1 Deficiency in Mice Results in Severe Anemia Related to Autoimmune Mechanisms  

PubMed Central

Autoimmune gastritis is an organ-specific autoimmune disease of the stomach associated with pernicious anemia. The previous work from us and other groups identified MCPIP1 as an essential factor controlling inflammation and immune homeostasis. MCPIP1-/- developed severeanemia. However, the mechanisms underlying this phenotype remain unclear. In the present study, we found that MCPIP1 deficiency in mice resulted in severe anemia related to autoimmune mechanisms. Although MCPIP1 deficiency did not affect erythropoiesis per se, the erythropoiesis in MCPIP1-/- bone marrow erythroblasts was significantly attenuated due to iron and vitamin B12 (VB12) deficiency, which was mainly resulted from autoimmunity-associated gastritis and parietal cell loss. Consistently, exogenous supplement of iron and VB12 greatly improved the anemia phenotype of MCPIP1-/- mice. Finally, we have evidence suggesting that autoimmune hemolysis may also contribute to anemia phenotype of MCPIP1-/- mice. Taken together, our study suggests that MCPIP1 deficiency in mice leads to the development of autoimmune gastritis and pernicious anemia. Thus, MCPIP1-/- mice may be a good mouse model for investigating the pathogenesis of pernicious anemia and testing the efficacy of some potential drugs for treatment of this disease. PMID:24324805

Zhou, Zhou; Miao, Ruidong; Huang, Shengping; Elder, Brandon; Quinn, Tim; Papasian, Christopher J.; Zhang, Jifeng; Fan, Daping; Chen, Y. Eugene; Fu, Mingui

2013-01-01

327

Strong association between a new marker of hemolysis and glomerulopathy in sickle cell anemia.  

PubMed

To perform a precise evaluation of the hemolytic status of patients with sickle cell anemia (SCA), advanced red blood cell parameters provided by the last generation analyzers were investigated in a series of SCA patients. The search for precise markers of hemolysis was performed to identify if patients so exposed develop organic complications related to a postulated hemolysis-linked endothelial dysfunction. Red blood cell survival was evaluated by the ratio between mature red blood cell (RBC) and reticulocyte (RET) hemoglobin (RBC-Hb/RET-Hb). In comparison with serum lactate dehydrogenase (LDH) and total bilirubin, the log (RBC-Hb/RET-Hb) was identified as the most discriminant hematological parameter to evaluate hemolysis. Furthermore, by combining this parameter with LDH, we defined a composite variable, which we called CVar, that is highly correlated with albuminuria and might constitute a powerful new marker of risk for this complication. PMID:20833087

Maier-Redelsperger, Micheline; L関y, Pierre; Lionnet, Fran鏾is; Stankovic, Katia; Haymann, Jean-Philippe; Lef鑦re, Guillaume; Avellino, Virginie; Perol, Jean-Pierre; Girot, Robert; Elion, Jacques

2010-12-15

328

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

PubMed Central

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爃ours 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

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

329

[Anemia in chronic kidney disease].  

PubMed

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

Amador-Medina, Lauro Fabi醤

2014-01-01

330

Synthesis, characterization, in vitro anti-proliferative and hemolytic activity of hydroxyapatite.  

PubMed

Hydroxyapatite (Ca10(PO4)6(OH)2, HAP) nanoparticles are widely used in several biomedical applications due to its compositional similarities to bone mineral, excellent biocompatibility and bioactivity, osteoconductivity. In this present investigation, HAP nanoparticles synthesized by precipitation technique using calcium nitrate and di-ammonium phosphate. The crystalline nature and the functional group analysis are confirmed using X-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR) and Fourier transform Raman spectroscopy (FT-Raman) respectively. The morphological observations are ascertained from field emission electron scanning electron microscope (FE-SEM) and transmission electron microscope (TEM). In vitro anti-proliferative and hemolytic activities are carried out on the synthesized HAP samples and the studies reveals that HAP have mild activity against erythrocytes. PMID:24650878

Palanivelu, R; Ruban Kumar, A

2014-06-01

331

Diagnosis of Atypical Hemolytic Uremic Syndrome and Response to Eculizumab Therapy  

PubMed Central

Atypical hemolytic uremic syndrome (aHUS) has a high mortality rate if not detected and treated early. While in the past, it was associated with renal failure in children, today, it has become increasingly identified among adults. Due to recent advances in the pathogenesis of aHUS and other major thrombotic microangiopathies (TMA), diagnosing it has become a lot easier. We present a case of a 62-year-old man who was initially thought to have thrombotic thrombocytopenic purpura (TTP), but after further evaluation was diagnosed with aHUS. We will discuss how to distinguish aHUS from other major TMA and the role of eculizumab in the management of aHUS. PMID:25285252

Mathew, Jacob J; Denunzio, Troy M; Carmichael, Mark G

2014-01-01

332

What Are the Signs and Symptoms of Sickle Cell Anemia?  

MedlinePLUS

... What Are the Signs and Symptoms of Sickle Cell Anemia? The signs and symptoms of sickle cell ... who have sickle cell anemia. Complications of Sickle Cell Anemia Sickle cell crises can affect many parts ...

333

Genetics Home Reference: Thiamine-responsive megaloblastic anemia syndrome  

MedlinePLUS

... PubMed Recent literature OMIM Genetic disorder catalog Conditions > Thiamine-responsive megaloblastic anemia syndrome On this page: Description ... names Glossary definitions Reviewed February 2009 What is thiamine-responsive megaloblastic anemia syndrome? Thiamine-responsive megaloblastic anemia ...

334

Autoimmune hemolytic anemia as a paraneoplastic phenomenon in solid tumors: A critical analysis of 52 cases reported in the literature  

Microsoft Academic Search

\\u000a Zusammenfassung牋Die autoimmunh鋗olytische An鋗ie ist ein wohlbekanntes paraneoplastisches Ph鋘omen bei lymphoproliferativen Erkrankungen.\\u000a Es gibt aber auch eine Reihe von Fallberichten einer Assoziation einer autoimmunh鋗olytischen An鋗ie mit soliden Tumoren.\\u000a Wir haben 52 publizierte F鋖le einer solchen Assoziation analysiert. Eine autoimmunh鋗olytische An鋗ie kann vor klinischer\\u000a Diagnose, gleichzeitig mit der klinischen Diagnose des Tumors oder nach Beendigung der Tumorbehandlung, entweder als Zeichen\\u000a eines Rezidivs

Joe Puthenparambil; Klaus Lechner; Gabriela Kornek

2010-01-01

335

Structural and functional effects of hereditary hemolytic anemia-associated point mutations in the alpha spectrin tetramer site  

PubMed Central

The most common hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP) mutations are ?-spectrin missense mutations in the dimer-tetramer self-association site. In this study, we systematically compared structural and functional properties of the 14 known HE/HPP mutations located in the ?-spectrin tetramer binding site. All mutant ?-spectrin recombinant peptides were well folded, stable structures, with only the R34W mutant exhibiting a slight structural destabilization. In contrast, binding affinities measured by isothermal titration calorimetry were greatly variable, ranging from no detectable binding observed for I24S, R28C, R28H, R28S, and R45S to approximately wild-type binding for R34W and K48R. Binding affinities for the other 7 mutants were reduced by approximately 10- to 100-fold relative to wild-type binding. Some sites, such as R28, were hot spots that were very sensitive to even relatively conservative substitutions, whereas other sites were only moderately perturbed by nonconservative substitutions. The R34W and K48R mutations were particularly intriguing mutations that apparently either destabilize tetramers through mechanisms not probed by the univalent tetramer binding assay or represent polymorphisms rather than the pathogenic mutations responsible for observed clinical symptoms. All ?0 HE/HPP mutations studied here appear to exert their destabilizing effects through molecular recognition rather than structural mechanisms. PMID:18218854

Gaetani, Massimiliano; Mootien, Sara; Harper, Sandra; Gallagher, Patrick G.

2008-01-01

336

Iron deficiency anemia: focus on infectious diseases in lesser developed countries.  

PubMed

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

Shaw, Julia G; Friedman, Jennifer F

2011-01-01

337

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

PubMed Central

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 pH7.0-7.4; 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爉M. Furthermore, incubation at 100癈 for 30 to 60爉inutes 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

2013-01-01

338

Humanized Mouse Model of Cooley's Anemia*S?  

PubMed Central

A novel humanized mouse model of Cooley's Anemia (CA) was generated by targeted gene replacement in embryonic stem (ES) cells. Because the mouse does not have a true fetal hemoglobin, a delayed switching human ? to ?0 globin gene cassette (??0) was inserted directly into the murine ? globin locus replacing both adult mouse ? globin genes. The inserted human ?0 globin allele has a mutation in the splice donor site that produces the same aberrant transcripts in mice as described in human cells. No functional human ? globin polypeptide chains are produced. Heterozygous ??0 mice suffer from microcytic anemia. Unlike previously described animal models of ? thalassemia major, homozygous ??0 mice switch from mouse embryonic globin chains to human fetal ? globin during fetal life. When bred with human ? globin knockin mice, homozygous CA mice survive solely upon human fetal hemoglobin at birth. This preclinical animal model of CA can be utilized to study the regulation of globin gene expression, synthesis, and switching; the reactivation of human fetal globin gene expression; and the testing of genetic and cell-based therapies for the correction of thalassemia. PMID:19098001

Huo, Yongliang; McConnell, Sean C.; Liu, Shan-Run; Yang, Rui; Zhang, Ting-Ting; Sun, Chiao-Wang; Wu, Li-Chen; Ryan, Thomas M.

2009-01-01

339

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

PubMed Central

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.

Ishnava, Kalpesh B; Shah, Pankit P.

2014-01-01

340

Isolation of a Variant of Subtilosin A with Hemolytic Activity?  

PubMed Central

Bacillus subtilis produces an anionic bacteriocin called subtilosin A that possesses antibacterial activity against certain gram-positive bacteria. In this study, we uncovered a hemolytic mutant of B. subtilis that produces an altered form of subtilosin A. The mutant bacteriocin, named subtilosin A1, has a replacement of threonine at position 6 with isoleucine. In addition to the hemolytic activity, subtilosin A1 was found to exhibit enhanced antimicrobial activity against specific bacterial strains. The B. subtilis albB mutant that does not produce a putative immunity peptide was more sensitive to both subtilosin A and subtilosin A1. A spontaneous suppressor mutation of albB that restored resistance to subtilosin A and subtilosin A1 was obtained. The sbr (subtilosin resistance) mutation conferring the resistance is not linked to the sboA-alb locus. The sbr mutation does not increase the resistance of B. subtilis to other cell envelope-targeted antimicrobial agents, indicating that the mutation specifically confers the resistance to subtilosins. The findings suggest possible bioengineering approaches for obtaining anionic bacteriocins with enhanced and/or altered bactericidal activity. Furthermore, future identification of the subtilosin-resistant mutation could provide insights into the mechanism of subtilosin A activity. PMID:19633086

Huang, Tai; Geng, Hao; Miyyapuram, Venugopal R.; Sit, Clarissa S.; Vederas, John C.; Nakano, Michiko M.

2009-01-01

341

Experimental studies on the hemolytic-uremic syndrome.  

PubMed

Ultrastructural studies of blood cells during the acute stage of the hemolytic-uremic syndrome (HUS) revealed striking, but transient, changes in erythrocyte structure. These included membrane disruption, vacuolar degeneration, and Heinz body formation. There was also evidence of platelet injury, and there were peculiar tactile interactions between histiocytes and impaired red cells. These changes disappeared as the patients recovered. These changes were considered to be important in the pathogenesis of the hemolytic and thrombolytic features of HUS, and studies were directed at reproducing them in vitro and in vivo. Treatment of red cells with purified clostridial phospholipase C induced changes in red cells and platelets that were comparable to those encountered in HUS. Rats infused with phospholipase C developed hemolysis, thrombocytopenia, and hemoglobinuria. Their kidneys did not, however, reveal glomerular alterations similar to those seen in patients with HUS. It is proposed that HUS in some cases might be initiated by a nonspecific infectious injury to the intestinal mucosa thereby allowing increased absorption of toxins derived from indigenous gut flora and that these toxins could be responsible for the hemolysis, thrombolysis, and even the renal injury. PMID:3974783

Bolande, R P; Kaplan, B S

1985-01-01

342

Pathology Case Study: Macrocytic Anemia  

NSDL National Science Digital Library

This is a case study presented by the University of Pittsburgh Department of Pathology in which an older man suffering from chronic bronchitis and macrocytic anemia also developed persistent flu symptoms. Visitors view the microscopic and gross descriptions, including images, and have the opportunity to diagnose the patient. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to use to introduce or test student learning in hematopathology.

Bahler, David

343

Severe isoniazid related sideroblastic anemia  

PubMed Central

Isoniazid induced sideroblastic anemia is a rare event. We report case of a 45 year old Caucasian women with development of severe anaemia 4 month after introduction of Isoniazid as part of Tuberculosis treatment. While haemoglobin fell to 47 g/L and erythrocyte count to 1.5 G/L, reticulocytes were very low (reticulocyte production index of 0.48), but bone marrow aspirate showed an accelerated erythropoiesis with ringsideroblasts. Anaemia rapidly resolved after cessation of Isoniazid. We postulate an Isoniazid induced inhibition of the ?-Amino-levulinat-synthase resulting in marked depletion of heam synthesis. PMID:22184524

Piso, Rein Jan; Kriz, Kveti; Desax, Marie-Claire

2011-01-01

344

The Student with Sickle Cell Anemia.  

ERIC Educational Resources Information Center

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)

Tetrault, Sylvia M.

1981-01-01

345

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2010 CFR

...Animal Products 2 2010-01-01 2010-01-01 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...

2010-01-01

346

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2014 CFR

...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...

2014-01-01

347

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2011 CFR

...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...

2011-01-01

348

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2012 CFR

...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...

2012-01-01

349

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2013 CFR

...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...

2013-01-01

350

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

PubMed Central

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抯 condition, especially if it is associated with acute pain and severe anemia. Considering the patient抯 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燿ays 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

2014-01-01

351

Homozygosity mapping of Fanconi anemia  

SciTech Connect

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.

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

1994-09-01

352

Fanconi anemia in Ashkenazi Jews.  

PubMed

Fanconi anemia (FA) should be included among the genetic diseases that occur at high frequency in the Ashkenazi Jewish population. FA exhibits extensive genetic heterogeneity; there are currently 11 complementation groups reported, and 8 (i.e., FANCA, FANCC, FANCD1/BRCA2, FANCD2, FANCE, FANCF, FANCG, and FANCL) genes have been isolated. While patients may be from widely diverse ethnic groups, a single mutation in complementation group FA-C, c.711 + 4A > T (commonly known as IVS4 + 4A > T prior to current nomenclature rules) is unique to FA patients of Ashkenazi Jewish ancestry, and has a carrier frequency of greater than 1/100 in this population. In addition, a mutation (c.65G > A) in FANCA (FA-A is the most common complementation group in non-Jewish patients) and the mutation c.6174delT in FANCD1/BRCA2 are also unique to the Ashkenazi Jewish population. Therefore, the study of Fanconi anemia can lend insight into the types of cancer-predisposing genetic diseases specific to the Ashkenazi. PMID:15516848

Kutler, David I; Auerbach, Arleen D

2004-01-01

353

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

PubMed

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

Kent, Alexandra J; Blackwell, Victoria J; Travis, Simon P L

2012-07-01

354

Anemia  

MedlinePLUS

... Being African-American Being a woman HIV disease progression is about 5 times more common in people ... the immune system. They appear to cause faster progression of HIV disease and to increase the risk ...

355

Anemia  

MedlinePLUS

... Division of Intramural Research Research Resources Scientific Reports Technology Transfer Clinical Trials What Are Clinical Trials? Children & ... Overview Blood is made up of many parts, including red blood cells, white blood cells, platelets (PLATE- ...

356

Anemia  

MedlinePLUS

... foods prevent your body from absorbing iron, including coffee, tea, egg whites, milk, fiber and soy protein. ... absorption. These include milk, soy protein, egg yolks, coffee and tea. Avoid these foods when eating iron- ...

357

Anemia  

MedlinePLUS

... the body over time. This condition is called hemochromatosis (HEE-moh-kroh-muh-TOH-suhss). The extra ... problems can cause iron overload. Most people with hemochromatosis inherit it from their parents. It is one ...

358

REACTIONS OF RABBITS TO NON-HEMOLYTIC STREPTOCOCCI  

PubMed Central

1. Accompanying and following the evolution of a secondary reaction in the skin of rabbits after inoculation with suitable doses of certain non-hemolytic streptococci there quickly develops a general state of hypersensitiveness or allergy towards these streptococci. 2. This state is made evident by ophthalmic reactions following corneal inoculations, by much increased reactivity of the skin following intracutaneous reinoculations, and by lethal reactions, resembling tuberculin shock, following intravenous inoculations. 3. In a given hypersensitive rabbit there is a rough parallelism in the intensities of these different kinds of reactions. 4. This type of hypersensitiveness or bacterial allergy does not follow primary intravenous inoculation of rabbits with comparable doses of the streptococci employed. 5. As the development of this type of hypersensitiveness or bacterial allergy seems to accompany the production of focal lesions of a certain intensity, it is probable that in these foci are produced the substances or conditions which lead to this type of bacterial allergy. PMID:19869568

Derick, C. L.; Swift, Homer F.

1929-01-01

359

Green light phototherapy in newborn infants with ABO hemolytic disease.  

PubMed

The efficacy of fluorescent green light phototherapy was compared with that of blue light phototherapy in the treatment of full-term infants with hemolytic disease and jaundice caused by ABO incompatibility. The efficacy of the treatment was expressed as actual (milligrams per hour) and quantum (milligrams per hour per square centimeter per megawatt) efficiency, taking into account the differential emission of energy from the green versus the blue fluorescent tubes. No statistically significant difference in the rate of serum bilirubin photodegradation was found between the two groups after treatment for 84.6 +/- 14.1 hours versus 81.5 +/- 14.2 hours with the green and the blue phototherapy, respectively. These results, coupled with the known effects of the blue light on the genetic apparatus of mammalian cells, support the application of the green light phototherapy for the treatment of neonatal hyperbilirubinemia caused by ABO incompatibility. PMID:3681556

Ayyash, H; Hadjigeorgiou, E; Sofatzis, J; Chatziioannou, A; Nicolopoulos, D; Sideris, E

1987-12-01

360

Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.  

PubMed

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

Schrezenmeier, Hubert; K鰎per, Sixten; H鯿hsmann, Britta

2015-02-01

361

Do cerebral blood flow velocities change in iron deficiency anemia?  

PubMed

Infants with iron deficiency had lower scores when tested for mental and motor development than their peers with better iron status. The aim of this study was to examine cerebral blood flow velocity in infants with iron deficiency anemia. Thirty-six infants (27 male, 9 female) with iron deficiency anemia, aged 6 to 36 months were divided into 2 groups according to the hemoglobin (Hb) values [group 1 (n=23) Hb<10 g/dL and group 2 (n=13) 11 >Hb> or =10 g/dL]. In anterior and middle cerebral arteries only end-diastolic velocity (EDV) was increased in group 1 as compared with group 2 (P=0.05 and P=0.016, respectively), whereas in posterior cerebral artery both EDV and peak-systolic velocity were different between the groups (P=0.024 and P=0.004). Both peak-systolic velocity and EDV showed significant correlation with Hb level in the posterior cerebral artery (r=-0.38, P=0.023 and r=-0.35, P=0.037) but not in the anterior and middle cerebral arteries. Increased cerebral blood flow velocities in children with lower Hb values may be due to increased cardiac output, decreased vascular resistivity caused by anemia. PMID:17984692

Aliefendioglu, Didem; Yilmaz, Sevda; Misirlioglu, Emine Dibek; Saygi, Semra; Ozdogan, Selver; Kocak, Ulker

2007-11-01

362

Protrusio acetabuli in sickle-cell anemia  

SciTech Connect

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.

Martinez, S.; Apple, J.S.; Baber, C.; Putman, C.E.; Rosse, W.F.

1984-04-01

363

High anemia prevalence in western China.  

PubMed

We assessed the prevalence of anemia among schoolchildren in western China as determined by seven cross-sectional surveys involving 12,768 children aged 8-12 years. Subjects were selected randomly from 283 primary schools in 41 economically disadvantaged counties of Ningxia, Qinghai, Shaanxi and Sichuan Provinces. Data were collected through questionnaires and hemoglobin levels were measured. The anemia prevalence was 34% using the WHO hemoglobin cutoff of < 120 g/l. Boarding students and girls were more likely to be anemic. The prevalence of anemia in schoolchildren was high. Iron deficiency is a significant nutrition issue in China. PMID:22299447

Luo, Renfu; Wang, Xiaobing; Zhang, Linxiu; Liu, Chengfang; Shi, Yaojiang; Miller, Grant; Rozelle, Scott; Yu, Elaine; Martorell, Reynaldo

2011-09-01

364

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

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.

Modi, Gaurang; Shah, Sandip; Panchal, Harsha; Patel, Apurva; Uparkar, Urmila; Anand, Asha; Parikh, Sonia; Patel, Kinnari; Shah, Kamlesh; Revannasiddaiah, Swaroop

2015-01-01

365

Retrospective cohort study of 205 cases with congenital dyserythropoietic anemia type II: definition of clinical and molecular spectrum and identification of new diagnostic scores.  

PubMed

Congenital Dyserythropoietic Anemia II (CDA II) is a rare hyporegenerative anemia of variable degree, whose causative gene is SEC23B. More than 60 causative mutations in 142 independent pedigrees have been described so far. However, the prevalence of the CDA II is probably underestimated, since its clinical spectrum was not yet well-defined and thus it is often misdiagnosed with more frequent clinically-related anemias. This study represents the first meta-analysis on clinical and molecular spectrum of CDA II from the largest cohort of cases ever described. We characterized 41 new cases and 18 mutations not yet associated to CDA II, thus expanding the global series to 205 cases (172 unrelated) and the total number of causative variants to 84. The 68.3% of patients are included in our International Registry of CDA II (Napoli, Italy). A genotype-phenotype correlation in three genotypic groups of patients was assessed. To quantify the degree of severity in each patient, a method based on ranking score was performed. We introduced a clinical index to easily discriminate patients with a well-compensated hemolytic anemia from those with ineffective erythropoiesis. Finally, the worldwide geographical distribution of SEC23B alleles highlighted the presence of multiple founder effects in different areas of the world. PMID:25044164

Russo, Roberta; Gambale, Antonella; Langella, Concetta; Andolfo, Immacolata; Unal, Sule; Iolascon, Achille

2014-10-01

366

Ecological Determinants of Anemia in Pregnant Women Living in Freetown: Urban Western Area, Sierra Leone.  

E-print Network

??Introduction Anemia prevalence in pregnancy ranges from 51%-60% globally. Genetic disorders, infectious diseases, reproductive factors, nutritional deficiencies, and poverty can affect anemia status. Anemia can (more)

M'Cormack, Fredanna A. D.

2008-01-01

367

Anemia - Multiple Languages: MedlinePlus  

MedlinePLUS

... on this page, please enable JavaScript. Anemia - Multiple Languages Arabic (???????) Bosnian (Bosanski) Chinese - Simplified (????) French ( ... Characters not displaying correctly on this page? See language display issues . Return to the MedlinePlus Health Information ...

368

Special Issues for People with Aplastic Anemia  

MedlinePLUS

... result in injuries and bleeding, such as contact sports. Pregnancy and Aplastic Anemia Pregnancy is possible for ... Strong Personal Support Team Role Models Share Your Success Story Communities of Hope Community Calendar Stories of ...

369

Avoiding Anemia: Boost Your Red Blood Cells  

MedlinePLUS

... our exit disclaimer . Subscribe Avoiding Anemia Boost Your Red Blood Cells If you抮e feeling constantly exhausted ... when your body doesn抰 have enough healthy red blood cells. You may either have too few ...

370

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 ...

371

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 .

372

Genetics Home Reference: Congenital dyserythropoietic anemia  

MedlinePLUS

... names Glossary definitions Reviewed July 2009 What is CDA? Congenital dyserythropoietic anemia (CDA) is an inherited blood ... three major types of CDA. How common is CDA? Several hundred cases of CDA have been reported ...

373

Characteristics of sickle cell anemia in Yemen.  

PubMed

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

Al-Ghazaly, Jameel; Al-Dubai, Waled; Abdullah, Munasser; Al-Mahagri, Altaf; Al-Gharasi, Leila

2013-01-01

374

Border between aplastic anemia and myelodysplastic syndrome.  

PubMed

Distinguishing between acquired aplastic anemia (AA) and myelodysplastic syndrome (MDS) with a low blast cell percentage is often difficult and problematic, as both diseases are syndromes primarily defined by morphological findings, and their diagnostic criteria do not necessarily reflect the pathophysiology of their bone marrow (BM) failure. As a result, many patients with benign BM failure that should be managed as AA are diagnosed as having MDS, due to the absence of BM hypocellularity and the presence of dysplastic signs in the BM, and are treated inappropriately with toxic therapies, such as hypomethylating agents, and stem cell transplantation from unrelated donors. BM failure syndromes need to be managed in ways appropriate to their pathophysiology, which is more accurately determined by using markers such as the presence of glycosylphosphatidylinositol-anchored protein-deficient cells and HLA-A lacking leukocytes. We recently found that plasma thromobopoietin level is one of the most useful markers for distinguishing benign and pre-leukemic BM failure syndromes. PMID:23613266

Yamazaki, Hirohito; Nakao, Shinji

2013-05-01

375

Successful Treatment of Severe Anemia using Erythropoietin in a Jehovah Witness with Non-Hodgkin Lymphoma  

PubMed Central

Blood transfusion many times works in a life-saving way when a patient is facing a critical situation. However, some patients, such as Jehovah抯 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抯 Witnesses have accepted rHuEPO as a treatment option to maintain and enhance erythropoiesis. This paper reports the case of a 57-year-old Jehovah抯 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

Agapidou, Alexandra; Vakalopoulou, Sofia; Papadopoulou, Theodosia; Chadjiaggelidou, Christina; Garypidou, Vasileia

2014-01-01

376

[Anemia impact on treatments of cervical carcinomas].  

PubMed

During the treatments of carcinomas of the cervix, anemia is relatively frequent and its origin is complex combining often hemorrhage, iron deprivation, inflammatory reactions and infection. The frequency of the primary anemia (hemoglobin level<12 g/dl) is correlated with clinical stage and varies from one publication to another, mainly from 25% for stage I, to 33% for stage II and can approach 40% for stage III. Anemia is correlated with patient survival and it appears to be one of the most powerful prognostic factor after clinical stage and tumor size. Anemia is a bad prognostic factor related to stage and tumor size but it has not been proven to be an independent factor. Anemia increases hypoxia of cervix carcinomas, which is an independent prognostic factor for patients N0. Moreover, we know that the oxygenation of these tumors is correlated with hemoglobin levels. The normalization of Hb levels by transfusion could certainly modify the prognosis of patients anemic before treatment, or of those becoming anemic during radiotherapy treatment. For smokers, anemia is certainly more important that we can appreciate from the Hb levels only, by the presence of carboxyhemoglobin. Concomitant chemotherapies with cisplatin compounds are actually standards and they can largely increase the risk of inducing anemia, therefore more than 50% of patients will experiment it during their different treatments. Transfusion is recommended by the SOR (Standards Options and Recommendations of the F閐閞ation nationale des centres de lutte contre le cancer) under 10 g/dl. The use of erythropoietin is a therapeutic option for Hb levels between 10 and 12 g/dl and strongly recommended after a Hb normalization by blood transfusion. For 70% of patients who respond to erythropoietin, a better control of the Hb level is obtained. The impact of this anemia on quality of life and treatments compliance justifies the use of erythropoietin, especially in cancers for which treatments induce a deep fatigue and a very bad tolerance, which could be a limiting factor. PMID:15820436

Marchal, C; Rangeard, L; Brunaud, C

2005-03-01

377

The Fanconi anemia pathway and ubiquitin  

Microsoft Academic Search

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

C閘ine Jacquemont; Toshiyasu Taniguchi

2007-01-01

378

The Fanconi Anemia Pathway and Ubiquitin  

Microsoft Academic Search

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

Toshiyasu Taniguchi; C閘ine Jacquemont

2007-01-01

379

Characterization of the hemolytic activity of Staphylococcus aureus strains associated with toxic shock syndrome.  

PubMed Central

The hemolytic activity of 32 vaginal isolates of Staphylococcus aureus from patients with typical toxic shock syndrome (TSS) was contrasted with that of 50 vaginal isolates from patients without TSS, using a standardized inoculum (10(5) CFU) on 5% sheep blood agar after 48 h of incubation under 30% CO2. Additionally, 7 nongenital isolates from patients with nonmenstrual TSS and 57 strains of nongenital control isolates were included for comparison. Vaginal TSS strains were significantly less hemolytic than non-TSS S. aureus strains of either genital (P less than 0.001) or nongenital (P less than 0.01) origin. Vaginal TSS S. aureus strains were also less hemolytic than were nongenital TSS S. aureus strains (P less than 0.02). This reduced hemolytic activity of genital TSS S. aureus strains may provide a useful marker for screening and further delineation of toxigenic S. aureus associated with menstrually related TSS. PMID:6841587

Chow, A W; Gribble, M J; Bartlett, K H

1983-01-01

380

Family structure and child anemia in Mexico.  

PubMed

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

Schmeer, Kammi K

2013-10-01

381

The evolution of hemolytic saponin content in wild and cultivated Alfalfa ( Medicago sativa , Fabaceae)  

Microsoft Academic Search

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.

Ernest Small; Marian Jurzysta; Constance Nozzolillo

1990-01-01

382

Effects of co-existing microalgae and grazers on the production of hemolytic toxins in Karenia mikimotoi  

NASA Astrophysics Data System (ADS)

Karenia mikimotoi (Miyake & Kominami ex Oda) Hansen & Moestrup is associated with harmful algal blooms in temperate and subtropical zones of the world. The hemolytic substances produced by K. mikimotoi are thought to cause mortality in fishes and invertebrates. We evaluated the composition of the hemolytic toxin produced by K. mikimotoi cultured in the laboratory using thin-layer chromatography. In addition, we evaluated the effect of co-occuring algae ( Prorocentrum donghaiense and Alexandrium tamarense) and the cladoceran grazer Moina mongolica on hemolytic toxin production in K. mikimotoi. The hemolytic toxins from K. mikimotoi were a mixture of 2 liposaccharides and 1 lipid. Waterborne clues from P. donghaiense and A. tamarense inhibited the growth of K. mikimotoi but increased the production of hemolytic toxins. Conversely, K. mikimotoi strongly inhibited the growth of caged P. donghaiense and A. tamarense. In addition, the ingestion of K. mikimotoi by M. mongolica induced the production of hemolytic toxins in K. mikimotoi. Taken together, our results suggest that the presence of other microalgae and grazers may be as important as environmental factors for controlling the production of hemolytic substances. K. mikimotoi secreted allelochemicals other than unstable fatty acids with hemolytic activity. The production of hemolytic toxins in dinoflagellates was not only dependent on resource availability, but also on the risk of predation. Hemolytic toxins likely play an important role as chemical deterrents secreted by K. mikimotoi.

Yang, Weidong; Zhang, Naisheng; Cui, Weimin; Xu, Yanyan; Li, Hongye; Liu, Jiesheng

2011-11-01

383

A Novel Atypical Hemolytic Uremic Syndrome-Associated Hybrid CFHR1/CFH Gene Encoding a Fusion Protein That Antagonizes Factor H-Dependent Complement Regulation.  

PubMed

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

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

384

Serogroup-Specific Bacterial Engineered Glycoproteins as Novel Antigenic Targets for Diagnosis of Shiga Toxin-Producing-Escherichia coli-Associated Hemolytic-Uremic Syndrome.  

PubMed

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

Melli, Luciano J; Ciocchini, Andr閟 E; Caillava, Ana J; Vozza, Nicol醩; Chinen, Isabel; Rivas, Marta; Feldman, Mario F; Ugalde, Juan E; Comerci, Diego J

2015-02-01

385

Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome  

PubMed Central

Background and objectives: Neurologic involvement is the most threatening complication of diarrhea-associated hemolytic uremic syndrome (D+HUS). Design, setting, participants, & measurements: We report a retrospective multicenter series of 52 patients with severe initial neurologic involvement that occurred in the course of D+HUS. Results: Verotoxigenic Escherichia coli infection was documented in 24. All except two patients had acute renal failure that required peritoneal dialysis, hemodialysis, or both techniques. A first group of eight patients remained with normal consciousness; five of them had protracted seizures. A second group of 23 patients had stuporous coma; five of these had protracted severe seizures, and 18 had a neurologic defect including pyramidal syndrome, hemiplegia or hemiparesia, and extrapyramidal syndrome. A third group of 21 patients had severe coma. Plasma exchanges were undertaken in 25 patients, 11 of whom were treated within 24 hours after the first neurologic sign; four died, two survived with severe sequelae, and five were alive without neurologic defect. Magnetic resonance imaging (MRI) for 29 patients showed that (1) every structure of the central nervous system was susceptible to involvement; (2) no correlation seemed to exist between special profile of localization on early MRI and the final prognosis; and (3) MRI did not exhibit any focal lesions in three patients. The overall prognosis of the series was marked by the death of nine patients and severe sequelae in 13. Conclusions: Neurologic involvement is associated with a severe renal disease but does not lead systematically to death or severe disability. PMID:20498239

Kwon, Th閞閟a; Elmaleh, Monique; Charbit, Marina; Launay, Emma Allain; Harambat, J閞鬽e; Brun, Muriel; Ranchin, Bruno; Bandin, Flavio; Cloarec, Sylvie; Bourdat-Michel, Guylhene; Pi鑤rement, Christine; Champion, G閞ard; Ulinski, Tim; Desch阯es, Georges

2010-01-01

386

Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.  

PubMed

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

Feng, Shuju; Eyler, Stephen J; Zhang, Yuzhou; Maga, Tara; Nester, Carla M; Kroll, Michael H; Smith, Richard J; Afshar-Kharghan, Vahid

2013-08-22

387

Management of hemolytic-uremic syndrome in children  

PubMed Central

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

Grisaru, Silviu

2014-01-01

388

Hemolytic Behavior of Staphylococci Isolated from Cows' Milk*  

PubMed Central

Patterns of hemolysis produced by staphylococci isolated from milk were investigated on media prepared with bovine, sheep, horse, and rabbit erythrocytes under a variety of cultural circumstances. Hemolytic patterns were sharper and easier to interpret on erythrocyte agar plates when incubated at 37癈 in an atmosphere containing 30% CO2. Alpha-hemolysin production was greatly enhanced in this environment and was not detected at times when cultures were grown in air only. This was also true of deltahemolysin but to a lesser extent. Very satisfactory identification of alpha, beta- and delta-hemolysins was obtained by streaking BEA plates with the unknown strains perpendicularly to a strain producing beta-hemolysin and incubating in 30% CO2. This procedure avoided use of erythrocytes of different species of origin and use of staphylococcal alpha-antitoxin. Prior action of the beta-hemolysin on bovine cells was found to completely inhibit hemolysis from alpha-hemolysin but the reverse was not true. A strain inhibiting beta-hemolysis in air failed to exhibit its antihemolytic properties for 24 hours when incubated in 30% CO2. Patterns of hemolysis within areas subject to multiple hemolysins were found to reflect the nature and relative strength of contributing hemolysins or antihemolysins and could be modified by the time sequence of exposure to certain hemolysins or antihemolysins. ImagesFig. 2. PMID:4223753

Jasper, D. E.; Jain, N. C.

1966-01-01

389

Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.  

PubMed

The differential diagnosis of thrombotic microangiopathy (TMA) has become clearer following the establishment of the relationships between (1) diarrhea-associated hemolytic uremic syndrome (HUS) and Shiga toxin-producing Escherichia coli-HUS (STEC-HUS), (2) a markedly reduced ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) level and typical thrombotic thrombocytopenic purpura (TTP), and (3) abnormalities in the complement regulatory system and atypical HUS (aHUS). These TMAs include typical TTP, other forms of TMA, STEC-HUS, and aHUS. The pathological mechanisms of TMA still overlap among several forms of TMA. With respect to the management of TMA, the use of plasma exchange (PE) for typical TTP, additional steroid therapy for TMA and rituximab for typical TTP with a high titer of the inhibitor of ADAMTS-13, as well as eculizumab for aHUS, have also been established. Although several issues remain in the pathophysiology and management of TMA, new findings will hopefully resolve these problems in the near future. PMID:25377323

Wada, Hideo; Matsumoto, Takeshi; Yamashita, Yoshiki

2014-11-01

390

Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations  

PubMed Central

Hemolytic uremic syndrome (HUS) is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and pathogenetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the complement proteins are clearly involved in all types of thrombotic microangiopathy: typical HUS, atypical HUS and thrombotic thrombocytopenic purpura (TTP). Furthermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic aspects of this rare disease, examining both 搕raditional therapy (including plasma therapy, kidney and kidney-liver transplantation) and 搉ew therapies. The latter include anti-Shiga-toxin antibodies and anti-C5 monoclonal antibody 揺culizumab. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases I and II. They include anti-C5 antibodies, which are more purified, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy. PMID:24255888

Salvadori, Maurizio; Bertoni, Elisabetta

2013-01-01

391

Recent improvement in outcome of unrelated donor transplantation for aplastic anemia  

Microsoft Academic Search

The aim was to determine whether outcome of unrelated donor transplantation for severe aplastic anemia has improved in recent years and whether this is due to patient selection or better transplant technology. We analyzed 498 patients transplanted during 19902005. By running univariate regression models dichotomizing year of transplantation we defined 1998 as the year of the most significant change in

R Viollier; G Soci; A Tichelli; A Bacigalupo; E T Korthof; J Marsh; J Cornish; P Ljungman; R Oneto; A N B閗醩sy; M Fuehrer; S Maury; H Schrezenmeier; M T van Lint; D Wojcik; A Locasciulli; J R Passweg; JR Passweg

2008-01-01

392

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

E-print Network

Anemia Gu铆a breve sobre la La anemia es un trastorno de la sangre. La sangre es un l铆quido esencial de anemia, como la anemia por deficien- cia de hierro, la anemia perniciosa, la anemia apl谩sica y la anemia hemol铆tica. Los distintos tipos de anemia tienen relaci贸n con diversas enfermedades y problemas de

Bandettini, Peter A.

393

Anemia and transfusion after subarachnoid hemorrhage.  

PubMed

Delayed cerebral ischemia after subarachnoid hemorrhage (SAH) may be affected by a number of factors, including cerebral blood flow and oxygen delivery. Anemia affects about half of patients with SAH and is associated with worse outcome. Anemia also may contribute to the development of or exacerbate delayed cerebral ischemia. This review was designed to examine the prevalence and impact of anemia in patients with SAH and to evaluate the effects of transfusion. A literature search was made to identify original research on anemia and transfusion in SAH patients. A total of 27 articles were identified that addressed the effects of red blood cell transfusion (RBCT) on brain physiology, anemia in SAH, and clinical management with RBCT or erythropoietin. Most studies provided retrospectively analyzed data of very low-quality according to the GRADE criteria. While RBCT can have beneficial effects on brain physiology, RBCT may be associated with medical complications, infection, vasospasm, and poor outcome after SAH. The effects may vary with disease severity or the presence of vasospasm, but it remains unclear whether RBCTs are a marker of disease severity or a cause of worse outcome. Erythropoietin data are limited. The literature review further suggests that the results of the Transfusion Requirements in Critical Care Trial and subsequent observational studies on RBCT in general critical care do not apply to SAH patients and that randomized trials to address the role of RBCT in SAH are required. PMID:21769459

Le Roux, Peter D

2011-09-01

394

What Are the Signs and Symptoms of Fanconi Anemia?  

MedlinePLUS

... What Are the Signs and Symptoms of Fanconi Anemia? Major Signs and Symptoms Your doctor may suspect ... sisters also should be tested for the disorder. Anemia The most common symptom of all types of ...

395

For Parents of Children with Diamond Blackfan Anemia  

MedlinePLUS

National Center on Birth Defects and Developmental Disabilities Division of Blood Disorders For Parents of Children with Diamond Blackfan Anemia Parenting Corner Q&A When your child is evaluated for Diamond Blackfan Anemia (DBA), the process can be stressful ...

396

Probing vasoocclusion phenomena in sickle cell anemia via mesoscopic simulations.  

PubMed

Vasoocclusion crisis is a key hallmark of sickle cell anemia. Although early studies suggest that this crisis is caused by blockage of a single elongated cell, recent experiments have revealed that vasoocclusion is a complex process triggered by adhesive interactions among different cell groups in multiple stages. However, the quantification of the biophysical characteristics of sickle cell anemia remains an open issue. Based on dissipative particle dynamics, we develop a multiscale model for the sickle red blood cells (SS-RBCs), accounting for diversity in both shapes and cell rigidities, to investigate the precise mechanism of vasoocclusion. First, we investigate the adhesive dynamics of a single SS-RBC in shear flow and static conditions, and find that the different cell groups (SS2: young-deformable SS-RBCs, ISCs: rigid-irreversible SS-RBCs) exhibit heterogeneous adhesive behavior due to the diverse cell morphologies and membrane rigidities. We quantify the observed adhesion behavior (in static conditions) in terms of a balance of free energies due to cell adhesion and deformation, and propose a power law that relates the free-energy increase as a function of the contact area. We further simulate postcapillary flow of SS-RBC suspensions with different cell fractions. The more adhesive SS2 cells interact with the vascular endothelium and trap ISC cells, resulting in vasoocclusion in vessels less than 12-14 ?m depending on the hematocrit. Under inflammation, adherent leukocytes may also trap ISC cells, resulting in vasoocclusion in even larger vessels. PMID:23798393

Lei, Huan; Karniadakis, George E

2013-07-01

397

Treatment of iron deficiency anemia associated with gastrointestinal tract diseases  

PubMed Central

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

Bayraktar, Ulas D; Bayraktar, Soley

2010-01-01

398

Secondary Hypoplastic Anemia in Patients with Familial Amyloidotic Polyneuropathy  

Microsoft Academic Search

The anemia of patients with familial amyloidotic polyneuropathy (FAP) was evaluated. Anemia was seen in 32 (91%) of the 35 FAP patients, more often with progression of the disease. The incidence of macrocytic hypochromic anemia was the most common type (40%). In 14 autopsied and 2 biopsied cases, no amyloid deposition was detected in the bone marrow. Thirteen (81%) of

Keiko Asahara; Yukio Ando; Yoshiya Tanaka; Shigehiro Yi; Taro Yamashita; Masayuki Ando

1993-01-01

399

Short Report Menstruation Does Not Cause Anemia: Endometrial Thickness  

E-print Network

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

Lummaa, Virpi

400

Genetic modulation of sickle cell anemia  

SciTech Connect

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.

Steinberg, M.H. [Univ. of Mississippi School of Medicine, Jackson, MS (United States)

1995-05-01

401

Do Blood Tests Cause Anemia in Hospitalized Patients?  

PubMed Central

OBJECTIVE To determine whether phlebotomy contributes to changes in hemoglobin and hematocrit levels in hospitalized general internal medicine patients. DESIGN Retrospective cohort study. SETTING General internal medicine inpatient service at a tertiary care hospital. PARTICIPANTS All adult patients discharged from the Toronto General Hospital's internal medicine service between January 1 and June 30, 2001. A total of 989 hospitalizations were reviewed and 404 hospitalizations were included in our analysis. MEASUREMENTS AND MAIN RESULTS Mean (SD) hemoglobin and hematocrit changes during hospitalization were 7.9 (12.6) g/L (P<.0001) and 2.1% (3.8%) (P<.0001), respectively. The mean (SD) volume of phlebotomy during hospital stay was 74.6 (52.1) mL. On univariate analysis, changes in hemoglobin and hematocrit were predicted by the volume of phlebotomy, length of hospital stay, admission hemoglobin/hematocrit value, age, Charlson comorbidity index, and admission intravascular volume status. The volume of phlebotomy remained a strong predictor of drop in hemoglobin and hematocrit after adjusting for other predictors using multivariate analysis (P<.0001). On average, every 100 mL of phlebotomy was associated with a decrease in hemoglobin and hematocrit of 7.0 g/L and 1.9%, respectively. CONCLUSIONS Phlebotomy is highly associated with changes in hemoglobin and hematocrit levels for patients admitted to an internal medicine service and can contribute to anemia. This anemia, in turn, may have significant consequences, especially for patients with cardiorespiratory diseases. Knowing the expected changes in hemoglobin and hematocrit due to diagnostic phlebotomy will help guide when to investigate anemia in hospitalized patients. PMID:15987327

Thavendiranathan, Paaladinesh; Bagai, Akshay; Ebidia, Albert; Detsky, Allan S; Choudhry, Niteesh K

2005-01-01

402

Gain-of-function Lyn induces anemia: appropriate Lyn activity is essential for normal erythropoiesis and Epo receptor signaling.  

PubMed

Lyn is involved in erythropoietin (Epo)-receptor signaling and erythroid homeostasis. Downstream pathways influenced following Lyn activation and their significance to erythropoiesis remain unclear. To address this, we assessed a gain-of-function Lyn mutation (Lyn(up/up)) on erythropoiesis and Epo receptor signaling. Adult Lyn(up/up) mice were anemic, with dysmorphic red cells (spherocyte-like, acanthocytes) in their circulation, indicative of hemolytic anemia and resembling the human disorder chorea acanthocytosis. Heterozygous Lyn(+/up) mice became increasingly anemic with age, indicating that the mutation was dominant. In an attempt to overcome this anemia, extramedullary erythropoiesis was activated. As the mice aged, the levels of different immature erythroid populations changed, indicating compensatory mechanisms to produce more erythrocytes were dynamic. Changes in Epo signaling were observed in Lyn(+/up) erythroid cell lines and primary CD71(+) Lyn(up/up) erythroblasts, including significant alterations to the phosphorylation of Lyn, the Epo receptor, Janus kinase 2, Signal Transducer and Action of Transcription-5, GRB2-associated-binding protein-2, Akt, and Forkhead box O3. As a consequence of altered Lyn signaling, Lyn(+/up) cells remained viable in the absence of Epo but displayed delayed Epo-induced differentiation. These data demonstrate that Lyn gene dosage and activity are critical for normal erythropoiesis; constitutively active Lyn alters Epo signaling, which in turn produces erythroid defects. PMID:23692855

Slavova-Azmanova, Neli S; Kucera, Nicole; Satiaputra, Jiulia; Stone, Leah; Magno, Aaron; Maxwell, Mhairi J; Quilici, Cathy; Erber, Wendy; Klinken, S Peter; Hibbs, Margaret L; Ingley, Evan

2013-07-11

403

Equine infectious anemia and equine infectious anemia virus in 2013: a review.  

PubMed

A detailed description of equine infectious anemia virus and host responses to it are presented. Current control and eradication of the infection are discussed with suggestions for improvements to increase their effectiveness. PMID:24183747

Cook, R F; Leroux, C; Issel, C J

2013-11-29

404

Iron-deficiency anemia caused by a proton pump inhibitor.  

PubMed

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

Hashimoto, Rintaro; Matsuda, Tomoki; Chonan, Akimichi

2014-01-01

405

A short review of malabsorption and anemia.  

PubMed

Anemia is a frequent finding in most diseases which cause malabsorption. The most frequent etiology is the combination of iron and vitamin B12 deficiency. Celiac disease is frequently diagnosed in patients referred for evaluation of iron deficiency anemia (IDA), being reported in 1.8%-14.6% of patients. Therefore, duodenal biopsies should be taken during endoscopy if no obvious cause of iron deficiency (ID) can be found. Cobalamin deficiency occurs frequently among elderly patients, but it is often unrecognized because the clinical manifestations are subtle; it is caused primarily by food-cobalamin malabsorption and pernicious anemia. The classic treatment of cobalamin deficiency has been parenteral administration of the vitamin. Recent data suggest that alternative routes of cobalamin administration (oral and nasal) may be useful in some cases. Anemia is a frequent complication of gastrectomy, and has been often described after bariatric surgery. It has been shown that banding procedures which maintain digestive continuity with the antrum and duodenum are associated with low rates of ID. Helicobacter pylori (H. pylori) infection may be considered as a risk factor for IDA, mainly in groups with high demands for iron, such as some children and adolescents. Further controlled trials are needed before making solid recommendations about H. pylori eradication in these cases. PMID:19787827

Fern醤dez-Ba馻res, Fernando; Monz髇, Helena; Forn, Montserrat

2009-10-01

406

Late clonal diseases of treated aplastic anemia  

Microsoft Academic Search

Recent progress in the treatment of aplastic anemia has dramatically changed the previously grim prognosis for these patients. Improvements in bone marrow transplantation and immunosuppression have increased the number of long-term survivors so that immediate survival is no longer the sole concern. Here, we review the major clinical studies and summarize recent analyses of risk factors for developing paroxysmal nocturnal

G閞ard Soci; Stephen Rosenfeld; Norbert Frickhofen; Eliane Gluckman; Andr Tichelli

2000-01-01

407

Alloantibodies to a paternally derived RBC KEL antigen lead to hemolytic disease of the fetus/newborn in a murine model  

PubMed Central

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

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

408

Intravenous Immunoglobulin G Treatment in ABO Hemolytic Disease of the Newborn, is it Myth or Real?  

PubMed

Intravenous Immunoglobulin G (IVIG) therapy has been used as a component of the treatment of hemolytic disease of the newborn. There is still no consensus on its use in ABO hemolytic disease of the newborn routinely. The aim of this study is to determine whether administration of IVIG to newborns with ABO incompatibility is necessary. One hundred and seventeen patients with ABO hemolytic disease and positive Coombs test were enrolled into the study. The subjects were healthy except jaundice. Infants were divided into two groups: Group I (n=71) received one dose of IVIG (1爂/kg) and LED phototherapy whereas Group II (n=46) received only LED phototherapy. One patient received erythrocyte transfusion in Group I, no exchange transfusion was performed in both groups. Mean duration of phototherapy was 3.1牨1.3燿ays in Group I and 2.27牨0.7燿ays in Group II (p<0.05). Mean duration of hospital stay was 5.34牨2.2燿ays in Group I and 3.53牨1.3燿ays in Group II (p<0.05). Mean duration of phototherapy was 4.0牨1.5燿ays and 2.73牨1.1燿ays in double and single doses of IVIG respectively, and this was statistically significant (p<0.05). IVIG therapy didn't decrease neither phototherapy nor hospitalization duration in infants with ABO hemolytic disease. Meticulus follow-up of infants with ABO hemolytic disease and LED phototherapy decreases morbidity. IVIG failed to show preventing hemolysis in ABO hemolytic disease. PMID:24554813

Beken, Serdar; Hirfanoglu, Ibrahim; Turkyilmaz, Canan; Altuntas, Nilgun; Unal, Sezin; Turan, Ozden; Onal, Esra; Ergenekon, Ebru; Koc, Esin; Atalay, Yildiz

2014-03-01

409

Prevalence of pernicious anemia in patients with macrocytic anemia and low serum B12  

PubMed Central

Objective: The current research evaluated the prevalence of pernicious anemia (PA) in patients with macrocytic anemia (high MCV) and low serum B12 in Riyadh. Methods: Blood testing was done in 77 patients (males: 45.5%, females: 54.5%) with macrocytic anemia; 84 patients; (males: 23.8%, females: 76.2%) with low serum B12 and 30 healthy subjects. Complete blood count, differential count, folic acid, vitamin B12, intrinsic factor, gastric parietal cell antibodies and holotranscobalamin II were assessed. Results: A total of five subjects from 161 patients had PA; three of these patients had macrocyticanemia (3.90%) and two patients had low serum B12 (2.38%). Significant differences (p<0.05) in some hematological, immunological, biochemical parameters were found in subjects with macrocytic anemia and low serum B12 compared to controls. Conclusions: Pernicious anemia in patients with macrocytic anemia and low serum B12 was for the selected sample size can be assumed to be uncommon in Riyadh, Saudi Arabia.

AA, Abdulmanea; AH, Alsaeed; AP, Shaik; FH, AlGahtani

2014-01-01

410

21 CFR 500.27 - Methylene blue-containing drugs for use in animals.  

Code of Federal Regulations, 2013 CFR

...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...

2013-04-01

411

21 CFR 500.27 - Methylene blue-containing drugs for use in animals.  

Code of Federal Regulations, 2014 CFR

...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...

2014-04-01

412

21 CFR 500.27 - Methylene blue-containing drugs for use in animals.  

Code of Federal Regulations, 2011 CFR

...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...

2011-04-01

413

21 CFR 500.27 - Methylene blue-containing drugs for use in animals.  

Code of Federal Regulations, 2012 CFR

...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...

2012-04-01

414

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 ...

415

CD34+ gene expression profiling of individual children with very severe aplastic anemia indicates a pathogenic role of integrin receptors and the proapoptotic death ligand TRAIL  

PubMed Central

Background Very severe aplastic anemia is characterized by a hypoplastic bone marrow due to destruction of CD34+ stem cells by autoreactive T cells. Investigation of the pathomechanism by patient-specific gene expression analysis of the attacked stem cells has previously been impractical because of the scarcity of these cells at diagnosis. Design and Methods Employing unbiased RNA amplification, patient-specific gene expression profiling was carried out for CD34+ cells from patients newly diagnosed with very severe aplastic anemia (n=13), refractory anemia (n=8) and healthy controls (n=10). These data were compared to profiles of myelodysplastic disease (n=55), including refractory anemia (n=18). To identify possible targets of autoimmune attack, presence of autoreactive antibodies was tested in pre-therapeutic sera of patients with very severe aplastic anemia (n=19). Results CD34+ gene expression profiling distinguished between healthy controls, children with aplastic or refractory anemia and clonal disease. Interferon stimulated genes such as the apoptosis inducing death ligand TRAIL were strongly up-regulated in CD34+ cells of patients with aplastic anemia, in particular in patients responding to immunosuppressive treatment. In contrast, mRNA expression of integrin GPVI and the integrin complexes GPIa/IIa, GPIIb/IIIa, GPIB/GPIX/GPV was significantly down-regulated and corresponding antibodies were detected in 7 of 11 profiled patients and in 11 of 19 aplastic anemia patients. Conclusions As a potential diagnostic tool, patient-specific gene expression profiling of CD34+ stem cells made it possible to make the difficult differential diagnosis of most patients with aplastic and refractory anemia. Profiling indicated a prognostic correlation of TRAIL expression and patient benefit from immunosuppressive therapy. Downregulation of integrin expression and concurrent presence of autoreactive anti-integrin-antibodies suggested a previously unrecognized pathological role of integrins in aplastic anemia. PMID:22315490

Fischer, Ute; Ruckert, Christian; Hubner, Bernd; Eckermann, Olaf; Binder, Vera; Bakchoul, Tamam; Schuster, Friedhelm R.; Merk, Sylvia; Klein, Hans-Ulrich; F黨rer, Monika; Dugas, Martin; Borkhardt, Arndt

2012-01-01

416

Acceleration of epithelial cell syndecan-1 shedding by anthrax hemolytic virulence factors  

PubMed Central

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

Popova, Taissia G; Millis, Bryan; Bradburne, Chris; Nazarenko, Svetlana; Bailey, Charles; Chandhoke, Vikas; Popov, Serguei G

2006-01-01

417

Cytomorphometric and cytomorphologic analysis of oral mucosa in children with sickle cell anemia  

PubMed Central

Background: Sickle cell anemia (SCA) is an autosomal recessive genetic disorder, characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs, consequence of vaso occlusive phenomenon and vasculopathy. Several forms of the chronic anemia, consequence of hemolysis, can be associated with oral epithelial cells changes. Exfoliative cytology can be used to detect real changes in the oral mucosa in SCA. Aims: To evaluate morphometric and morphological changes in oral epithelial cells by exfoliative cytology in children with SCA. Materials and Methods: Oral smears were collected from clinically normal-appearing mucosa by liquid-based exfoliative cytology in 20 SCA children (SCA group) and 20 healthy children (C group), matched for age and gender. The slides were prepared and stained by the Papanicolaou technique. Cell morphology and cellularity were analyzed and compared by Chi-square test (P < 0.05). Images of 50 cells per slide were captured and the nuclear area (NA) and cytoplasmic area (CA) were analyzed using an image analysis system. The nucleus-to-cytoplasmic area ratio (NA/CA) was calculated. To compare the means of groups SCA and C, the Student's t-test (P < 0.05) was applied to NA and CA; test non-parametric Mann Whitney U (P < 0.05) was used to compare NA/CA. Results: Mean values for SCA and C groups were: NA (69.38 and 59.63 ?m; P = 0.01); CA (2321.85 and 2185.60 ?m; P = 0.24); NA/CA (0.03 and 0.02; P = 0.13), respectively. A significant increase in NA for SCA group (P = 0.01) was seen. No morphological differences were found between the groups. There was a predominance of nucleated cells of the superficial layer in the smears of both groups. Class I smears were predominant in both groups. Conclusions: This study revealed that SCA was able to induce significant changes on nuclear area of the oral epithelial cells. PMID:23833399

Paraizo, Juliana Umetsu; Rech, Itauana Aliete Vettorello; Azevedo-Alanis, Luciana Reis; Pianovski, Mara Albonei Dudeque; De Lima, Antonio Adilson Soares; Machado, Maria 耼gela Naval

2013-01-01

418

Cost-effectiveness of Anemia Screening in Vulnerable Groups: A Systematic Review  

PubMed Central

Background: Anemia is the most common blood disorder observed in vulnerable groups and affects their efficiency in their everyday activities. Possible complications of the disease may be reduced or prevented by screening of patients. Screening programs impose certain costs upon the health system, which may offset their positive effects. Whether the positive impacts of screening outweigh its costs is a subject of debate among policy-makers. In this research, we have conducted a systematic review of the cost-effectiveness of anemia screening. Methods: The Pubmed, Science Direct, SCOPUS, EMBASE, and CINAHL databases were searched for relevant results dating between 1962-2010 using key words. The references of the related articles were gone over manually. In the end, Persian databases were also examined for results. Results: Using data from the four mentioned databases, a total of 722 articles were elected, which, after evaluation, were narrowed down to 4. Of these, 3 focused on newborns and infants. Disparity existed among obtained results, such that no two articles were similar, and this made making comparisons between them cumbersome and sometimes even impossible. Only one study evaluated cost-effectiveness of anemia screening in vulnerable target groups. Conclusions: Research findings show that there is not enough evidence of cost-effectiveness of screening for decision-making. Bearing in mind the importance of the matter to health policy-makers, due to high prevalence of iron-deficiency anemia in low- and middle-income countries, conduction of research in this field seems necessary. PMID:25104991

Nosratnejad, Shirin; Barfar, Eshagh; Hosseini, Hamed; Barooti, Esmat; Rashidian, Arash

2014-01-01

419

In vitro hemolytic activity of Lonomia obliqua caterpillar bristle extract on human and Wistar rat erythrocytes.  

PubMed

Human accidental envenomation caused by skin contact with the bristles of Lonomia obliqua caterpillar causes coagulation and fibrinolysis disorders. Alterations of hematologic parameters are observed only in severe cases of envenomation, but with no clinical evidence of intravascular hemolysis. However, since we have observed intravascular hemolysis in preliminary studies using Wistar rats as an experimental model for investigating L. obliqua envenomation, the objective of the present study was to investigate the in vitro hemolytic activity of the bristle extract of L. obliqua caterpillars on human and rat erythrocytes. Our results showed that the bristle extract has indirect and direct hemolytic activity on human and rat erythrocytes, although direct hemolytic activity was only observed at higher bristle extract concentrations. We also observed that the bristle extract has a proteolytic activity on band 3 of human and rat erythrocyte membranes. Thus, crude L. obliqua bristle extract was found to contain at least two components with hemolytic activity on erythrocytes, a phospholipase enzyme and another protein with a direct activity on the erythrocyte membrane. PMID:12782083

Seibert, Carla Simone; Shinohara, Elvira Maria Guerra; Sano-Martins, Ida Sigueko

2003-06-01

420

Antiproliferative, Cytotoxic and Hemolytic Activities of a Triterpene Glycoside from Psolus patagonicus and Its Desulfated Analog  

Microsoft Academic Search

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

Valeria P. Careaga; Carlos Bueno; Claudia Muniain; Laura Alch; Marta S. Maier

2009-01-01

421

Comparing micellar, hemolytic, and antibacterial properties of di-and tricarboxyl dendritic amphiphiles  

E-print Network

amphiphiles Bhadreshkumar B. Maisuria a, , Marcelo L. Actis a,脿 , Shauntrece N. Hardrict a,搂 , 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

Falkinham, Joseph

422

Assessment of phytochemicals, antioxidant, anti-lipid peroxidation and anti-hemolytic activity of extract and various fractions of Maytenus royleanus leaves  

PubMed Central

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

2013-01-01

423

Schilling evaluation of pernicious anemia: current status  

SciTech Connect

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.

Zuckier, L.S.; Chervu, L.R.

1984-09-01

424

Sideroblastic anemia recurring during two pregnancies.  

PubMed

A 25-year-old lady presented with a severe normocytic anemia (Hb 5.3 g/dl) and a sideroblastic marrow at the end of her first pregnancy. Six months into the puerperium, after the transfusion of a total of 8 units of red cells, there was apparent spontaneous improvement and then she was lost to follow-up. After a second pregnancy without clinical problems, she presented during a third pregnancy, at the age of 30 years, with similar hematological findings. Twenty-two months later she was well with a normal blood count. One possible reason for relapse in pregnancy is the increased demand for pyridoxine that occurs, but only one other case of sideroblastic anemia relapsing during pregnancies has been reported. PMID:8990629

Jackson, N; Hamizah, I

1996-12-01

425

Iron-Deficiency Anemia After Partial Gastrectomy  

PubMed Central

Although the mechanism for its development is not well understood, iron-deficiency anemia is a well-recognized consequence of partial gastrectomy. The reported incidence varies considerably, depending upon the criteria used to define anemia, and other factors. Rapid emptying of the gastric remnant, intestinal 揾urry, and borderline dietary-iron intake, with or without concomitant blood loss, produce malabsorption of some forms of iron that appears to be responsible for development of the deficiency. The diagnosis rests on hematological findings in the peripheral blood, the evaluation of iron stores, epithelial changes, and the response to adequate treatment. Oral iron therapy can be both effective and inexpensive and should form the mainstay of treatment. PMID:6019057

Geokas, M. C.; McKenna, R. D.

1967-01-01

426

Animal Models of Anemia of Inflammation  

PubMed Central

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

Rivera, Seth; Ganz, Tomas

2009-01-01

427

[Molecular study of Fanconi anemia in Tunisia].  

PubMed

Fanconi anemia (FA) is an autosomal recessive rare disease characterized by progressive pancytopenia, congenital malformations and predisposition to acute myeloid leukemia. Fanconi anemia is genetically heterogeneous, with at least eight complementation groups of FA (FAA to FAD2). In order to characterize the molecular defects underlying FA in Tunisia, fourty-one families were genotyped with microsatellite markers linked to known FA gene. Haplotype analysis and homozygosity mapping showed that 92% of these families belong to FAA group. We demonstrated the effectiveness of the molecular analysis for a better selection of bone marrow graft donor and for the evaluation of chimerism after bone marrow transplantation. This study also allows genetic counselling for FA family members. PMID:15453041

Bouchlaka, Chiraz; Abdelhak, Sonia; Dellagi, Koussay

2004-05-01

428

Cerebral vasculopathy in children with sickle cell anemia.  

PubMed

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

Fasano, Ross M; Meier, Emily R; Hulbert, Monica L

2015-01-01

429

Exacerbation of microcytic anemia associated with cessation of anti-retroviral therapy in an HIV-1-infected patient with beta thalassemia.  

PubMed

We report a patient with Japanese minor ? thalassemia and HIV-1 infection. The patient showed prolonged anemia, which was originally attributed to chronic parvovirus B19 infection. Twelve years later, the patient presented with exacerbation of microcytic anemia following cessation of anti-retroviral therapy; the exacerbation resolved when anti-retroviral therapy was resumed. Sequencing of the ? globin gene revealed heterozygosity for a four-nucleotides deletion at codon 41/42 and minor ? thalassemia was confirmed. Because HIV-1-infected patients frequently show anemia due to nutritional deficiencies, opportunistic infections, AIDS-related malignancies, drug treatment and a direct effect of HIV-1 on the bone marrow, it is likely to overlook other causes of anemia. Thalassemia should be considered in the differential diagnosis of anemia even in HIV-1 infected patients, when microcytic anemia without iron deficiency is observed. Our case suggested that active HIV infection may have worsened ? thalassemia, and early introduction of anti-retroviral therapy is beneficial for the recovery of anemia. PMID:24613601

Furukawa, Yoshitaka; Hashiguchi, Teruto; Minami, Rumi; Yamamoto, Masahiro; Takashima, Hiroshi

2014-06-01

430

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

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

Martin, Catherine; Poudel-Tandukar, Kalpana; Poudel, Krishna C.

2014-01-01

431

Trichomonas vaginalis: identification of a phospholipase A-dependent hemolytic activity in a vesicular subcellular fraction.  

PubMed

Trichomonad total extracts (TTE), or vesicular (P30) and soluble (530) subcellular fractions from 3 pathogenic Trichomonas vaginalis strains (GT-3. GT-13. and GT-15), lysed both human and Sprague-Dawley rat erythrocytes in a time- and dose-dependent manner. The entire hemolytic activity of TTE was located in P30, showing 2 peaks of maximum activity, one at pH 6.0 and another at pH 8.0. in the presence of 1 mM Ca2+. Hemolytic activity on rat erythrocytes was greater at pH 6.0 16.71 +/- 0.33 hemolytic units IHU]/mg/hr to 11.60 +/- 0.24 HU/mg/hr) than at pH 8.0 (3.81 +/- 0.30 HU/mg/hr to 5.75 +/- 0.65 HU/mg/hr). and it was greater than that on human red blood cells at pH 6.0 (2.67 +/- 0.19 HU/mg/hr to 4.08 +/- 0.15 HU/mg/hr) or pH 8.0 (2.24 +/- 0.0 9 HU/mg/hr to 2.81 +/- 0.06 HU/mg/hr). The alkaline and acidic hemolytic activity diminished (60-93% at pH 6.0 and 78-93% at pH 8.0) by the effect of 80 microM Rosenthal's inhibitor, which also inhibited 27-45% and 29-54% trichomonad alkaline and acidic phospholipase A activities, respectively. Vesicles, vacuoles, and hydrogenosomes were rich in P30. Trichomonas vaginalis has a hemolytic PLA, which could be involved in its cytopathogenic mechanism. PMID:12659311

Vargas-Villarreal, Javier; Mata-C醨denas, Benito D; Gonz醠ez-Salazar, Francisco; Lozano-Garza, Hector G; Cortes-Gutierrez, Elva I; Palaclos-Corona, Rebeca; Mart韓ez-Rodr韌uez, Herminia G; Ram韗ez-Bon, Enrique; Said-Fern醤dez, Salvador

2003-02-01

432

[Copper deficiency anemia morphologically mimicking myelodysplastic syndrome].  

PubMed

A 64-year-old man underwent kidney transplantation for progressive chronic renal failure which had developed 8 years after allogeneic bone marrow transplantation for acute myeloid leukemia. Because of post-operative complications, he had been placed on intravenous hyperalimentation. Three months after the transplantation, anemia rapidly progressed (hemoglobin, 7.9 g/dl). The proportion of reticulocytes was 0.2%, but white blood cell and platelet counts remained within normal ranges. Serum iron, vitamin B12, and folate levels were normal. Bone marrow examination showed the presence of ringed sideroblasts and cytoplasmic vacuoles in a fraction of erythroid cells. Megakaryocytes were adequate in number with normal morphology. Although the findings were consistent with refractory anemia with ringed sideroblasts according to the WHO classification, cytoplasmic vacuolations were also observed in myeloid cells, suggesting copper deficiency. Indeed, serum copper and ceruloplasmin levels were found to be low (33 ?g/dl and 11 mg/dl, respectively), and oral copper supplementation at a daily dose of 1 mg was initiated. There was a prompt increase in reticulocytes, and the hemoglobin level was normalized within one month, in response to this regimen. In progressive anemia cases with ringed sideroblasts in the bone marrow, copper deficiency should be considered in the differential diagnosis. PMID:24681939

Kikuchi, Taku; Mori, Takehiko; Shimizu, Takayuki; Morita, Shinya; Kono, Hidaka; Nakagawa, Ken; Mitsuhasi, Takayuki; Murata, Mitsuru; Okamoto, Shinichiro

2014-03-01

433

Secondary hypoplastic anemia in patients with familial amyloidotic polyneuropathy.  

PubMed

The anemia of patients with familial amyloidotic polyneuropathy (FAP) was evaluated. Anemia was seen in 32 (91%) of the 35 FAP patients, more often with progression of the disease. The incidence of macrocytic hypochromic anemia was the most common type (40%). In 14 autopsied and 2 biopsied cases, no amyloid deposition was detected in the bone marrow. Thirteen (81%) of the 16 FAP patients showed hypoplastic bone marrow. Bone marrow aspiration of 2 patients revealed a decreased ratio of erythrocytic/myelocytic cells. The plasma levels of vitamin B12 and folate were within normal ranges. Neither oral nor intravenous administration of iron had any effect on the anemia of FAP patients. Intravenous erythropoietin elevated blood hemoglobin levels and blood pressure in 2 patients. Orthostatic hypotension, one of the most common symptoms of FAP, was unexpectedly improved. Secondary hypoplastic anemia is common in FAP, but treatment of anemia in this disease using erythropoietin is promising. PMID:8291371

Asahara, K; Ando, Y; Tanaka, Y; Yi, S; Yamashita, T; Ando, M

1993-01-01

434

Anemia in heart failure: an overview of current concepts.  

PubMed

Chronic heart failure is a substantial public health problem. Anemia is an important comorbidity frequently observed in patients with the disease and, in heart failure, anemia has only recently started to attract systematic epidemiological and therapeutical research endeavor. This article describes the many aspects of anemia in chronic heart failure, starting with the ongoing discussion of how to define anemia, which has important consequences for the estimation of its prevalence and incidence. Further, we discuss prognostic implications of anemia in patients with chronic or acute heart failure, the etiology of anemia in heart failure and treatment possibilities. Such therapeutic avenues embrace intravenous iron preparations and subcutaneous administration of erythropoietin and its derivatives, all of which have been extensively studied over the last several years. Finally, this article describes the potential costs incurred by treating anemic patients with heart failure. PMID:21174515

von Haehling, Stephan; Jankowska, Ewa A; Ponikowski, Piotr; Anker, Stefan D

2011-01-01

435

Anemia evaluation and management in nursing home residents.  

PubMed

Anemia is often an unrecognized and/or undertreated diagnosis in older adults. Failure to diagnose anemia leads to delayed treatment and thus delayed relief of symptoms. Given the potentially significant impact of anemia on cardiovascular disease and physical performance among older nursing home (NH) residents, it is important to evaluate current clinical practice related to anemia.The purpose of this secondary data analysis was to evaluate the frequency of laboratory evaluation and medication treatment for anemia among older NH residents. Results indicated that more than half of NH residents were anemic at baseline, and of those, less than 20% had additional testing done to further evaluate for an underlying cause of their anemia and only 45.3% received any pharmacologic treatment. Future research is needed to clarify the potential benefits of timely diagnosis and appropriate treatment for anemic older adults in long-term-care settings and establish evidence-based guidelines to direct care in this area. PMID:20685904

Sabol, Valerie K; Resnick, Barbara; Galik, Elizabeth; Gruber-Baldini, Ann L; Gonce Morton, Patricia; Hicks, Gregory E

2010-06-01

436

A novel ubiquitin ligase is deficient in Fanconi anemia  

Microsoft Academic Search

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

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

437

Determinants of anemia among preschool children in rural, western Kenya.  

PubMed

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

Foote, Eric M; Sullivan, Kevin M; Ruth, Laird J; Oremo, Jared; Sadumah, Ibrahim; Williams, Thomas N; Suchdev, Parminder S

2013-04-01

438

Biomarkers for the differentiation of anemia and their clinical usefulness  

PubMed Central

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

Northrop-Clewes, Christine A; Thurnham, David I

2013-01-01

439

Iron deficiency anemia--bridging the knowledge and practice gap.  

PubMed

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

Shander, Aryeh; Goodnough, Lawrence T; Javidroozi, Mazyar; Auerbach, Michael; Carson, Jeffrey; Ershler, William B; Ghiglione, Mary; Glaspy, John; Lew, Indu

2014-07-01

440

Sequential Plasmodium chabaudi and Plasmodium berghei infections provide a novel model of severe malarial anemia.  

PubMed

Lack of an adequate animal model of Plasmodium falciparum severe malarial anemia (SMA) has hampered the understanding of this highly lethal condition. We developed a model of SMA by infecting C57BL/6 mice with P. chabaudi followed after recovery by P. berghei infection. P. chabaudi/P. berghei-infected mice had an initial 9- to 10-day phase of relatively low parasitemia and severe anemia, followed by a second phase of hyperparasitemia, more profound anemia, reticulocytosis, and death 14 to 21 days after infection. P. chabaudi/P. berghei-infected animals had more intense splenic hematopoiesis, higher interleukin-10 (IL-10)/tumor necrosis factor alpha and IL-12/gamma interferon (IFN-?) ratios, and higher antibody levels against P. berghei and P. chabaudi antigens than P. berghei-infected or P. chabaudi-recovered animals. Early treatment with chloroquine or artesunate did not prevent the anemia, suggesting that the bulk of red cell destruction was not due to the parasite. Red cells from P. chabaudi/P. berghei-infected animals had increased surface IgG and C3 by flow cytometry. However, C3(-/-) mice still developed anemia. Tracking of red cells labeled ex vivo and in vivo and analysis of frozen tissue sections by immunofluorescence microscopy showed that red cells from P. chabaudi/P. berghei-infected animals were removed at an accelerated rate in the liver by erythrophagocytosis. This model is practical and reproducible, and its similarities with P. falciparum SMA in humans makes it an appealing system with which to study the pathogenesis of this condition and explore potential immunomodulatory interventions. PMID:22689817

Harris, Juliana V; Bohr, Tiffany M; Stracener, Catherine; Landmesser, Mary E; Torres, Vladimir; Mbugua, Amos; Moratz, Chantal; Stoute, Jos A

2012-09-01

441

Treatment of Anemia in Inflammatory Bowel Disease Systematic Review and Meta-Analysis  

PubMed Central

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 1.041.51, 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

Avni, Tomer; Bieber, Amir; Steinmetz, Tali; Leibovici, Leonard; Gafter-Gvili, Anat

2013-01-01

442

Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-.  

PubMed

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

Ba?, Sema ?anal; Ozl, Onur

2012-10-01

443

Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-  

PubMed Central

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

謟l, Onur

2012-01-01

444

[Successful use of "juzen-taiho-to", a kampo medicine, for the treatment of perioperative anemia and hypoalbuminemia].  

PubMed

An 88-year-old woman suffering from femoral neck fracture was transported to the emergency room of a hospital. The patient and her family refused transfusion, despite anemia, stating their affiliation with Jehova's Witnesses. Surgery was performed under general anesthesia, and the following day, anemia (hemoglobin, 7.5 g x dl(-1)) and hypoalbuminemia (albumin, 2.7 g x dl(-1)) were observed, in addition to anorexia and general fatigue. The patient underwent nutritional treatment with a kampo medicine (Juzen-taiho-to), which was administered as a medication due to difficulties with swallowing the powdered form. On the 18th day after admission, anemia (hemoglobin, 8.9 g x dl(-1)) and hypoalbuminemia (3.6 g x dl(-1)) improved, as did anorexia and general fatigue. It is thought that the components Shimotsu-to, a component known to improve anemia, and Shikunshi-to, a vital energy supplementing component, were the main ingredients that conferred the improvements in anemia and hypoalbuminemia. These findings suggest that Chinese herbal medicine for the nutritional treatment of the elderly has minimal side effects. PMID:25199335

Tanaka, Tomohiro; Komasawa, Nobuyasu; Kataiwa, Maiko; Hashimoto, Tsutomu; Ohue, Mutsumi; Minami, Toshiaki

2014-08-01

445

Unveiling the Function of Fanconi Anemia Complementation Group A Protein (FANCA).  

E-print Network

??Fanconi anemia is a rare autosomal recessive or X-linked genetic disease characterized by progressive bone marrow failure, various developmental anomalies, and cancer predisposition. Fanconi anemia (more)

Qian, Liangyue

2014-01-01

446

Improved survival in severe acquired aplastic anemia of childhood.  

PubMed

Multi-agent immunosuppressive therapy has produced improved survival for severe acquired aplastic anemia in children. Recently, some investigators have suggested that immunosuppressive therapy may replace bone marrow transplantation as first-line therapy for this disorder. To assess its validity, we compared the outcomes of bone marrow transplantation vs immunosuppressive therapy in one institution from 1987 to 1997. We studied 46 consecutive patients less than 18 years of age who presented between January 1987 and April 1997. Inherited marrow failure syndromes and myelodysplastic syndromes were excluded. Patients received immunosuppressive therapy vs bone marrow transplantation based on availability of HLA-matched donors. The main outcome measures were survival, complete marrow and hematological remission, or partial remission but achieving independence from transfusional support. Twenty patients received multi-agent immunosuppressive therapy (cyclosporine, antithymocyte globulin and methylprednisolone); 11 attained complete remission and three partial remission for a transfusion-independent survival of 70%. Six patients died of infectious and hemorrhagic complications. Twenty-six patients were trans