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1

[Hemolytic anemias in adults].  

PubMed

The erythrocyte lifespan in haemolytic anemia is shortened while erythropoesis is increased. Important labaratory findings are increased reticulocytes, LDH, indirect bilirubin and a decreased haptoglobin level. The most important diagnostic tool for further work up of hemolytic anemia is the direct antiglobulin test (DAT, Coombs test) to differentiate autoimmune hemolytic anemia (AIHA) from other causes. Another important group are fragmentation syndroms (hemolytic uremic syndrome and thrombotic thrombocytopenic purpura). In these forms of haemolytic anemia fragmented red blood cells can be found in the blood smear together with thrombocytopenia. A severe problem in paroxysmal nocturnal hematuria is the incidence of thrombosis. The following review describes the most important forms of hemolytic anemia in the adult and the diagnostic and therapeutic strategies. PMID:22048936

Müller, A; Zimmermann, R; Krause, S W

2011-11-02

2

Autoimmune hemolytic anemia due to auto anti-N in a patient with sepsis.  

PubMed

We describe a case of autoimmune hemolytic anemia (AIHA) due to anti-N in a young male patient with cellulitis. There have been several reports of anti-N in N positive individuals. But in all these reports, auto anti-N was mostly associated with underlying immunological conditions. We report here a case of auto anti-N in a patient of bacterial sepsis without any underlying immune disorder. PMID:22985536

Singh, Lakhvinder; Malhotra, Sheetal; Kaur, Gagandeep; Basu, Sabita; Kaur, Ravneet

2012-09-15

3

[Congenital hemolytic anemia].  

PubMed

Gene analysis in Japanese patients with congenital hemolytic anemia due to red cell membrane disorders, thalassemias, unstable hemoglobinopathies and red cell enzymopathies were summarized. In hereditary spherocytosis, twenty-four mutations of band 3, five mutations of protein 4.2 and twenty mutations of ankyrin have been identified. In beta thalassemia, fourty-seven mutations of beta globin have been found, and ten mutations among them comprise 80% of beta thalassemia patients in Japan. Most common alpha0 and alpha+ thalassemia are--SEA and--alpha3.7, respectively. Fourty glucose-6-phosphate dehydrogenase mutations and twenty-three pyruvate kinase mutations have been identified, allowing a better understanding of the structure-function relationships of these enzymes. PMID:15773339

Ideguchi, Hiroshi

2005-03-01

4

Idiopathic autoimmune hemolytic anemia due to lecithin overdose: a case report  

PubMed Central

Introduction Idiopathic Autoimmune Hemolytic Anemia is a potentially fatal condition which requires prompt and potent treatment. Diagnosis of idiopathic autoimmune hemolytic anemia requires both serologic evidence of autoantibody presence and hemolysis. Although most of the times it is considered idiopathic, several underlying causes have been identified, like autoimmune and connective tissue diseases, viral infections, drugs or hyper function of the immune system. To our knowledge, this is the first case in the international literature describing lecithin-induced autoimmune hemolytic anemia. Case Presentation This case report is to highlight a rare but dangerous adverse reaction to overdose of lecithin. A 38 year old white female from Greece, presented to our emergency room with progressive fatigue over a period of ten days and icteric discoloration of her skin and conjunctiva. The patient had been taking lecithin supplements (1200 mg, 3 capsules a day) over a period of ten days for weight loss. She reports that the last 3 days, prior to the examination, she took 5 capsules/day, so that the supplement would take effect more rapidly. Her past medical, social and family history showed no disturbance. Relatives of the patient were requested to submit any blood-tests taken over a period of 20 days prior to the onset of symptoms caused by Lecithin. All tests proved that all functions were within normal scale. Her physical examination revealed pallor and jaundice without palpable hepatosplenomegaly. Blood biochemistry tests showed total bilirubin 7.5 mg/dl, with indirect bilirubin 6.4 mg/dl and complete blood count showed hemoglobin 7.6 g/dl with blood levels 21.4%. Conclusion In every case of idiopathic autoimmune hemolytic anemia the administration of pharmaceutical substances should always be examined, except for the standard reasons that cause it. In this case the cause of hemolysis was attributed to the excessive intake of lecithin capsules for the loss of body weight. It is important that clinicians and immunologists are aware of this adverse effect.

2009-01-01

5

HEMOLYTIC ANEMIAS, Recent Advances in Diagnosis and Treatment  

PubMed Central

The hemolytic anemias of unknown cause can be separated into two main groups: (1) those produced by a defect in cell structure, which is usually hereditary, and (2) those due to a hemolysin of immune-body type. The hemolytic anemias associated with hypersensitivity to drugs and disease processes such as leukemia are less well understood and need further investigation. Splenectomy is the only effective treatment in congenital hemolytic jaundice and in acquired hemolytic anemia; the operation should be carried out promptly in most cases. Transfusion may be used in all varieties of hemolytic disease and is the only effective form of therapy in sickle-cell anemia and paroxysmal nocturnal hemoglobinuria.

Evans, Robert S.; Duane, Rose

1949-01-01

6

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

7

Rare cause of hemolytic anemia.  

PubMed

Hemolysis is commonly seen in patients with mechanical heart valves and is secondary to destruction of red blood cells by mechanical action of artificial valve. It is very unusual after repair of native heart valve. Here we present a case of hemolytic anemia in association with mitral valve repair. PMID:23808095

Shailesh, Fnu; Deshmukh, Abhishek J; Singla, Sandeep

2013-06-01

8

Types of Hemolytic Anemia  

MedlinePLUS

... States, sickle cell anemia mainly affects African Americans. Thalassemias Thalassemias (thal-a-SE-me-ahs) are inherited blood ... make fewer healthy red blood cells than normal. Thalassemias most often affect people of Southeast Asian, Indian, ...

9

Chronic Hemolytic Anemia due to Cold Agglutinins: the Mechanism of Resistance of Red Cells to C? Hemolysis by Cold Agglutinins*  

PubMed Central

The red cells of patients with chronic hemolytic anemia due to cold agglutinins are agglutinated by antiglobulin serum in a nongamma reaction due to the coating of ?-globulins, C?4 and C?3. The red cells of such patients are abnormally resistant to C? hemolysis by cold agglutinin. Normal red cells can be made equally resistant to C? hemolysis by incubation with cold agglutinin and normal serum at temperatures which allow transient reactions between the red cells and cold agglutinins. The development of resistance to C? hemolysis was related to increasing susceptibility to agglutination in anti-?1c- and anti-?1e-sera and by increasing uptake of 131I activity from labeled anti-?-globulin serum containing antibodies for both globulins. There was decrease in the adsorption of 131I-labeled cold agglutinin during the development of resistance to C? hemolysis and reduced susceptibility to agglutination by cold agglutinins. Since cold agglutinins have been demonstrated to dissociate from the red cell, leaving fractions of C? globulin attached, it is postulated that repeated transient reactions produce the accumulation of incomplete C? complexes. Steric hindrance by the adsorbed C? complexes is probably responsible for the inhibition of the reaction with cold agglutinin. There is evidence that the adsorbed C? complexes also interfere with the hemolytic action of C? even when cold agglutinin has become reattached to the red cells. The accumulation of C? complexes by cold agglutinins appears to be the most important factor in the abnormal resistance to C? hemolysis exhibited by the patient's red cells. Other factors, such as the heterogeneity within a population of normal cells, appear to be of minor significance.

Evans, Robert S.; Turner, Elizabeth; Bingham, Margaret

1967-01-01

10

[Hemolytic anemia due to silent left ventricular rupture after stent thrombosis].  

PubMed

A 51-year-old woman with known ischemic cardiomyopathy presented to the family doctor for investigation of mild anemia. A routinely performed computed tomography of the abdomen resulted in the suspicion of intracardiac thrombi. The subsequent cardiac examinations showed a pseudoaneurysm of the left ventricle as a result of a silent rupture of a pre-existing left ventricular aneurysm. PMID:23179051

Krapivsky, A; Altilar, T; Vockelmann, C; Niroomand, F

2012-11-22

11

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 ‘Mosul’ (homozygote), PK ‘Bukarest1,2’, PK ‘Hamburg1’, PK ‘Köln1’, and PK ‘Essen’ (compound heterozygote). PK ‘Mosul’ showed normal positive cooperative substrate

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

1994-01-01

12

Rituximab in steroid refractory autoimmune hemolytic anemia.  

PubMed

Autoimmune hemolytic anemia is rare in children and infants and steroids are the corner stone of therapy. Management of the patients with steroid refractory/dependent disease is difficult .Rituximab is being used in the treatment of a variety of autoimmune diseases including Autoimmune hemolytic anemia (AIHA),especially in adults but there is scarce data regarding the use of this agent in pediatric AIHA patients.The authors report two cases of steroid refractory AIHA, who responded to rituximab with review the literature of its use in pediatrics. PMID:21830023

Gupta, Nitin; Sharma, Sanjeev; Seth, Tulika; Mishra, Pravas; Mahapatra, Manoranjan; Kumar, Suman; Kapoor, Rajan; Agarwal, Narendra

2011-08-10

13

Morphological Findings in Microangiopathic Hemolytic Anemia  

Microsoft Academic Search

The clinical diagnosis of microangiopathic hemolytic anemia (MHA) can be ascertained only by the prove of fragmentocytes (schistocytes) in the peripheral blood. Those fragmented red cells caused by different processes of microangiopathy are partly destroyed in the blood vessels, partly absorbed by the reticuloendothelial system and may produce typical histomorphological changes. Therefore, besides the histological classification of the microangiopathy in

P. J. Klein; H. E. Schaefer; Féaux de Lacroix; R. Fischer

1973-01-01

14

[Biermer's disease and autoimmune hemolytic anemia].  

PubMed

Biermer's disease is an autoimmune atrophic gastritis of the fundus predominantly responsible for a malabsorption of vitamin B12. Despite its association with several autoimmune disorders, few observations have reported an association with autoimmune hemolytic anemia (AIHA). We report a case of Biermer's disease associated with AIHA in a patient of 66 years old. PMID:22796620

Nafil, Hatim; Tazi, Illias; Mahmal, Lahoucine

15

Nonimmune hydrops fetalis caused by G6PD deficiency hemolytic crisis and congenital dyserythropoietic anemia.  

PubMed

We present a case of a female neonate who had a nonimmune hydrops fetalis and severe hemolytic anemia due to a rare combination of glucose-6-phosphate dehydrogenase (G6PD) deficiency and congenital dyserythropoietic anemia. We conclude that in severe cases with persistent anemia one should search after delivery for a second reason other than G6PD deficiency alone. PMID:23719252

Molad, M; Waisman, D; Rotschild, A; Auslander, R; Kessel, I; Soloviechick, M; Goldberg, Y; Shabad, E

2013-06-01

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

Gu, Wangang

2013-01-01

17

PATHOGENESIS OF THYMIC CHANGES IN NZB MICE WITH HEMOLYTIC ANEMIA  

PubMed Central

Lymphoid follicles evolve in the perivascular connective tissue of many organs, including the thymuses, in NZB/Bl mice with hemolytic anemia. In previously published studies, these thymic follicles have been held to be causal in the autoimmune genesis of the hemolytic anemia. The present study contradicts this interpretation by demonstrating: (a) lymphoid follicles develop in the perivascular connective tissue of many organs in NZB mice, and are not restricted to the thymuses; and (b) thymic lymphoid follicles develop in aged Swiss mice without hemolytic anemia. Contrary to previous reports, the thymuses of NZB mice contain normal numbers of Hassall's corpuscles, which develop from preexisting thymic epithelial cells, and not from blood vessels.

Siegler, Richard

1965-01-01

18

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

2013-05-21

19

Mechanism of autoimmune hemolytic anemia in chronic lymphocytic leukemia.  

PubMed

Chronic lymphocytic leukemia (CLL) is a malignant clonal expansion of CD5+B lymphocytes. The CD5+B lymphocytes have been postulated to produce autoantibodies. CLL patients may demonstrate features of autoimmunity including autoimmune hemolytic anemia. However, the origin of the autoantibodies causing the hemolysis is not clear. The present studies were performed to determine whether these autoantibodies are the products of the neoplastic B-CLL clones. Immunoglobulins (Ig) were eluted from washed red blood cells (RBC) obtained from two CLL patients at the time they had autoimmune (DAT-direct antiglobulin test-positive) hemolytic anemia. The light chain phenotypes of these eluted autoantibodies were determined and found to be monotypic with exact correlation to the light chain expressed on the surface of the B-CLL clones. Elutions from RBC of DAT negative patients or normal volunteers failed to demonstrate measurable amounts of Ig. In contrast, Ig eluted from RBC obtained from SLE patients with DAT positive hemolytic anemia found to be polyclonal autoantibodies exhibiting both light chain types. Furthermore, CD5+B lymphocytes obtained from the same two CLL patients (DAT+) produce, in vitro understimulation with phorbal myristate acetate (PMA), monoclonal antibodies which react and bind to RBC. Thus these studies provide direct evidence demonstrating that the antibodies causing the autoimmune hemolytic anemia in our two CLL patients are the products of the B-CLL neoplastic clones. PMID:7690517

Sthoeger, Z M; Sthoeger, D; Shtalrid, M; Sigler, E; Geltner, D; Berrebi, A

1993-08-01

20

Intracardiac Thrombus in a Patient with Autoimmune Hemolytic Anemia Leading to a Diagnosis of Antiphospholipid Syndrome  

Microsoft Academic Search

We report a case of antiphospholipid syndrome (APS) which presented with clinical and laboratory signs of an autoimmune hemolytic anemia (AHA), in the absence of manifestations typically related to APS. The diagnosis of APS was made only after the occurrence of a sudden severe heart failure due to an intraventricular thrombus requiring a surgical approach. An accurate thrombophilic screening is

Roberto Latagliata; Francesca Celesti; Velia Bongarzoni; Giandomenico Di Nucci; Concetta Torromeo; Salvatore Giacomo Morano; Giuseppe Cimino; Giuliana Alimena

2002-01-01

21

Pure red cell aplasia following autoimmune hemolytic anemia: an enigma.  

PubMed

A 26-year-old previously healthy female presented with a 6-month history of anemia. The laboratory findings revealed hemolytic anemia and direct antiglobulin test was positive. With a diagnosis of autoimmune hemolytic anemia (AIHA), prednisolone was started but was ineffective after 1 month of therapy. A bone marrow trephine biopsy revealed pure red cell aplasia (PRCA) showing severe erythroid hypoplasia. The case was considered PRCA following AIHA. This combination without clear underlying disease is rare. Human parvovirus B19 infection was not detected in the marrow aspirate during reticulocytopenia. The patient received azathioprine, and PRCA improved but significant hemolysis was once again documented with a high reticulocyte count. The short time interval between AIHA and PRCA phase suggested an increased possibility of the evolution of a single disease. PMID:23525059

Saha, M; Ray, S; Kundu, S; Chakrabarti, P

22

Immunosuppressive therapy for canine immune-mediated hemolytic anemia.  

PubMed

The mortality for dogs with severe immune-mediated hemolytic anemia (IMHA) is unacceptably high, and better immunosuppressive regimens are needed to increase survival. Understanding the basic immunology of the disease and the mechanisms of action of the available immunosuppressive therapies will help clinicians choose an appropriate immunosuppressive protocol. Prospective, randomized clinical studies must be conducted to evaluate the efficacy and safety of different combined immunosuppressive modalities to treat canine IMHA and improve patients' outcomes. PMID:19241356

Al-Ghazlat, Suliman

2009-01-01

23

Serological and Clinical Aspects of Autoimmune Hemolytic Anemias  

Microsoft Academic Search

SummaryAutoimmune hemolytic anemias (AIHAs) may occur when specific autoantibodies react with red blood cell (RBC) antigens. Decompensated hemolysis and detectable autoantibodies against RBCs are classical findings. The autoantibodies preferentially react at 37 °C (warm autoantibodies). The majority of these autoantibodies are of the IgG class; IgM and IgA warm autoantibodies are less common. Roughly 50% of the patients have an

A. Salama; N. Ahrens; H. Kiesewetter

2002-01-01

24

Study of serum hepcidin in hereditary hemolytic anemias.  

PubMed

The aim of this study was to assess the level of hepcidin in hereditary chronic hemolytic anemias and to correlate the serum hepcidin levels to the need for blood transfusions (frequency of blood transfusions and the serum ferritin level). Seventy pediatric patients with hereditary chronic hemolytic anemias, attending to hematology clinics of Cairo University and Misr University for Science and Technology (MUST) hospitals were the subjects of this study [53 patients with ?-thalassemia major (?-TM), 10 patients with ?-thalassemia intermedia (?-TI), four patients with congenital spherocytosis and three patients with sickle cell disease) (38 males and 32 females)]; their ages ranged from 1-14 years. Seventy normal children, age- and sex-matched, served as the control group. The results of this study revealed decreased hepcidin levels in patients (all types of congenital chronic hemolytic anemias) [mean ± SD (standard deviation) = 22.9 ± 6.0] compared to controls (mean ± SD = 132.4 ± 16.7) with highly significant statistical difference in between. Hepcidin levels were higher in ?-TM patients (mean ± SD = 23.7 ± 6.2) than in ?-TI patients (mean ± SD = 21.8 ± 4.0), the hepcidin to ferritin ratio was significantly less than one. In ?-TM patients, the mean ± SD was 0.03 ± 0.004, and in ?-TI patients the mean ± SD = 0.025 ± 0.002, with highly significant statistical difference with hepcidin-to-ferritin ratios in controls being mean ± SD = 2.3 ± 0.7. Hepcidin and hepcidin/ferritin ratios can be used as good markers of hemolytic anemia and iron overload as they have very high sensitivity (99.0 and 99.0%, respectively) and very high specificity (98.0 and 97.0%, respectively). Our findings highlight the potential usefulness of hepcidin measurement as a diagnostic tool. The use of hepcidin as an adjuvant therapy with iron chelators is important as it has a vital role in combating hemosidrosis. PMID:23088733

El Beshlawy, Amal; Alaraby, Ibrahim; Abdel Kader, Mohamed S E M; Ahmed, Dina H; Abdelrahman, Hossam E M

2012-10-23

25

A transgenic model of autoimmune hemolytic anemia  

PubMed Central

We made double transgenic mice bearing immunoglobulin heavy and light chain genes encoding an autoantibody against the mouse erythrocyte by the cross of C57BL/6 mice carrying the transgene for each chain of the immunoglobulin. Although no obvious disorders were found in the single- chain transgenic mice, severely anemic symptoms were found in some of the double transgenic mice, in which most B cells express, at least on their surface, the autoantibody reactive to self-antigens on the erythrocyte. Individual double-transgenic mice showed a wide variation of phenotypes between severe anemia and no symptoms. Both deletion and anergy of autoreactive B cells were seen in each individual mouse, but their relative contribution to self-tolerance was variable and not directly related to the severity of anemia or the amount of the autoantibody produced. This transgenic system provides a good autoimmune disease model for exploring its onset mechanism, and means of its treatment and prevention.

1992-01-01

26

Hematologic characterization and chromosomal localization of the novel dominantly inherited mouse hemolytic anemia, neonatal anemia (Nan).  

PubMed

One of the most commonly inherited anemias in man is Hereditary Spherocytosis (HS) with an incidence of 1 in 2000 for persons of Northern European descent. Mouse models of HS include spontaneous inherited hemolytic anemias and those generated by gene targeting. The Neonatal anemia (Nan) mouse is a novel model of HS generated by N-ethyl-N-nitrosurea mutagenesis and suffers from a severe neonatal anemia. Adult Nan mice have a lifelong hemolytic anemia with decreased red blood cell numbers, hematocrit, and hemoglobin, but elevated zinc protoporphyrin levels. Blood smears taken from Nan mice show a hypochromic anemia characterized by poikilocytosis, anisocytosis and polychromasia. The Nan phenotype can be transferred by bone marrow transplantation indicating that the defect is intrinsic to bone marrow. The hemolytic anemia in adult Nan mice can be identified by osmotic fragility testing. Examination of the erythrocyte membrane skeleton proteins (EMS) reveals a global deficiency of these proteins with protein 4.1a being completely absent. The Nan locus maps to mouse Chromosome 8 and does not co-localize with any known EMS genes. The identification of the Nan gene will likely uncover a novel protein that contributes to the stability of the EMS and may identify a new mutation for HS. PMID:19409822

White, Robert A; Sokolovsky, Inna V; Britt, Margaret I; Nsumu, Ndona N; Logsdon, Derek P; McNulty, Steven G; Wilmes, Leigh A; Brewer, Brandon P; Wirtz, Eric; Joyce, Heather R; Fegley, Barbara; Smith, Ann; Heruth, Daniel P

2009-05-01

27

Classification and therapeutic approaches in autoimmune hemolytic anemia: an update.  

PubMed

Autoimmune hemolytic anemia (AIHA) is an uncommon autoantibody-mediated immune disorder that affects both children and adults. The diagnosis of AIHA relies mainly on the direct antiglobulin test, which is a highly sensitive and relatively specific test. The classification of AIHA is based on the pattern of the direct antiglobulin test and on the immunochemical properties of the autoantibody (warm or cold type), but also on the presence or absence of an underlying condition or disease (secondary vs primary AIHAs) that may have an impact on treatment and outcome. The distinction between AIHAs due to warm antibody (wAIHA) and AIHAs due to cold antibody is a crucial step of the diagnostic procedure as it influences the therapeutic strategy. Whereas corticosteroids are the cornerstone of treatment in wAIHA, they have no or little efficacy in cold AIHA. In wAIHA that is refractory or dependent to corticosteroids, splenectomy and rituximab are both good alternatives and the benefit?risk ratio of each option must be discussed on an individual basis. In chronic agglutinin disease, the most common variety of cold AIHA in adults, beyond supportive measures, rituximab given either alone or in combination with chemotherapy may be helpful. In this article, the classification of AIHA and the recent progress in therapeutics are discussed. PMID:22077525

Michel, Marc

2011-12-01

28

Fatal hemolytic anemia associated with metformin: A case report  

PubMed Central

Introduction Metformin is a widely prescribed biguanide antidiabetic drug that has been implicated as a cause of hemolytic anemia in three previous case reports. We report a case of rapidly fatal hemolysis that was temporally associated with the initiation of metformin treatment for diabetes. Clinicians need to be aware of this rare but potentially serious side effect of metformin. Case presentation A 56-year-old Caucasian man with type 2 diabetes mellitus was started on metformin to improve glycemic control. Shortly afterwards, he developed progressive fatigue, exertional dyspnea, cranberry-colored urine and jaundice. Laboratory studies showed severe hemolysis, with a drop in hemoglobin from 14.7 to 6.6 g/dl over 4 days, markedly elevated lactate dehydrogenase, bilirubin and reticulocyte counts, and a low haptoglobin level. A peripheral blood smear showed no schistocytes, and a direct Coombs test was positive for anti-IgG and negative for anti-C3. Despite corticosteroid treatment and transfusion of packed red blood cells, the patient developed increasing dyspnea, hypotension, further decline in hemoglobin to 3.3 g/dl, and fatal cardiorespiratory arrest 12 hours after admission. Conclusion The serologic findings in this case suggest an autoimmune hemolytic anemia, caused either by a drug-induced autoantibody or a warm autoantibody. Based on the temporal association with metformin and the lack of other clear precipitating causes, we propose that metformin-induced hemolysis with a drug-induced autoantibody is a strong possibility. This mechanism differs from a previously described case with a possible antibody to the erythrocyte-drug complex. It has been shown, however, that hemolysis may occur via multiple mechanisms from the same drug. Clinicians should consider the possibility of metformin-associated immune hemolytic anemia in patients with otherwise unexplained hemolysis.

Packer, Clifford D; Hornick, Thomas R; Augustine, Sarah A

2008-01-01

29

IgA-mediated autoimmune hemolytic anemia in an infant.  

PubMed

Autoimmune hemolytic anemia (AIHA) is characterized by the presence of autoantibodies, most frequently of the IgG isotype, directed against erythrocyte surface antigens. The direct antiglobulin test (DAT) is the critical laboratory test for the diagnosis of AIHA, but is negative in 3-11% of cases. In these cases of DAT negative AIHA, a wider spectrum of clinical data including more specialized testing for erythrocyte autoantibodies may be required. We describe the unique and challenging case of an infant with corticosteroid-responsive, DAT negative AIHA, in which specialized gel card testing identified an isolated IgA autoantibody on the erythrocyte surface. PMID:21370419

McGann, Patrick T; McDade, Jenny; Mortier, Nicole A; Combs, Martha R; Ware, Russell E

2010-12-22

30

[Autoimmune hemolytic anemia in a patient with idiopathic interstitial pneumonia].  

PubMed

We report a rare case of autoimmune hemolytic anemia (AIHA) complicated by idiopathic interstitial pneumonia (IIP). A sixty-year-old man was diagnosed as having IIP in January 2009. In March, when he was hospitalized for the introduction of home oxygen therapy, severe anemia was detected. Based on the findings showing elevated levels of lactate dehydrogenase and indirect bilirubin, a decreased level of haptoglobin, positive Coombs test, and splenomegaly, a diagnosis of AIHA was made. Although anti-DNA antibody was found, diagnostic criteria for systemic lupus erythematosus and other collagen diseases were not fulfilled. Therefore, we concluded that AIHA coexisted with IIP. Treatment with prednisolone led to improvement of both AIHA and IIP. There has not been any exacerbation even after a gradual reduction of prednisolone to 7.5 mg/day. Coexistence of AIHA and IIP is rare, and accumulation of case reports is needed to gain a better understanding of this condition. PMID:21378476

Fukuda, Kuniyoshi; Yokoyama, Yasuhisa; Kamada, Yuhei; Taoka, Kenichi; Suzukawa, Kazumi; Chiba, Shigeru

2011-01-01

31

Risk of immune hemolytic anemia in children following immunization.  

PubMed

Several case reports have described immune hemolytic anemia (IHA) following vaccination in children. We examined the risk of IHA in the 42 days following vaccination exposure using a self-controlled case series study design. In our population-based cohort of nearly 4.5 million children in the Vaccine Safety Datalink, we identified 55 confirmed cases of new-onset IHA from 1991 through 2000. We found no association between IHA and diphtheria-pertussis-tetanus vaccination (incidence rate ratio (IRR)=0.65, 95% CI: 0.19-2.24), hepatitis B vaccination (IRR=1.73, 95% CI: 0.59-5.01), or any vaccination (IRR=1.04, 95% CI: 0.46-2.32). PMID:19766577

Naleway, Allison L; Belongia, Edward A; Donahue, James G; Kieke, Burney A; Glanz, Jason M

2009-09-18

32

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.

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

2010-01-01

33

Intravenous immunoglobulin-induced hemolytic anemia in a patient with juvenile dermatomyositis.  

PubMed

We report a case of hemolytic anemia in a female patient with juvenile dermatomyositis. Rapidly progressive hemolysis developed during prednisone and azathioprine combination therapy 4 days after completing intravenous immunoglobulin (IVIG) treatment. While the blood smear revealed spherocytes and polychromasia, direct and indirect antiglobulin tests were negative. In the following case report, we propose probable IVIG-induced hemolytic anemia and explore the pathomechanisms that may account for hemolysis. PMID:23377338

Clemenz, Melanie R; McGowan Iv, Joseph Wilson; Shuler, Marshall J; Lynn, Annette W

2013-01-01

34

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

Microsoft Academic Search

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

Elizabeth S. Klings; Mark T. Gladwin

35

Cold agglutinin induced autoimmune hemolytic anemia and NK-cell leukemia: a new association.  

PubMed

Cold agglutinin induced autoimmune hemolytic anemia is uncommonly associated with leukemia and lymphomas. We present a case of a young Mexican female presenting with a cold agglutinin hemolytic anemia with expression of a rare Pr antigen specificity and an aggressive NK-cell leukemia. Our patient had a rapid fatal course. To our knowledge this is the first reported case of such an association. PMID:17301976

Skorupa, Amy; Chaudhary, Uzair B; Lazarchick, John

2007-07-01

36

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

PubMed Central

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

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

2012-01-01

37

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

PubMed

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

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

2012-05-15

38

Autoimmune hemolytic anemia in a patient with Malaria  

PubMed Central

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

Sonani, Rajesh; Bhatnagar, Nidhi; Maitrey, Gajjar

2013-01-01

39

Autoimmune hemolytic anemia in a patient with Malaria.  

PubMed

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

Sonani, Rajesh; Bhatnagar, Nidhi; Maitrey, Gajjar

2013-07-01

40

Autoimmune hemolytic anemia with gel-based immunohematology tests.  

PubMed

We used gel centrifugation tests (GCTs) to analyze the relationship between the diagnosis and immunohematology tests used for autoimmune hemolytic anemia (AIHA). The study included 588 samples positive for the direct antiglobulin test (DAT). Of these, 52 were from patients diagnosed with AIHA. Immunoglobulin (Ig) class, IgG1, IgG3, and complement were measured. DAT strength had the strongest correlation with AIHA diagnosis (odds ratio [OR], 23), followed by anti-IgG titer 300 (OR, 8.4), anti-IgG titer 1,000 (OR, 10.5), and C3d agglutination strength (OR, 1.7). Decision tree analysis revealed that DAT strength and anti-IgG titer higher than 100 were the best predictors of AIHA. Multidimensional scanning analysis found a high grade of similarity among DAT strength, anti-IgG titer, and IgG strength in the AIHA samples. This observation was not detected in DAT-positive samples from patients without AIHA. DAT strength remained the best diagnostic indicator for AIHA and had the strongest association with AIHA compared with other commercially available immunohematology tests. The other tests, despite good correlation with AIHA diagnosis, did not add useful information. PMID:23525616

Lai, Marco; Leone, Giuseppe; Landolfi, Raffaele

2013-04-01

41

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

PubMed

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

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

2013-05-24

42

The Lbw2 locus promotes autoimmune hemolytic anemia.  

PubMed

The lupus-prone New Zealand Black (NZB) strain uniquely develops a genetically imposed severe spontaneous autoimmune hemolytic anemia (AIHA) that is very similar to the corresponding human disease. Previous studies have mapped anti-erythrocyte Ab (AEA)-promoting NZB loci to several chromosomal locations, including chromosome 4; however, none of these have been analyzed with interval congenics. In this study, we used NZB.NZW-Lbw2 congenic (designated Lbw2 congenic) mice containing an introgressed fragment of New Zealand White (NZW) on chromosome 4 encompassing Lbw2, a locus previously linked to survival, glomerulonephritis, and splenomegaly, to investigate its role in AIHA. Lbw2 congenic mice exhibited marked reductions in AEAs and splenomegaly but not in anti-nuclear Abs. Furthermore, Lbw2 congenics had greater numbers of marginal zone B cells and reduced expansion of peritoneal cells, particularly the B-1a cell subset at early ages, but no reduction in B cell response to LPS. Analysis of a panel of subinterval congenic mice showed that the full effect of Lbw2 on AEA production was dependent on three subloci, with splenomegaly mapping to two of the subloci and expansions of peritoneal cell populations, including B-1a cells to one. These results directly demonstrated the presence of AEA-specific promoting genes on NZB chromosome 4, documented a marked influence of background genes on autoimmune phenotypes related to Lbw2, and further refined the locations of the underlying genetic variants. Delineation of the Lbw2 genes should yield new insights into both the pathogenesis of AIHA and the nature of epistatic interactions of lupus-modifying genetic variants. PMID:22371393

Scatizzi, John C; Haraldsson, Maria K; Pollard, K Michael; Theofilopoulos, Argyrios N; Kono, Dwight H

2012-02-27

43

The Lbw2 Locus Promotes Autoimmune Hemolytic Anemia  

PubMed Central

The lupus-prone NZB strain uniquely develops a genetically imposed severe spontaneous autoimmune hemolytic anemia (AIHA) that is very similar to the corresponding human disease. Previous studies have mapped anti-erythrocyte Ab (AEA)-promoting NZB loci to several chromosomal locations, including chromosome 4, however, none of these have been analyzed with interval congenics. Here, we used NZB.NZW-Lbw2 congenic (designated Lbw2 congenic) mice containing an introgressed fragment of NZW on chromosome 4 encompassing Lbw2, a locus previously linked to survival, glomerulonephritis, and splenomegaly, to investigate the role in AIHA. Lbw2 congenic mice exhibited marked reductions in AEAs and splenomegaly, but not in anti-nuclear Abs. Furthermore, Lbw2 congenics had greater numbers of marginal zone B cells and reduced expansion of peritoneal cells, particularly the B-1a cell subset at early ages, but no reduction in B cell response to LPS. Analysis of a panel of subinterval congenic mice showed that the full effect of Lbw2 on AEA production was dependent on three subloci, with splenomegaly mapping to two of the subloci, and expansions of peritoneal cell populations, including B-1a cells to one. These results directly demonstrated the presence of AEA-specific promoting genes on NZB chromosome 4, documented a marked influence of background genes on autoimmune phenotypes related to Lbw2, and further refined the locations of the underlying genetic variants. Delineation of the Lbw2 genes should yield new insights into both the pathogenesis of AIHA and the nature of epistatic interactions of lupus-modifying genetic variants.

Scatizzi, John C.; Haraldsson, Maria K.; Pollard, K. Michael; Theofilopoulos, Argyrios N.; Kono, Dwight H.

2012-01-01

44

Uncommon neurological manifestations of hemolytic anemia: a report of two cases.  

PubMed

Neurological complications of hemolytic anemias are rather uncommon. We are reporting two cases of hemolytic anemia presenting as chorea and recurrent ischemic stroke. The first one is a case of chorea in a patient with sickle cell trait. Reviewing the literature we could find only one case report of chorea in sickle cell disease disease. The second is a case of recurrent ischemic stroke in hereditary spherocytosis. We could trace two reports on a Medline search, though their association was less certain. PMID:18688151

Ramu, Chitela Sita; Raju, Garuda Butchi; Rao, Kolli Satya; Venkateswarlu, Kolichana

45

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

PubMed Central

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

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

2013-01-01

46

Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis.  

PubMed

Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs' positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease. PMID:22607576

Korkmaz, Serdal; Elaldi, Nazif; Kayatas, Mansur; Sencan, Mehmet; Yildiz, Esin

2012-05-18

47

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

2007-08-06

48

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

49

Sickle Cell Disease and Hereditary Spherocytosis, a Rare Combination of Hemolytic Anemia Presenting as Cholelithiasis  

Microsoft Academic Search

We describe a 17-year-old African American female with known sickle cell disease who presented with right upper quadrant pain. During her hospitalization it was discovered that she also had nu- merous spherocytes on her peripheral smear, and subsequent os- motic fragility testing confirmed the secondary diagnosis of heredi- tary spherocytosis. This is a unique case of combination hemolytic anemias presenting

Edwin Robins; Deepti Dev; Anjali Limaye

50

Warm-Antibody Autoimmune Hemolytic Anemia Developing after Thrombotic Thrombocytopenic Purpura  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) and warm-antibody autoimmune hemolytic anemia (AIHA) are uncommon diseases. Although TTP has been increasingly described in association with autoimmune antibodies, there are very few reports of the association with autoimmune hematological conditions, including idiopathic thrombocytopenic purpura and AIHA. Here we describe a patient with classic manifestations of TTP, who was successfully treated with plasma exchange. A

Daniel Morgensztern; Mohamed A. Kharfan-Dabaja; Han-Mou Tsai; Eric C.-Y. Lian

2002-01-01

51

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

PubMed

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

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

2011-10-01

52

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.

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

2003-01-01

53

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 ...

54

The acute infection-associated hemolytic anemia of childhood: Immunofluorescent detection of microbial antigens altering the erythrocyte membrane  

Microsoft Academic Search

Summary The majority of acute infection-associated hemolytic diseases of infancy and childhood have been suggested to be caused by exogenic alterations of the erythrocyte surface, though laboratory methods for their further evaluation were not yet available. Investigating 96 children, the present study characterizes 72% of cases as corresponding to this type of acute acquired hemolytic anemia, which cannot be clearly

R.-C. Seitz; G. Buschermöhle; G. Dubberke; R. Herbrand; M. Maiwald; H. H. Hellwege

1993-01-01

55

[Hemolytic anemia in patients with heart valve prosthesis].  

PubMed

This article analyzes the historic evolution of the Starr-Edwards prosthesis manufacture and its association to hemolysis. It describes also the information related to bioprosthesis and hemolysis. The mechanisms involved in mechanical hemolysis are discussed (turbulent flux, red cells trapping, construction material and autoimmunity). Reviews the pathophysiology and criteria for clinical and laboratory diagnosis of hemolysis. We describe the value of the quantitation of unconjugated bilirubin, free plasmatic hemoglobin, DHL and it's DHL1 iso enzyme, methemalbumin and urinary hemosiderin for the specific diagnosis of this entity. Finally we comment on the utility of bed rest, cellular maturity inductors, propranolol and sulfinpyrazone therapy for the control of the hemolytic process. PMID:2198853

Cervantes Escárcega, J L; Izaguirre Avila, R

56

[Diabetic microangiopathic hemolytic anemia--rare complication of a common disease].  

PubMed

It is well-known that long-standing diabetes damages the vasculature. It is less frequently known that the plasticity of red blood cells may also be impaired so that they do not always survive intact in a vasculature roughened by diabetes, but are instead lysed. Lysis of red blood cells results in fragmentation hemolysis, which may lead to anemia if formation of red blood cells is deficient. We describe two patients with this rare complication, both of whom were diagnosed with diabetic microangiopathic hemolytic anemia (DMHA) during pregnancy. PMID:22737788

Laitinen, Kalevi; Anttila, Pekka

2012-01-01

57

[Successful treatment with rituximab for autoimmune hemolytic anemia associated with chronic lymphocytic leukemia].  

PubMed

A 68-year-old man was diagnosed with chronic lymphocytic leukemia (CLL) 3 years ago. His course was progressive, and he was complicated with autoimmune hemolytic anemia (AIHA). After the lack of efficacy of prednisone and cyclo-phosphamide, rituximab (375mg/m(2)) was administered based on the presence of CD20 positive leukemic cells by flow cytometric analysis of bone marrow. During 4 courses of rituximab administration, both anemia and hemolysis improved dramatically. Furthermore, the percentage of CLL cells in his peripheral blood was reduced. Rituximab may be one of the effective treatments for CLL associated AIHA in Japan as well as in foreign countries. PMID:23470833

Tanaka, Yuko; Ito, Yoshikazu; Yoshizawa, Sei-ichiro; Fujimoto, Hiroaki; Gotoh, Moritaka; Tauchi, Tetsuzo; Kimura, Yukihiko; Ohyashiki, Kazuma

2013-02-01

58

Phosphoglycerate kinase San Francisco: a new variant associated with hemolytic anemia but not with neuromuscular manifestations.  

PubMed

Phosphoglycerate kinase deficiency is a rare, x-linked glycolytic defect that, when severe, can be associated with hemolytic anemia, rhabdomyolysis, or neurological disorders. We report here a new phosphoglycerate kinase variant discovered in a boy with severe hemolytic anemia but no evidence of neuromuscular disease or developmental delay. The biochemical properties of the variant enzyme (greatly increased kmATP and km3-phosphoglycerate; normal pH optimum, electrophoretic mobility, and substrate specificity; resistance to heat inactivation) establish its uniqueness. Separation of light and dense red cells by centrifugation showed no greater loss of phosphoglycerate kinase activity in dense ("old") variant cells than in normal cells. We postulate that the striking stability of the variant enzyme allows cells capable of protein synthesis to accumulate sufficient enzyme to limit neuromuscular sequelae. PMID:3605066

Guis, M S; Karadsheh, N; Mentzer, W C

1987-06-01

59

Three Cases of Hereditary Nonspherocytic Hemolytic Anemia Associated With Red Blood Cell Glutathione Deficiency  

Microsoft Academic Search

Three unrelated Japanese patients with chronic nonsphero- cytic hemolytic anemia were found to have marked deft- ciency of red blood cell (RBC) reduced glutathione (GSH) (4.4%. 13.1%. and 6.9% of normal, respectively). A panel of RBC enzyme assays showed that one patient had decreased glutathione synthetase activity and the other two were moderately deficient in y-glutamylcysteine synthetase. Some family members

Akira Hirono; Junichi Ueyama; Hirotane Chiba; Hitoshi Kanno; Hisaichi Fujii; Shiro Miwa

1996-01-01

60

Autoimmune hemolytic anemia by coexisting Anti-I and Anti-Fl cold agglutinins  

Microsoft Academic Search

In association with atypical pneumonia, a patient developed acute severe autoimmune hemolytic anemia. Hemoglobin temporarily was only 7.0 g\\/100 ml, so that the patient needed red blood cell (RBC) transfusion. Hemolysis was found to be caused by high titer cold agglutinins (CA), which occurred transiently during the acute period of the disease. CA of two different specificities, anti-I and anti-Fl,

A. L. König; H. Kather; D. Roelcke

1984-01-01

61

Protective role of the free glucocorticoid pool in the development of autoimmune hemolytic anemia  

Microsoft Academic Search

The development of autoimmune hemolytic anemia in NZB mice aged 2–3, 6–7, and 10–12 months is associated with decreased corticosterone\\u000a and increased corticosteroid-binding globulin concentrations in the blood. An increase in the pool of free glucocorticoids\\u000a induced by dexamethasone decelerates the autoimmune processes. (NZBDBZ\\/2) F1 mice, which never develop the disease, are characterized by a high adrenal activity and a

A. Yu. Karyagina; V. M. Chesnokova; A. N. Ivanova; A. A. Tinnikov; L. N. Ivanova

1998-01-01

62

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:23389337

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

2013-02-01

63

Cutaneous anthrax associated with microangiopathic hemolytic anemia and coagulopathy in a 7-month-old infant.  

PubMed

A 7-month-old infant with cutaneous anthrax developed severe systemic illness despite early treatment with antibiotics. The infant displayed severe microangiopathic hemolytic anemia with renal involvement, coagulopathy, and hyponatremia. These findings are unusual with cutaneous anthrax, but have been described in illness resulting from spider toxin and may delay correct diagnosis. The systemic manifestations of the disease persisted for nearly a month despite corticosteroid therapy, but resolved. PMID:11851579

Freedman, Abigail; Afonja, Olubunmi; Chang, Mary Wu; Mostashari, Farzad; Blaser, Martin; Perez-Perez, Guillermo; Lazarus, Herb; Schacht, Robert; Guttenberg, Jane; Traister, Michael; Borkowsky, William

2002-02-20

64

Autoimmune hemolytic anemia: mixed type-a case report.  

PubMed

Mixed autoimmune haemolytic anemia (AIHA) is defined by the presence of both warm and cold auto antibodies. Diagnosis is based on detection of autoantibodies by monospecific direct antiglobulin test showing a pattern of IgG and complement C3d and presence of cold agglutinins. We report a rare case of primary mixed AIHA in a 12 year old girl who responded to corticosteroids. PMID:22654303

Sudha Reddy, V R; Samayam, Purnima; Ravichander, B; Bai, Uma

2011-05-07

65

Production of the effector cytokine interleukin-17, rather than interferon-?, is more strongly associated with autoimmune hemolytic anemia  

PubMed Central

Background Interleukin-17A is the signature cytokine of the Th17 subset and drives inflammatory pathology, but its relevance to autoantibody-mediated diseases is unclear. Th1 cells secreting interferon-? have been implicated in autoimmune hemolytic anemia, so the aim was to determine which cytokine is more closely associated with disease severity. Design and Methods Interferon-? and interleukin-17A were measured in the sera of patients with autoimmune hemolytic anemia and healthy donors, and in peripheral blood mononuclear cell cultures stimulated with autologous red blood cells, or a panel of peptides spanning red blood cell autoantigen. Results Serum interleukin-17A, but not interferon-?, was significantly raised in patients with autoimmune hemolytic anemia (P <0.001), and correlated with the degree of anemia. Interleukin-17A was also more prominent in the responses of peripheral blood mononuclear cells from patients with autoimmune hemolytic anemia to red blood cells, and, again unlike interferon-?, significantly associated with more severe anemia (P <0.005). There were no interleukin-17A responses to red blood cells by peripheral blood mononuclear cells from healthy donors. Specific autoantigenic peptides were identified that elicit patients' interleukin-17A responses. Conclusions Interleukin-17A makes a previously unrecognized contribution to the autoimmune response in autoimmune hemolytic anemia, challenging the model that the disease is driven primarily by Th1 cells. This raises the possibility that Th17, rather than Th1, cells should be the target for therapy.

Hall, Andrew M.; Zamzami, Omar M.; Whibley, Natasha; Hampsey, Daniel P.; Haggart, Anne M.; Vickers, Mark A.; Barker, Robert N.

2012-01-01

66

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

Microsoft Academic Search

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

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

1983-01-01

67

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

Microsoft Academic Search

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

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

2011-01-01

68

Hemolytic Anemia as a Sequela of Arsenic Intoxication Following Long-Term Ingestion of Traditional Chinese Medicine  

PubMed Central

We report on a 51-yr-old woman who developed intravascular hemolytic anemia caused by arsenic after long-term ingestion of a traditional Chinese medicine (TCM). Twelve years before the admission, she was diagnosed as neurocysticercosis. She has ingested a TCM for about 12 yr instead of undergoing medical therapy for the disease. She was presented with a severe Coombs'-negative hemolytic anemia with hemosiderinuria. The urine arsenic level was elevated suggesting the arsenic intoxication as a cause of the anemia. She was treated successfully with therapeutic red cell exchange without any sequelae.

Lee, Je-Jung; Kim, Yeo-Kyeoung; Cho, Sang-Hee; Park, Kyeong-Soo; Chung, Ik-Joo; Cho, Duck; Ryang, Dong-Wook

2004-01-01

69

Pentoxifylline for the treatment of hemolytic anemia in a patient who developed recurrent gastrointestinal bleeding while on continuous-flow left ventricular assist device support.  

PubMed

Pentoxifylline is an agent that improves red blood cell deformability (known as a hemorrheologic effect) and reduces blood viscosity. Here, we present a case of a patient with hemolytic anemia after continuous-flow left ventricular assist device (CF-LVAD) implantation that was successfully treated with pentoxifylline. Our case is a 64-year-old African American woman who was implanted with a HeartMate II device on August 6, 2010, as a bridge to transplant for end-stage heart failure. Her postoperative course was complicated by recurrent gastrointestinal bleeding and antiplatelet therapy was discontinued. On October 25, 2011, she was readmitted with anemia and hemoglobin of 6.6 mg/dl and no identifiable source of bleeding. Her lactate dehydrogenase (LDH) was 936 IU/L, indicating severe hemolysis. Due to her evidence of hemolytic anemia and her inability to tolerate antiplatelet therapy due to recurrent bleeding, she was discharged on pentoxifylline 400 mg thrice daily on October 27, 2011, with hemoglobin of 11.2 mg/dl after transfusion. After 60 days of pentoxifylline, her hemoglobin and LDH in clinic were 10.1 mg/dl and 223 IU/L, respectively. The patient was successfully bridged to transplant in June 2012. Additional analysis of pentoxifylline as a therapeutic modality to manage hemolytic anemia after CF-LVAD implantation may be warranted. PMID:23896772

Jennings, Douglas L; Williams, Celeste T; Morgan, Jeffrey A

70

Successful Treatment with Cyclosporine and High-Dose Gamma Immunoglobulin for Persistent Parvovirus b19 Infection in a Patient with Refractory Autoimmune Hemolytic Anemia  

Microsoft Academic Search

We describe a patient with persistent pure red cell aplasia due to human parvovirus B19 (HPVB19) infection during immunosuppressive\\u000a therapy for refractory autoimmune hemolytic anemia (AIHA).The patient had been given corticosteroid (CS) and\\/or azathioprine\\u000a for AIHA. During the course of treatment, reticulocyte count and hemoglobin levels decreased suddenly. Bone marrow aspirate\\u000a showed erythroid lineage-specific aplasia with a few giant proerythroblasts,

Shigeki Ito; Tatsuo Oyake; Toshiyuki Uchiyama; Takeshi Sugawara; Kazunori Murai; Yoji Ishida

2004-01-01

71

Severe Hemolytic Anemia Associated with Mild Pneumonia Caused by Mycoplasma pneumonia  

PubMed Central

We report a case of M. pneumoniae infection presenting with severe hemolytic anemia in a 4-year-old girl, with a ten-day history of paleness, weakness, and nonproductive cough. She was very pale and tachycardic. However, she was not tachypneic. Chest examination showed normal breath sounds. No rhoncus or whistling was heard. As the erythrocyte sedimentation rate was excessively elevated, the differential diagnosis primarily comprised hematological malignancies. Direct Coombs' test was positive. Diagnosis of M. pneumoniae infection was confirmed by elevated levels of M. pneumoniae IgG and IgM antibodies and a chest X-ray suggestive of atypical pneumonia. The patient was treated with clarithromycin and packed red cell transfusion and showed a favorable recovery within ten days after admission. In conclusion, this case demonstrates that severe hemolytic anemia caused by M. pneumoniae is not always associated with severe pulmonary involvement, even when the respiratory infection is very mild, M. pneumoniae may be the cause of severe anemia.

Kurugol, Zafer; Onen, Serife Sebnem; Koturoglu, Guldane

2012-01-01

72

Altered sulfhydryl reactivity of hemoglobins and red blood cell membranes in congenital heinz body hemolytic anemia  

PubMed Central

The mechanisms of hemoglobin precipitation into Heinz bodies and hemolytic anemia that characterize congenital Heinz body hemolytic anemia (CHBHA) were studied in patients with the unstable hemoglobins, Köln (?-98 valine ? methionine) and Hammersmith (?-42 phenylalanine ? serine). The cysteines in the 93rd position of the ?-chains of CHBHA hemoglobins bound glutathione excessively in mixed disulfide linkage. The resulting diminished “free” GSH within the cell accelerated hexose monophosphate shunt metabolism. The unique precipitability of CHBHA hemoglobins when heated at 50°C could be induced in normal hemoglobin A by artificially blockading its sulfhydryl groups with paramercuribenzoate (PMB). Reflecting the previously reported excessive flux of hemes from hemoglobin Köln, the expected heme/globin ratio in this hemoglobin was reduced by 30%. The further increment in heme loss that occurs with heat (50°C) underlies the unique heat precipitability of CHBHA hemoglobins; it was retarded if detachment of heme was inhibited by cyanide or carbon monoxide. Heinz bodies were attached to red cell membrane thiol groups presumably through mixed disulfide bonds, being released by mercaptoethanol. Binding of hemoglobin Köln-59Fe to red cell ghosts, which was markedly enhanced when Heinz bodies were generated at 50°C, was inhibited if membrane thiols were preblockaded by PMB. The depletion of membrane thiols by their reaction with Heinz bodies rendered CHBHA red cells hypersusceptible to membrane sulfhydryl inhibitors, as manifested by inordinate cation leakage, osmotic fragility, and autohemolysis. We conclude that both cellular and membrane thiols bind ?-93 sulfhydryls of CHBHA hemoglobins as mixed disulfides. Concomitantly, heme avidity to ?-92 lessens, suggesting that degradation of the resulting excessively freed heme may produce the pigmented dipyrroluria of this syndrome. Heinz bodies, reflecting the heightend precipitability of heme-deficient globin, attach to, thereby depleting, membrane sulfhydryl groups. This, as shown previously, could underlie the hemolytic anemia of this syndrome by causing membrane hyperpermeability, premature splenic entrapment, and ultimately osmotic destruction of red blood cells.

Jacob, Harry S.; Brain, Michael C.; Dacie, John V.

1968-01-01

73

The First Study on Nucleotide-level Identification of Hb Koriyama in a Patient with Severe Hemolytic Anemia  

PubMed Central

Hereditary hemolytic anemia comprises a group of disorders in which red blood cells are destroyed faster than they are produced in the bone marrow; various hereditary factors can cause this condition, including production of defective Hb and erythrocyte membrane. Recently, we identified Hb Koriyama, a rare Hb variant that was undetectable in Hb electrophoresis and stability tests, in a patient with severe hemolytic anemia. This is the first study to show the nucleotide-level sequence variations in Hb Koriyama. On the basis of our results, we conclude that unstable Hb may not be detectable by conventional Hb electrophoresis or stability tests. Thus, we suggest further genetic workup in cases of unexplained hereditary hemolytic anemia.

Park, Seungman; Park, Jun Eun; Cho, Sung Im; Jeon, Yongbum; Park, Sung Sup

2012-01-01

74

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’s 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.

Sachdeva, Mickey; Abdulhaq, Haifaa

2013-01-01

75

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

PubMed

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; Lee, Hye Ran

2011-12-27

76

Cephalosporin-induced Hemolytic Anemia in a Sicilian Child; Erythropoiesis.  

PubMed

A 27-month-old child developed acute hemolysis on two occasions after the administration of cephalosporin. On the first occasion, hemolysis was intravascular and was due to the formation of complexes between antibodies and the drug, which bound to red blood cells and caused severe hemolysis. On the second occasion, hemolysis was extravascular and was probably due to antibody-dependent cell-mediated cytotoxicity. Marked increases in levels of CD(19) (+) and CD(57) (+) CD(8) (+) cells were detected among the subpopulations of the patient's lymphocytes but only in the level of CD(19) (+) cells from the patient's father, after incubation of a sample of whole blood with a solution of cephalosporins. These results might explain the differences between the immune response of the patient and those of other members of his family and of an unrelated control. PMID:11399632

Malaponte, G.; Arcidiacono, C.; Mazzarino, C.; Pelligra, S.; Li Volti, G.; Bevelacqua, V.; Li Volti, S.

2000-01-01

77

Development of Mixed-Type Autoimmune Hemolytic Anemia and Evans’ Syndrome following Chicken Pox Infection in a Case of Low-Titer Cold Agglutinin Disease  

Microsoft Academic Search

We describe a patient with low-titer cold agglutinin disease (CAD) who developed mixed-type autoimmune hemolytic anemia (AIHA)\\u000a and idiopathic thrombocytopenia following chicken pox infection. At least 1 year before admission to hospital, the patient\\u000a had mild hemolytic anemia associated with low-titer cold agglutinins. A severe hemolytic crisis and thrombocytopenia (Evans’\\u000a syndrome) occurred several days after infection with chicken pox, and

Yumi Tanaka; Masahiro Masuya; Naoyuki Katayama; Eri Miyata; Yuka Sugimoto; Tetsunori Shibasaki; Kentaro Yamamura; Kohshi Ohishi; Nobuyuki Minami; Hiroshi Shiku; Tsutomu Nobori

2006-01-01

78

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

PubMed Central

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

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

2013-01-01

79

Enteropathy-associated T-cell lymphoma type II complicated by autoimmune hemolytic anemia.  

PubMed

A 74-year-old man was admitted to hospital because of persistent fever, diarrhea, and abdominal pain. CT scanning showed extensive wall thickening of the colon. He was transferred to our hospital because he further developed ascites and paraaortic lymph node swelling. On presentation, he was extremely emaciated with superficial lymph node swelling, ascitic signs, and leg edema. Histological image of a biopsied mesenteric lymph node demonstrated diffuse infiltration of large abnormal T cells. Surface antigen analysis of abnormal cells in the ascites revealed positivity for CD3, CD8, CD56, and weak positivity for CD103. Polymerase chain reaction analysis showed monoclonal rearrangement of the T cell receptor (TCR) gene. The subtype of TCR was ??. A diagnosis of enteropathy-associated T cell lymphoma (EATL) type II was made. The lymphoma involved the bone marrow. The patient also had severe hemolytic anemia with a positive Coomb's test result. An additional diagnosis for autoimmune hemolytic anemia (AIHA) was made, which was resistant to methylprednisolone therapy. We first treated him with only vincristine in addition to the steroid to avoid acute tumor lysis syndrome ; however, he died of septic shock that occurred soon after vincristine administration. To the best of our knowledge, this may be the first reported case of EATL complicated by AIHA. PMID:22104311

Kato, Aiko; Takiuchi, Yoko; Aoki, Kazunari; Ono, Yuichiro; Arima, Hiroshi; Nagano, Seiji; Tabata, Sumie; Yanagita, Soshi; Matsushita, Akiko; Maruoka, Hayato; Wada, Masaya; Imai, Yukihiro; Ishikawa, Takayuki; Takahashi, Takayuki

2011-01-01

80

Gingival overgrowth in a dog that received long-term cyclosporine for immune-mediated hemolytic anemia  

PubMed Central

Gingival mass lesions developed when cyclosporine was administered for 600 days to a female, 7-year-old, longhaired dachshund diagnosed with intractable immune-mediated hemolytic anemia (IMHA). Histopathology indicated hyperplastic suppurative gingivitis. As the anemia improved, the dosage of cyclosporine A (CsA) was markedly decreased, and the mass lesions decreased in size and disappeared, thus suggesting that the mass lesions were an adverse reaction to CsA.

Namikawa, Kazuhiko; Maruo, Takuya; Honda, Mayumi; Hirata, Hitomi; Lynch, Jonathan; Madarame, Hiroo

2012-01-01

81

Gingival overgrowth in a dog that received long-term cyclosporine for immune-mediated hemolytic anemia.  

PubMed

Gingival mass lesions developed when cyclosporine was administered for 600 days to a female, 7-year-old, longhaired dachshund diagnosed with intractable immune-mediated hemolytic anemia (IMHA). Histopathology indicated hyperplastic suppurative gingivitis. As the anemia improved, the dosage of cyclosporine A (CsA) was markedly decreased, and the mass lesions decreased in size and disappeared, thus suggesting that the mass lesions were an adverse reaction to CsA. PMID:22753966

Namikawa, Kazuhiko; Maruo, Takuya; Honda, Mayumi; Hirata, Hitomi; Lynch, Jonathan; Madarame, Hiroo

2012-01-01

82

Characterization of direct antiglobulin test-negative autoimmune hemolytic anemia: a study of 154 cases.  

PubMed

Direct antiglobulin test (DAT)-negative (DAT-)autoimmune hemolytic anemia (AIHA) is empirically thought to show the same clinical conditions as DAT-positive (DAT+)AIHA, with the exception of an adequate amount of red blood cell (RBC)-bound immunoglobulin (Ig)G. We investigated the clinical characteristics of DAT-AIHA in comparison with DAT+AIHA. Of the 582 patients referred to our laboratory with undiagnosed hemolytic anemia, AIHA was clinically diagnosed in 216 patients (DAT-AIHA, n = 154; DAT+AIHA, n = 62). The percentage of reticulocytes, mean corpuscular volume, RBC-IgG levels, white blood cell count, and total protein (TP) levels were significantly higher in patients with DAT+AIHA than patients with DAT-AIHA. The hemoglobin level was significantly lower in patients with DAT+AIHA. No significant differences between patients with DAT-AIHA and DAT+AIHA existed with respect to age, gender, idiopathic/secondary nature, complications such as Evans syndrome, effectiveness of steroid treatment, or survival rate at 1 year following diagnosis. Patients with DAT-AIHA required significantly lower doses of steroids for maintenance therapy. Based on multivariate analysis of idiopathic DAT-AIHA (n = 110), TP and Evans syndrome were associated with the effectiveness of steroids (adjusted odds ratio [aOR], 1.36/[0.1 g/dl]; 95% confidence interval [CI], 1.01-1.84) and survival at the 1-year follow-up (aOR, 0.1; 95% CI, 0.01-0.88). Our results indicate that patients with DAT-AIHA generally suffer milder anemia and hemolysis than patients with DAT+AIHA, respond equally well to steroids, and have comparable survival at 1-year. PMID:23169533

Kamesaki, Toyomi; Toyotsuji, Tomonori; Kajii, Eiji

2012-11-21

83

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.

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

84

Human erythrocyte pyruvate kinase: characterization of the recombinant enzyme and a mutant form (R510Q) causing nonspherocytic hemolytic anemia  

Microsoft Academic Search

Human erythrocyte pyruvate kinase plays an important role in erythrocyte metabo- lism. Mutation on the gene results in pyruvate kinase deficiency and is an im- portant cause of hereditary nonsphero- cytic hemolytic anemia. Because of diffi- culties in isolating the mutant enzymes from patients, these mutations have not been fully studied. In this study, a comple- mentary DNA (cDNA) encoding

Changqing Wang; Laurent R. Chiarelli; Paola Bianchi; Donald J. Abraham; Alessandro Galizzi; Andrea Mattevi; Alberto Zanella; Giovanna Valentini

2001-01-01

85

Erythrocyte membrane defects in hemolytic anemias found through derivative thermal analysis of electric impedance.  

PubMed

Hereditary hemolytic anemias originate mainly from defects in hemoglobin and plasma membrane proteins. Here, we propose a new method, thermal analysis of impedance, sensitive to membrane defects. It detects three processes in erythrocyte membrane; fall in membrane capacity at 49.5 degrees C and activation of passive PO(4)(2+) permeability at 37 degrees C and inorganic ions at 61.5 degrees C. The denaturation of spectrin is involved in the first process whilst the anion channel is involved in latter processes. Using this method three persons with xerocytosis were found whereby the fall in membrane capacity and spherization of erythrocytes were both postponed (53 degrees C) compared to control (49.5 degrees C). In contrast to control cells, strong activation of passive permeability for Cl(-) at 37 degrees C and sucrose at 61 degrees C were detected that were both eliminated by pre-inhibition of the anion channel with 4,4'-diisothiocyanato-stilbene-2,2'-disulfonic acid (DIDS). In addition, erythrocytes from 15 patients with various forms of anemia were studied in intact state and after refreshment. The results were compared with the data of clinical laboratory and osmotic fragility test. The final conclusion is that this method detects membrane defects with altered spectrin and anion channel syndrome (hereditary xerocytosis, spherocytosis, poikilocytosis and pyropoikilocytosis, elliptocytosis and stomatocytosis) and, after refreshment, helps differentiate them from the anemia with hemoglobinopathy. PMID:17395266

Ivanov, I T; Tolekova, A; Chakaarova, P

2007-03-01

86

Successful rituximab treatment for acquired amegakaryocytic thrombocytopenic purpura complicated with Coombs-negative autoimmune hemolytic anemia.  

PubMed

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m(2) per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab. PMID:23823096

Hashimoto, Akari; Fujimi, Akihito; Kanisawa, Yuji; Matsuno, Teppei; Okuda, Toshinori; Minami, Shinya; Doi, Tadashi; Ishikawa, Kazuma; Uemura, Naoki; Tomaru, Utano

2013-06-01

87

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

PubMed Central

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

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

2013-01-01

88

Lymphocyte Rich Hodgkin's Lymphoma Presented with Warm Hemolytic Anemia: A Case Report and Literature Review.  

PubMed

Hodgkin's lymphoma accounts for ten percent of all lymphomas. In the United States, there are about 8000 new cases every year. This paper describes a case of lymphocyte-rich Hodgkin's lymphoma (LRHL) manifested by autoimmune hemolytic anemia (AIHA). A 27-year-old Israeli male presented with dizziness associated with one month of low-grade fevers and night sweats; he also complained of persistent cough, pruritus, and ten-pound weight lost during this time. The CBC revealed hemoglobin of 5.9?gm/dL, and direct Coomb's test detected multiple nonspecific antibodies consistent with the diagnosis of AIHA. Chest, abdomen, and pelvic CT scan showed mediastinal lymphadenopathy and splenomegaly. Lymph node biopsy revealed classic LRHL. AIHA resolved after completion of the first cycle of chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD); after six cycles, he went into complete remission. Although infrequent, AIHA can be responsible for the presenting symptoms of HL. PMID:22937306

Hurtado-Cordovi, Jorge M; Verma, Vaibhav; Gotlieb, Vladimir; Frieri, Marianne

2011-09-28

89

Lymphocyte Rich Hodgkin's Lymphoma Presented with Warm Hemolytic Anemia: A Case Report and Literature Review  

PubMed Central

Hodgkin's lymphoma accounts for ten percent of all lymphomas. In the United States, there are about 8000 new cases every year. This paper describes a case of lymphocyte-rich Hodgkin's lymphoma (LRHL) manifested by autoimmune hemolytic anemia (AIHA). A 27-year-old Israeli male presented with dizziness associated with one month of low-grade fevers and night sweats; he also complained of persistent cough, pruritus, and ten-pound weight lost during this time. The CBC revealed hemoglobin of 5.9?gm/dL, and direct Coomb's test detected multiple nonspecific antibodies consistent with the diagnosis of AIHA. Chest, abdomen, and pelvic CT scan showed mediastinal lymphadenopathy and splenomegaly. Lymph node biopsy revealed classic LRHL. AIHA resolved after completion of the first cycle of chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD); after six cycles, he went into complete remission. Although infrequent, AIHA can be responsible for the presenting symptoms of HL.

Hurtado-Cordovi, Jorge M.; Verma, Vaibhav; Gotlieb, Vladimir; Frieri, Marianne

2011-01-01

90

PHYSICAL PROPERTIES OF THE RED CELL AGGLUTININS IN ACQUIRED HEMOLYTIC ANEMIA  

PubMed Central

The sera of 8 patients with acquired hemolytic anemia associated with elevated levels of cold agglutinins were studied by various procedures of zone electrophoresis. The agglutinating activity was found associated with proteins of variable mobility in the different cases. The majority represented "fast" ?-globulins. The 4 sera with the highest titers of cold agglutinins showed distinguishable abnormal electrophoretic components. The titers correlated with the height of the abnormal components. Ultracentrifugal analysis of the electrophoretic fractions indicated that the cold agglutinins were associated with proteins having a sedimentation coefficient of approximately 19 S. The abnormal component from the serum with the highest biological activity showed almost no contamination with lower molecular weight proteins. The amount of 19 S material found correlated with the titer of agglutinating activity. The high molecular weight character of the cold agglutinins was confirmed by procedures of density gradient zone centrifugation. The biological activity sedimented with proteins of the 19 S class in all the sera including those of relatively low titer with which no abnormal electrophoretic components were observed. Dissociation of the abnormal high molecular weight components was possible by means of certain sulfhydryl compounds. This resulted in disappearance of cold agglutinin activity. Some of the cases could be classified as macroglobulinemias because of the very large content of high molecular weight components. However, the same disease picture occurred without recognizable elevation of these components. The sera of 3 patients with severe acquired hemolytic anemia of the warm type associated with warm incomplete antibodies failed to show similar abnormal electrophoretic components and the antibody activity sedimented with proteins of the 7 S class.

Fudenberg, H. H.; Kunkel, H. G.

1957-01-01

91

Direct antiglobulin test reactive with complement only in warm type autoimmune hemolytic anemia.  

PubMed

Direct antiglobulin test (DAT) with only complement detected on red blood cells is a rare laboratory finding, and its significance in the setting of warm autoimmune hemolytic anemia (AIHA) is controversial. During 2 years (2003-2004) 277 patients with positive DAT were recorded in the blood bank registries, 17 of them had DAT reactive with C3 alone with no cold agglutinin or other nonimmune causes for hemolysis diagnosed. Red cell eluate disclosed small amounts of IgG in two patients. In nine patients no signs of clinical hemolysis were found, however, all these patients had underlying conditions that are known to be associated with red cells autoantibodies (autoimmune disorder or malignancy). Eight patients developed AIHA, seven of them with severe hemolysis. Three patients had idiopathic AIHA, and the others have been diagnosed with infectious, lymphoproliferative and autoimmune disorders. In two patients with acute infection the hemolytic process spontaneously resolved, three responded to corticosteroid therapy, while three patients were refractory to two lines of drug therapy and underwent splenectomy. Reticulocytopenia was found in four patients. Our results emphasize that AIHA with DAT reactive with complement alone is a rare disorder and might be accompanied by severe, refractory to conventional treatment and life-threatening hemolysis. PMID:18983301

Shvidel, L; Shtalrid, M; Duek, A; Haran, M; Berrebi, A; Sigler, E

2008-12-01

92

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

93

Successful treatment of autoimmune hemolytic anemia associated with multicentric Castleman disease by anti-interleukin-6 receptor antibody (tocilizumab) therapy.  

PubMed

We describe herein the successful treatment of severe autoimmune hemolytic anemia (AIHA) in a patient with multicentric Castleman disease (MCD) by humanized anti-interleukin-6 (IL-6) receptor antibody (tocilizumab) therapy. Inflammatory anemia is commonly reported; however, AIHA is a very rare complication of MCD. In 1996, a 45-year-old Japanese woman was referred to our hospital because of generalized lymphadenopathy, anemia and skin eruptions. Lymph node biopsy demonstrated MCD. She was treated with prednisolone (1 mg/kg/day), which improved the anemia and skin eruptions. In 2009, she suddenly developed Coombs-positive hemolytic anemia. The blood count was as follows: hemoglobin 4.7 g/dl, platelets 490 × 10(9)/l and white blood cell count 9.8 × 10(9)/l. Both direct and indirect Coombs' tests were strongly positive. She was treated with 8 mg/kg tocilizumab every 2 weeks. One month later, her hemoglobin levels rose dramatically to 10.9 g/dl and her haptoglobin level, hypergammaglobulinemia and clinical symptoms had also markedly improved. To the best of our knowledge, this is the first report of the efficacy of tocilizumab in AIHA associated with MCD. The well-established role of IL-6 in the pathogenesis of MCD may have been responsible for the improvement in the AIHA associated with MCD. Anti-IL-6 receptor antibody treatment could be an attractive therapeutic approach for AIHA associated with MCD. PMID:21757886

Yuzuriha, Akinori; Saitoh, Takayuki; Koiso, Hiromi; Mitsui, Takeki; Uchiumi, Hideki; Yokohama, Akihiko; Handa, Hiroshi; Kojima, Masaru; Tsukamoto, Norifumi; Karaswa, Masamitsu; Murakami, Hirokazu; Nojima, Yoshihisa

2011-07-14

94

B-lymphocyte reconstitution after repeated rituximab treatment in a child with steroid-dependent autoimmune hemolytic anemia  

PubMed Central

We report the detailed long-term reconstitution of B-lymphocyte subpopulations, immunoglobulins, and specific antibody production after two courses of rituximab in a young, previously healthy girl with steroid-dependent autoimmune hemolytic anemia. B-lymphocyte subpopulations were surprisingly normal directly after reconstitution. However, there was a slower reconstitution after the second rituximab course, especially of non-switched and switched memory B-lymphocytes, and a temporary decline in IgM below age-matched reference values.

van der Linde, Annelieke A.A.; Schatorje, Ellen J.H.; van der Weij, Annemieke M.; Gemen, Eugenie F.A.; de Vries, Esther

2011-01-01

95

Incipient Coombs' test negative autoimmune hemolytic anemia precedes non-Hodgkin's lymphoma.  

PubMed

The cases of lymphoma accompanied or preceded by Coombs' test positive autoimmune hemolytic anemia (AIHA) have been reported. However, Coombs' test negative AIHA prior to the diagnosis of lymphoma was rarely described. Herein, this article reports a case of non-Hodgkin's lymphoma (NHL) preceded about 1.5 years by Coombs test negative AIHA. A woman aged 69 was diagnosed with HA based on the history and laboratory tests. Further studies revealed that this patient was negative with Coombs' test for IgG, IgM, IgA and C3. After all possible causes of HA, especially malignancies were ruled out, the patient was diagnosed with Coombs' test negative AIHA and treated with prednisolone. The patient responded well initially to steroid treatment. Two recurrences of acute HA were presented at time of 10 months post steroid cessation, and immediately after an attempt to withdraw steroid, respectively, but the hemolysis was effectively controlled by reinstitution of prednisolone. At third recurrence, however, the patient was no longer responding to steroid, and was found with cervical lymphadenopathy. Coombs' test for IgG, IgM, IgA and C3 remained negative. B cell NHL was diagnosed by pathology. After receiving 6 cycles of CHOP chemotherapy, the patient was lymphoma free, but the hemolysis was not improved, however, which was effectively controlled by the following low dose-rituximab (RTX) therapy. The patient was still kept in a remission of lymphoma free of anemia. In conclusion, this report presented a very rare case of NHL with Coombs' test negative AIHA as initial major clinical manifestation. PMID:22391174

Wan, Sui-Gui; Lin, Yang; Xia, Chang-Qing; Zhao, Hong; Xu, Juan

2012-02-01

96

Fragmentation and myelin formation in hereditary xerocytosis and other hemolytic anemias.  

PubMed

Erythrocytes from a heterogeneous group of hemolytic anemias have been found to release acetylcholinesterase-enriched fragments and show myelin forms during ATP depletion in vitro. The highest amount of fragmentation was found in hereditary spherocytosis and xerocytosis, two inherited membrane defects. Our data suggest ATP depletion plays a role in producing fragmentation or myelin forms. The addition of external CaCl2 1 mM had no effect on the degree of fragmentation. However, propranolol hydrochloride, a cationic anesthetic that does not prevent ATP depletion, inhibited fragmentation and the appearance of myelin forms in both hereditary spherocytes and xerocytes. A more detailed study of the xerocyte fragments showed that they had the same protein composition as those from normal red cells, primarily integral membrane proteins and glycoproteins. The red cells from patients with PNH and G6PD deficiency had the shortest survival in vivo (51Cr) and produced the smallest amount of fragmentation and myelin forms in vitro, whereas xerocytosis with only mild to moderate hemolysis in vivo was associated with the highest amount of myelin forms and membrane fragments in vitro. PMID:687829

Snyder, L M; Lutz, H U; Sauberman, N; Jacobs, J; Fortier, N L

1978-10-01

97

Autoimmune hemolytic anemia and nodular lymphocyte-predominant hodgkin lymphoma: a rare association.  

PubMed

Autoimmune hemolytic anemia (AIHA) has been associated with chronic lymphocytic leukemia, non-Hodgkin lymphoma, and classical Hodgkin lymphoma, but to the best of our knowledge, the association of AIHA and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) has not been reported previously. A 20-year-old woman presented with conjunctival jaundice, fever, asthenia, and hemoglobin 9.2?g/dL revealing IgG-mediated warm antibody AIHA. Computed tomography (CT) scan and positron-emission tomography (PET) scan showed mediastinal and axillary lymph nodes with increased [(18)F]-fluorodeoxyglucose uptake. A mediastinal lymph node was biopsied during mediastinoscopy, and NLPHL was diagnosed by an expert hematopathologist. The hemoglobin level declined to 4.6?g/dL. The treatment consisted of four 28-day cycles of R-ABVD (rituximab 375?mg/m(2) IV, adriamycin 25?mg/m(2) IV, bleomycin 10?mg/m(2) IV, vinblastine 6?mg/m(2) IV, and dacarbazine 375?mg/m(2) IV, each on days 1 and 15). Prednisone was progressively tapered over 10 weeks. After the first chemotherapy cycle, the hemoglobin level rose to 12?g/dL. After the four cycles, PET and CT scans showed complete remission (CR). At the last followup (4 years), AIHA and NLPHL were in sustained CR. PMID:23710384

Salmeron, Géraldine; Molina, Thierry Jo; Fieschi, Claire; Zagdanski, Anne-Marie; Brice, Pauline; Sibon, David

2013-04-28

98

Autoimmune Hemolytic Anemia and Nodular Lymphocyte-Predominant Hodgkin Lymphoma: A Rare Association  

PubMed Central

Autoimmune hemolytic anemia (AIHA) has been associated with chronic lymphocytic leukemia, non-Hodgkin lymphoma, and classical Hodgkin lymphoma, but to the best of our knowledge, the association of AIHA and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) has not been reported previously. A 20-year-old woman presented with conjunctival jaundice, fever, asthenia, and hemoglobin 9.2?g/dL revealing IgG-mediated warm antibody AIHA. Computed tomography (CT) scan and positron-emission tomography (PET) scan showed mediastinal and axillary lymph nodes with increased [18F]-fluorodeoxyglucose uptake. A mediastinal lymph node was biopsied during mediastinoscopy, and NLPHL was diagnosed by an expert hematopathologist. The hemoglobin level declined to 4.6?g/dL. The treatment consisted of four 28-day cycles of R-ABVD (rituximab 375?mg/m2 IV, adriamycin 25?mg/m2 IV, bleomycin 10?mg/m2 IV, vinblastine 6?mg/m2 IV, and dacarbazine 375?mg/m2 IV, each on days 1 and 15). Prednisone was progressively tapered over 10 weeks. After the first chemotherapy cycle, the hemoglobin level rose to 12?g/dL. After the four cycles, PET and CT scans showed complete remission (CR). At the last followup (4 years), AIHA and NLPHL were in sustained CR.

Salmeron, Geraldine; Molina, Thierry Jo; Fieschi, Claire; Zagdanski, Anne-Marie; Brice, Pauline

2013-01-01

99

Autoimmune hemolytic anemia and autoimmune thrombocytopenia at diagnosis and during follow-up of Hodgkin lymphoma.  

PubMed

Autoimmune hemolytic anemia and thrombocytopenia (AIHA/AITP) frequently complicate the course of non-Hodgkin lymphomas, especially low-grade, but they are very rarely observed in Hodgkin lymphoma (HL). Consequently the frequency and the profile of patients with HL-associated AIHA/AITP have not been well defined. Among 1029 patients with HL diagnosed between 1990 and 2010, two cases of AIHA (0.19%) and three of AITP (0.29%) were identified at the presentation of disease. These patients were significantly older, and more frequently had features of advanced disease and non-nodular sclerosing histology, compared to the majority of patients, who did not have autoimmune cytopenias at diagnosis. ABVD combination chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine) provided effective control of HL and the autoimmune condition as well. During approximately 6600 person-years of follow-up for the remaining 1024 patients, seven (0.7%) patients developed autoimmune cytopenias (three AITP, three AIHA, one autoimmune pancytopenia) for a 10- and 15-year actuarial incidence of 0.95% and 1.40%, respectively. Their features did not differ compared to the general population of adult HL. In this large series of consecutive, unselected patients, those who presented with autoimmune cytopenias had a particular demographic and disease-related profile. In contrast, patients developing autoimmune cytopenias during follow-up did not appear to differ significantly from those who did not. PMID:22280533

Dimou, Maria; Angelopoulou, Maria K; Pangalis, Gerassimos A; Georgiou, Georgios; Kalpadakis, Christina; Pappi, Vassiliki; Tsopra, Olga; Koutsoukos, Konstantinos; Zografos, Eleftherios; Boutsikas, George; Moschogianni, Maria; Vardounioti, Ioanna; Petevi, Kyriaki; Karali, Vassiliki; Kanellopoulos, Alexandros; Ntalageorgos, Themis; Yiakoumis, Xanthis; Bartzis, Vasiliki; Bitsani, Aikaterini; Pessach, Elias; Efthimiou, Anna; Korkolopoulou, Penelope; Rassidakis, George; Kyrtsonis, Marie-Christine; Patsouris, Efstratios; Meletis, John; Panayiotidis, Panayiotis; Vassilakopoulos, Theodoros P

2012-04-02

100

Critical role of Th17 cells in development of autoimmune hemolytic anemia.  

PubMed

Autoimmune hemolytic anemia (AIHA) is defined as the increased destruction of red blood cells (RBCs) in the presence of anti-RBC autoantibodies with or without complement activation. However, the underlying mechanism for the development of AIHA remains largely unclear. In this study, we carefully evaluated the potential role of Th17 cells in the development of AIHA. We found an elevated frequency of Th17 cells in patients with AIHA, which were closely correlated with their disease activity, including the level of anti-RBC IgG antibodies, hemoglobin, serum C3, and lactate dehydrogenase activity. Furthermore, we observed that interleukin (IL)-17 was also closely correlated with the disease activity in AIHA patients. To further elucidate the potential role of Th17 cells in induction of AIHA, we used the Marshall-Clarke and Playfair model of murine AIHA. Notably, we found that Th17 cells affected development of AIHA by enhancing the adaptive humoral responses. Specifically, we found that adoptive transfer of Th17 cells heightened the initial anti-rat RBC antibody responses and concomitantly increased the onset of AIHA. In addition, in vivo neutralization of IL-17 abrogated the development of AIHA, while initiation of anti-rat RBC IgG responses and induction of AIHA in IL-17(-/-) mice were impaired. Our findings suggest that Th17 cells contribute to the development of AIHA, which could facilitate our better understanding of AIHA pathogenesis and provide clues to developing novel forms of immunotherapy against AIHA. PMID:22960264

Xu, Lin; Zhang, Tenglong; Liu, Zhongmin; Li, Qinchuan; Xu, Zengguang; Ren, Tao

2012-09-05

101

Generalized hexokinase deficiency in the blood cells of a patient with nonspherocytic hemolytic anemia.  

PubMed

In a patient with nonspherocytic hemolytic anemia, a hexokinase deficiency was detected in the red cells (residual activity about 25% of normal) and in blood platelets (20%-35% of normal activity). Although the total hexokinase activity in lymphocytes was normal, the amount of hexokinase type I was decreased to about 50% of normal. However, the deficiency was compensated for by the appearance of type III hexokinase. Compartmentation studies with controlled digitonin-induced cell lysis showed that this type III enzyme was localized in the cytosol, while almost all hexokinase activity in normal lymphocytes is particulate. No abnormal lymphocyte functions could be detected. The patient was homozygous for the defect. The parents and three of five sibs of the patient were apparently heterozygous with residual activities of 50%-67% of normal in their red cells, but did not show any clinical signs of hexokinase deficiency. The variant enzyme had a slightly decreased affinity for MgATP2- and a strongly increased inhibition constant for glucose-1,6-P2. Affinity for glucose, heat stability, and pH optimum were normal. In the electrophoretic pattern of red cell hexokinase, only one subtype of hexokinase I could be detected, while in normal red cells, at least three subtypes are present. In the heterozygous individuals, no enzymatic abnormalities could be detected, except for an aberration in the electropherogram of one sib. PMID:6848140

Rijksen, G; Akkerman, J W; van den Wall Bake, A W; Hofstede, D P; Staal, G E

1983-01-01

102

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

PubMed

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

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

2011-03-15

103

GLUT1 mutations are a cause of paroxysmal exertion-induced dyskinesias and induce hemolytic anemia by a cation leak  

PubMed Central

Paroxysmal dyskinesias are episodic movement disorders that can be inherited or are sporadic in nature. The pathophysiology underlying these disorders remains largely unknown but may involve disrupted ion homeostasis due to defects in cell-surface channels or nutrient transporters. In this study, we describe a family with paroxysmal exertion-induced dyskinesia (PED) over 3 generations. Their PED was accompanied by epilepsy, mild developmental delay, reduced CSF glucose levels, hemolytic anemia with echinocytosis, and altered erythrocyte ion concentrations. Using a candidate gene approach, we identified a causative deletion of 4 highly conserved amino acids (Q282_S285del) in the pore region of the glucose transporter 1 (GLUT1). Functional studies in Xenopus oocytes and human erythrocytes revealed that this mutation decreased glucose transport and caused a cation leak that alters intracellular concentrations of sodium, potassium, and calcium. We screened 4 additional families, in which PED is combined with epilepsy, developmental delay, or migraine, but not with hemolysis or echinocytosis, and identified 2 additional GLUT1 mutations (A275T, G314S) that decreased glucose transport but did not affect cation permeability. Combining these data with brain imaging studies, we propose that the dyskinesias result from an exertion-induced energy deficit that may cause episodic dysfunction of the basal ganglia, and that the hemolysis with echinocytosis may result from alterations in intracellular electrolytes caused by a cation leak through mutant GLUT1.

Weber, Yvonne G.; Storch, Alexander; Wuttke, Thomas V.; Brockmann, Knut; Kempfle, Judith; Maljevic, Snezana; Margari, Lucia; Kamm, Christoph; Schneider, Susanne A.; Huber, Stephan M.; Pekrun, Arnulf; Roebling, Robert; Seebohm, Guiscard; Koka, Saisudha; Lang, Camelia; Kraft, Eduard; Blazevic, Dragica; Salvo-Vargas, Alberto; Fauler, Michael; Mottaghy, Felix M.; Munchau, Alexander; Edwards, Mark J.; Presicci, Anna; Margari, Francesco; Gasser, Thomas; Lang, Florian; Bhatia, Kailash P.; Lehmann-Horn, Frank; Lerche, Holger

2008-01-01

104

Inborn Anemias in Mice.  

National Technical Information Service (NTIS)

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 alpha ...

S. E. Bernstein J. E. Barker E. S. Russell

1981-01-01

105

Primaquine-induced hemolytic anemia: role of splenic macrophages in the fate of 5-hydroxyprimaquine-treated rat erythrocytes.  

PubMed

Primaquine-induced hemolytic anemia is known to result from premature sequestration of damaged (but intact) erythrocytes by the spleen. We have shown previously that a phenolic metabolite, 5-hydroxyprimaquine (5-HPQ), is a direct-acting hemolytic agent in rats, suggesting that 5-HPQ is a mediator of the hemolytic response to primaquine. To investigate the fate of erythrocytes in vivo after in vitro exposure to 5-HPQ, rat (51)Cr-labeled erythrocytes were incubated with hemolytic concentrations of 5-HPQ and then readministered intravenously to rats. The time course of loss of radioactivity from blood and uptake into the spleen and liver was measured. In rats given 5-HPQ-treated erythrocytes, an increased rate of removal of radioactivity from the circulation was observed as compared with the vehicle control. The loss of blood radioactivity was accompanied by a corresponding increase in radioactivity appearing in the spleen but not in the liver. When rats were pretreated with clodronate-loaded liposomes to deplete splenic macrophages, there was a decreased rate of removal of radioactivity from the circulation and a markedly diminished uptake into the spleen. A role for phagocytic removal of 5-HPQ-treated red cells was confirmed in vitro using the J774A.1 macrophage cell line. Furthermore, depletion of red cell GSH with diethyl maleate significantly enhanced in vitro phagocytosis of 5-HPQ-treated red cells. The data indicate that splenic macrophages are responsible for removing 5-HPQ-treated red cells and support the postulate that this metabolite is a contributor to the hemolytic anemia induced after administration of the parent compound. PMID:16099929

Bowman, Zachary S; Jollow, David J; McMillan, David C

2005-08-12

106

Clinical features and outcomes of autoimmune hemolytic anemia: a retrospective analysis of 32 cases  

PubMed Central

Background There has been no report on the clinical features or natural history of autoimmune hemolytic anemia (AIHA) in the Korean adult population. This study retrospectively analyzed the clinical characteristics and long-term outcomes of AIHA in the Korean adults. Methods Patients newly diagnosed with AIHA between January 1994 and December 2010 at Chungnam National University Hospital were enrolled. Patient characteristics at diagnosis, response to treatment, and the natural course of the disease were documented. Results Thirty-two patients (31 females and 1 male) with a median age of 48 years (range, 17-86) were enrolled. Of these, 21.9% were initially diagnosed with secondary AIHA. Thirteen patients (40.6%) were initially diagnosed with Evans' syndrome. Of the 29 patients who were placed on therapy, 27 (93.1%) showed a partial response or better. Nevertheless, 1 year after initiating treatment, 80% of the patients were still treatment-dependent. During follow-up (median length 14 months; range, 0.5-238), 14 of 25 patients (56.0%) who were initially diagnosed with primary warm antibody AIHA were found to have systemic lupus erythematosus (SLE). Median time to conversion to SLE was 8.0 months (95% CI, 4.3-11.7), and the probabilities of conversion at 12 and 24 months were 63% and 91%, respectively. Younger age (<60 years) and a positive fluorescent anti-nuclear antibody test were associated with a higher probability of SLE conversion (P=0.01 and P<0.001, respectively). Conclusion Primary AIHA is rare. Regular, vigilant testing for SLE is required in patients initially diagnosed with AIHA.

Baek, Seung-Woo; Lee, Myung-Won; Ryu, Hae-Won; Lee, Kyu-Seop; Song, Ik-Chan; Lee, Hyo-Jin; Yun, Hwan-Jung; Kim, Samyong

2011-01-01

107

[Autoimmune hemolytic anemia associated with B-cell chronic lymphoproliferative disorders].  

PubMed

This study was purposed to investigate the clinical characteristics of B-cell chronic lymphoproliferative disorders (B-CLPD) complicated by autoimmune hemolytic anemia (AIHA) so as to improve the understanding of this disease. The clinical characteristics, laboratory data, therapy and outcome of 14 patients suffering from B-CLPD complicated by AIHA were restrospectively analyzed in Wuxi People Hospital and the First Affiliated Hospital of Nanjing Medical University from 2000 to 2012. The results showed that 9 cases of the 14 patients were patients with chronic lymplocytic leukemia (CLL), 5 cases were patients with lymphoma, at time of hemolysis the median level of hemoglobine was 61 (33 - 84)g/L, the median ratio of reticulocytes was 12.0 (3.1 - 35.0)%, the positive rate of Coombs test was 100%. 1 case received corticosteroid alone, 5 cases were treated with chemotherapy combined with corticosteroid, 8 cases were treated with immunochemotherapy rituximab combined with corticosteroid. Overall response rate was 100%, in which CR was 78.6% (11/14), PR was 21.4% (3/14). The follow-up for these patients were performed to now, 35.7% (5/14) patients relapsed with hemolysis again, but they showed therapeutic response to treatment with above-menthoned therapy. From patients treated with rituximab alone, only 1 patient relapsed. Among 14 patients, 6 cases died, 1 case was lost, the other cases are still alive. It is concluded that the AIHA is the commonest complication of B-CLPD, it can be observed at different stages of B-CLPD, the treatment with corticosteroids can give well therapeutic effect for these patients, but the long time CR is lower, the rituximab has been confirmed to be effective for B-CLPD complicated by AIHA. PMID:23815912

Zhuang, Yun; Fan, Lei; Shen, Yun-Feng; Xu, Wei; Li, Jian-Yong

2013-06-01

108

A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura.  

PubMed

Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying malignancy. We report a case of ITP and Coombs' negative AIHA associated with diffuse large B-cell lymphoma. PMID:22500164

Park, So Yeon; Kim, Soyon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

2012-03-31

109

A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura  

PubMed Central

Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying malignancy. We report a case of ITP and Coombs' negative AIHA associated with diffuse large B-cell lymphoma.

Park, So Yeon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

2012-01-01

110

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

PubMed Central

Introduction Mixed warm and cold autoimmune hemolytic anemia runs a chronic course with severe intermittent exacerbations. Therapeutic options for the treatment of hemolysis associated with autoimmune hemolytic anemia are limited. There have been only two reported cases of the effective use of rituximab in the treatment of patients with mixed autoimmune hemolytic anemia. We report a case of severe mixed autoimmune hemolytic anemia that did not respond to steroids and responded to four weekly doses of rituximab (one cycle). Case presentation A 62-year-old Caucasian man presented with dyspnea, jaundice and splenomegaly. His blood work revealed severe anemia (hemoglobin, 4.9 g/dL) with biochemical evidence of hemolysis. Exposure to cold led to worsening of the patient's hemolysis and hemoglobinuria. A direct antiglobulin test was positive for immunoglobulin G and complement C3d, and cold agglutinins of immunoglobulin M type were detected. A bone marrow biopsy revealed erythroid hyperplasia. A positron emission tomographic scan showed no sites of pathologic uptake. There was no other evidence of a lymphoid or myeloid disorder. Initial therapy consisted of avoidance of cold, intravenous methylprednisolone and a trial of plasmapheresis. However, there was no clinically significant response, and the patient continued to be transfusion-dependent. He was then started on 375 mg/m2/week intravenous rituximab therapy. After two treatments, his hemoglobin stabilized and the transfusion requirement diminished. Rituximab was continued for a total of four weeks and led to the complete resolution of his hemolytic anemia and associated symptoms. At the patient's last visit, about two years after the initial rituximab treatment, he continued to be in complete remission. Conclusion To the best of our knowledge, this is the first reported case of mixed-type autoimmune hemolytic anemia that did not respond to steroid therapy but responded completely to only one cycle of rituximab. The previous two reports of rituximab use in mixed autoimmune hemolytic anemia described an initial brief response to steroids and the use of rituximab at the time of relapse. In both of these case reports, the response to one cycle of rituximab was short-lived and a second cycle of rituximab was required. Our case report demonstrates that severe hemolysis associated with mixed autoimmune hemolytic anemia can be unresponsive to steroid therapy and that a single cycle of rituximab may lead to prompt and durable complete remission.

2011-01-01

111

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

Microsoft Academic Search

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

E. J. Victoria; S. W. Pierce; M. J. Branks; S. P. Masouredis

1990-01-01

112

Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome).  

PubMed

Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) associated with Evans syndrome; combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) has rarely been reported. We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. Diagnose of PBC-AIH overlap was made by clinical, serological and histological investigations. AIHA and ITP was identified with clinical-laboratory findings and bone marrow puncture. She was treated with IVIG followed by prednisolone and ursodeoxycholic acid. Hemoglobin-thrombocytes increased rapidly and transaminases improved at day 8. We have reported the first case in the literature with AIH-PBC overlap syndrome concurrent by ITP and AIHA which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including AIH, PBC, ITP and AIHA. PMID:23273499

Korkmaz, Huseyin; Bugdaci, Mehmet Sait; Temel, Tuncer; Dagli, Mehmet; Karabagli, Pinar

2012-12-27

113

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

PubMed Central

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

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

2013-01-01

114

End-stage liver cirrhosis with severe autoimmune hemolytic anemia, treated by blood type-incompatible living donor liver transplantation: a case report.  

PubMed

We present a case of successful living donor liver transplantation (LDLT) for liver cirrhosis caused by hepatitis B virus with severe autoimmune hemolytic anemia (AIHA) using an ABO-incompatible (ABOi) graft. The patient was a 47-year-old woman who had a history of ruptured esophageal varices, accumulation of intractable ascites, frequent hepatic encephalopathy and severe anemia, with a hemoglobin value of approximately 3 g/dL due to AIHA. We treated the patient by LDLT using an ABOi liver graft. The treatment strategy included anti-CD20 antibody, plasma exchange and transfusion before LDLT. The patient's anemia improved after surgery; she required only 2 units of irradiated red blood cell concentrates-leukocytes reduced. The patient was discharged from the hospital on postoperative day 35. Two years after surgery, the patient still shows normal hepatic and hematological findings. The immunomodulation protocol for ABOi LDLT was effective not only to avoid humoral reactions associated with ABOi LDLT, but also those associated with AIHA. PMID:21693332

Sanefuji, K; Ikegami, T; Nagata, S; Sugimachi, K; Gion, T; Uchiyama, H; Soejima, Y; Taketomi, A; Shirabe, K; Maehara, Y

2011-06-01

115

Low-dose rituximab in adult patients with idiopathic autoimmune hemolytic anemia: clinical efficacy and biologic studies.  

PubMed

This prospective study investigated the efficacy, safety, and response duration of low-dose rituximab (100 mg fixed dose for 4 weekly infusions) together with a short course of steroids as first- or second-line therapy in 23 patients with primary autoimmune hemolytic anemia (AIHA). The overall response was 82.6% at month +2, and subsequently stabilized to ? 90% at months +6 and +12; the response was better in warm autoimmune hemolytic anemia (WAIHA; overall response, 100% at all time points) than in cold hemagglutinin disease (CHD; average, 60%); the relapse-free survival was 100% for WAIHA at +6 and +12 months versus 89% and 59% in CHD, respectively, and the estimated relapse-free survival at 2 years was 81% and 40% for the warm and cold forms, respectively. The risk of relapse was higher in CHD and in patients with a longer interval between diagnosis and enrollment. Steroid administration was reduced both as cumulative dose (? 50%) and duration compared with the patient's past history. Treatment was well tolerated and no adverse events or infections were recorded; retreatment was also effective. The clinical response was correlated with amelioration biologic markers such as cytokine production (IFN-?, IL-12, TNF-?, and IL-17), suggesting that low-dose rituximab exerts an immunomodulating activity. This study is registered at www.clinicaltrials.gov as NCT01345708. PMID:22267606

Barcellini, Wilma; Zaja, Francesco; Zaninoni, Anna; Imperiali, Francesca Guia; Battista, Marta Lisa; Di Bona, Eros; Fattizzo, Bruno; Consonni, Dario; Cortelezzi, Agostino; Fanin, Renato; Zanella, Alberto

2012-01-20

116

Atypical hemolytic uremic syndrome due to factor H autoantibody.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a disease caused by pathologies in the alternative complement system. The prevalence of aHUS is 10% of all aHUS cases. The subgroup of aHUS designated as DEAP (DEficiency of CFHR Proteins and CFH Autoantibody Positive)-HUS because of autoantibody to complement factor H (CFH) and CFH-related protein deficiency is seen very rarely, and the prevalence is 6% of all aHUS cases in the literature. We present here a female patient with DEAP-HUS. A 7.5-year-old girl with recurrent attacks of HUS had low C3 level. We initiated plasmapheresis treatment. After further analysis of the complement system, the result was compatible with DEAP-HUS, so we initiated immunosuppressive treatment. There were also family members with deficiency of CFHR-1 and CFHR-3, but they had no CFH autoantibody and no symptoms of HUS. In atypical cases of HUS, we should investigate complement status, especially for factor H autoantibody, for which treatment options differ from those of the other types of aHUS. PMID:23692839

Uslu-Gökceo?lu, Arife; Do?an, Cagla Serpil; Comak, Elif; Koyun, Mustafa; Akman, Sema

117

Prolonged extracorporeal membrane oxygenation therapy for severe acute respiratory distress syndrome in a child affected by rituximab-resistant autoimmune hemolytic anemia: a case report  

Microsoft Academic Search

INTRODUCTION: Autoimmune hemolytic anemia in children younger than 2 years of age is usually characterized by a severe course, with a mortality rate of approximately 10%. The prolonged immunosuppression following specific treatment may be associated with a high risk of developing severe infections. Recently, the use of monoclonal antibodies (rituximab) has allowed sustained remissions to be obtained in the majority

Chiara Beretta; Veronica Leoni; Mario Renato Rossi; Momcilo Jankovic; Nicolo Patroniti; Giuseppe Foti; Ettore Biagi

2009-01-01

118

Comparative proteomics reveals deficiency of NHE-1 (Slc9a1) in RBCs from the beta-adducin knockout mouse model of hemolytic anemia.  

PubMed

Hemolytic anemia is one of the most common inherited disorders. To identify candidate proteins involved in hemolytic anemia pathophysiology, we utilized a label-free comparative proteomic approach to detect differences in RBCs from normal and beta-adducin (Add2) knock-out mice. We detected 7 proteins that were decreased and 48 proteins that were increased in the beta-adducin knock-out RBC ghost. Since hemolytic anemias are characterized by reticulocytosis, we compared reticulocyte-enriched samples from phenylhydrazine-treated mice with mature RBCs from untreated mice. Label-free analysis identified 47 proteins that were increased in the reticulocyte-enriched samples and 21 proteins that were decreased. Among the proteins increased in Add2 knockout RBCs, only 11 were also found increased in reticulocytes. Among the proteins decreased in Add2 knockout RBCs, beta- and alpha-adducin showed the greatest intensity difference, followed by NHE-1 (Slc9a1), the sodium-hydrogen exchanger. We verified these mass spectrometry results by immunoblot. This is the first example of a deficiency of NHE-1 in hemolytic anemia and suggests new insights into the mechanisms leading to fragile RBCs. Our use of label-free comparative proteomics to make this discovery demonstrates the usefulness of this approach as opposed to metabolic or chemical isotopic labeling of mice. PMID:21592827

Gilligan, Diana M; Finney, Greg L; Rynes, Eric; Maccoss, Michael J; Lambert, Amy J; Peters, Luanne L; Robledo, Raymond F; Wooden, Jason M

2011-05-18

119

[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

120

Hematopoietic effect of Bacillus subtilis-fermented antler extract on phenylhydrazine-induced hemolytic anemia in Sprague-Dawley rats.  

PubMed

This study examined the effect of fermentation on the ability of antler to act as a stimulator of hematopoietic activity. Hemolytic anemia was induced by phenylhydrazine (PHZ) in female Sprague-Dawley rats. The vehicle or antler extract (nonfermented or fermented) mixed in drinking water was administered from Days 2 to 15 after PHZ injection. On Day 15, red blood cell counts in the fermented antler group (6.33×10?/?L) were significantly higher than those in the nonfermented antler group (5.90×10?/?L) (P<.05), and rats treated with fermented antler extract tended to have higher hemoglobin compared with rats treated with nonfermented antler extract, but not significantly. In addition, rats treated with fermented antler extract had slightly lower serum erythropoietin levels compared with nonfermented antler extract, which were not statistically different from serum erythropoietin levels of nonanemic rats. We conclude therefore that the hematopoietic activity of antler might be increased by the fermentation process. PMID:22870931

Lee, Sang Hun; Suh, Hyung Joo; Lee, Hyun-Sun; Park, Yooheon; Park, Jang-Woo; Jung, Eun Young

2012-08-07

121

Myeloneuropathy and anemia due to copper malabsorption  

Microsoft Academic Search

Dietary deficiency of copper results in a progressive ataxic myelopathy in ruminants called swayback. Menkes disease is a human disease due to an inherited defect in copper absorption; survival into adulthood is typically not known to occur. We report a 63-year-old woman who was evaluated by us for a myeloneuropathy that occurred in the setting of copper malabsorption. Her neurological

Neeraj Kumar; Phillip A. Low

2004-01-01

122

Anemias  

Microsoft Academic Search

\\u000a Anemia is defined as a reduction in the red cell mass due to decreased production, increased loss\\/ decreased survival, or\\u000a increased destruction of red blood cells (RBCs). As most of the oxygen is transported by the RBCs to the body tissues, a reduction\\u000a in the red cell mass causes reduced oxygen supply to the body cells. Consequently, anemia is a

Rosalind Bryant

123

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.

2012-01-01

124

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

PubMed

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

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

2011-04-12

125

Myeloneuropathy and anemia due to copper malabsorption.  

PubMed

Dietary deficiency of copper results in a progressive ataxic myelopathy in ruminants called swayback. Menkes disease is a human disease due to an inherited defect in copper absorption; survival into adulthood is typically not known to occur. We report a 63-year-old woman who was evaluated by us for a myeloneuropathy that occurred in the setting of copper malabsorption. Her neurological deterioration stopped with copper supplementation. The limited literature on neurological manifestations of acquired copper deficiency suggests that the clinical presentation resembles the myeloneuropathy seen with vitamin B12 deficiency. PMID:15311353

Kumar, Neeraj; Low, Phillip A

2004-06-01

126

Diphenylhydantoin-induced severe yet reversible anemia during pregnancy.  

PubMed

Diphenylhydantoin (DPH) therapy, often used in treating epileptic seizures, can cause anemia in some patients. A 26-year-old female suffered from convulsions due to encephalitis and was placed on DPH therapy. About two months after the initiation of DPH therapy, her hemoglobin level was 3.8 g/dL. Her anemia improved after the discontinuation of DPH, confirming that the anemia was caused by DPH. Pure red-cell aplasia (PRCA) combined with hemolytic anemia was indicated by results such as erythroid aplasia, an increased LDH level, and a decreased haptoglobin level. PRCA complicated by hemolytic anemia could be responsible for anemia associated with DPH. PMID:21088360

Sugaya, Akinori; Nakamagoe, Kiyotaka; Okoshi, Yasushi; Obata-Yasuoka, Mana; Tamaoka, Akira

2010-11-15

127

[Autoimmune hemolytic anemia in a case of chronic hepatitis type C 56 weeks after initiation of second line treatment with pegylated interferon alpha2b/ribavirin combination therapy].  

PubMed

A 49-year-old man with chronic type C hepatitis had agreed to undergo pegylated interferon alpha2b/ribavirin (RBV) combination therapy during 48 weeks, but his hepatitis relapsed. Despite of second line treatment with the same combination, 56 weeks later, his hemoglobin decreased and the dose of RBV was decreased. He was then admitted to our hospital because of increasing anemia and this combination therapy was stopped. The results of blood chemistry and immunological examination revealed he had contracted autoimmune hemolytic anemia (AIHA). In cases of deterioration of anemia during this combination, we must discuss about not only RBV-induced hemolytic anemia but also AIHA. PMID:21891998

Nishino, Ryuhei; Ikeda, Naoki; Unoura, Masashi

2011-09-01

128

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

PubMed

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

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

2012-10-18

129

Total Blood Volume Is Maintained in Nonhydropic Fetuses with Severe Hemolytic Anemia  

Microsoft Academic Search

Objective: Fetal alloimmune anemia is associated with increased blood flow velocities and cardiomegaly. In severe cases, hydrops can develop. We investigated whether the decrease of red blood cell volume is associated with a reduction or expansion of plasma volume. Methods: In 86 alloimmunized fetuses that received a first intrauterine transfusion, we calculated fetal total blood volumes (i.e. fetoplacental blood volumes)

S. A. Pasman; C. P. Bil-van den Brink; M. A. Kamping; P. N. Adama van Scheltema; D. Oepkes; F. P. H. A. Vandenbussche

2009-01-01

130

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

PubMed

Hemolysis can saturate the hemoglobin (Hb)/heme scavenging system, resulting in increased circulating cell-free Hb (CF-Hb) in hereditary and acquired hemolytic disease. While recent studies have suggested a central role for intravascular hemolysis and CF-Hb in the development of vascular dysfunction, this concept has stimulated considerable debate. This highlights the importance of determining the contribution of CF-Hb to vascular complications associated with hemolysis. Therefore, a novel Hb-binding peptide was synthesized and linked to a small fragment of apolipoprotein E (amino acids 141-150) to facilitate endocytic clearance. Plasma clearance of hE-Hb-b10 displayed a rapid phase t(1/2) of 16 min and slow phase t(1/2) of 10 h, trafficking primarily through the liver. Peptide hE-Hb-B10 decreased CF-Hb in mice treated with phenylhydrazine, a model of acute hemolysis. Administration of hE-Hb-B10 also attenuated CF-Hb in two models of chronic hemolysis: Berkeley sickle cell disease (SS) mice and mice with severe hereditary spherocytosis (HS). The hemolytic rate was unaltered in either chronic hemolysis model, supporting the conclusion that hE-Hb-B10 promotes CF-Hb clearance without affecting erythrocyte lysis. Interestingly, hE-Hb-B10 also decreased plasma ALT activity in SS and HS mice. Although acetylcholine-mediated facialis artery vasodilation was not improved by hE-Hb-B10 treatment, the peptide shifted vascular response in favor of NO-dependent vasodilation in SS mice. Taken together, these data demonstrate that hE-Hb-B10 decreases CF-Hb with a concomitant reduction in liver injury and changes in vascular response. Therefore, hE-Hb-B10 can be used to investigate the different roles of CF-Hb in hemolytic pathology and may have therapeutic benefit in the treatment of CF-Hb-mediated tissue damage. PMID:23125208

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

2012-11-02

131

IgA-mediated human autoimmune hemolytic anemia as a result of hemagglutination in the spleen, but independent of complement activation and Fc?RI.  

PubMed

Autoimmune hemolytic anemia (AIHA) due to warm-acting IgA autoantibodies is rare. We explored the pathogenic mechanisms underlying destruction of red blood cells (RBCs) in a patient with severe AIHA mediated exclusively by polymeric immunoglobulin A (pIgA) anti-Band 3 autoantibodies. The follow-up period was 17 months. RBCs were not destroyed by complement activation as no deposition of complement was observed on the patient's RBCs. pIgA eluted from the patient's RBCs did not induce RBC destruction through phagocytosis by monocytes or antibody-dependent cell-mediated cytotoxicity by natural killer cells. Induction of eryptosis (ie, RBC apoptosis) due to direct alteration of the RBC membrane by pIgA autoantibodies was also excluded. By contrast, upon incubation with pIgA-opsonized RBCs, substantial RBC membrane transfers (ie, trogocytosis) to monocytes were observed that might contribute to RBC immune destruction. This effect was poorly inhibited by blockers of Fc receptors, excluding a major contribution of Fc?RI to this process. Histologic analysis revealed a massive accumulation of agglutinated RBCs with little sign of erythrophagocytosis in the spleen. These results, together with the efficacy of splenectomy 17 months after AIHA onset, suggest that the trapping and subsequent sequestration of agglutinated RBCs in the spleen are the principal pathogenic mechanisms of pIgA-mediated AIHA. PMID:20644119

Chadebech, Philippe; Michel, Marc; Janvier, Daniel; Yamada, Kazunori; Copie-Bergman, Christiane; Bodivit, Gwellaouen; Bensussan, Armand; Fournie, Jean-Jacques; Godeau, Bertrand; Bierling, Philippe; Izui, Shozo; Noizat-Pirenne, France

2010-07-19

132

[A hemolytic transfusion reaction due to Anti-Ku antibody in a patient with Knull phenotype: the first case in Korea].  

PubMed

Knull phenotype completely lacks all Kell system antigens. Anti-Ku antibody is seen in immunized persons with Knull phenotype by transfusion or pregnancy. It can cause a fatal hemolytic transfusion reaction. A 66-yr-old male patient with liver cirrhosis visited emergency center due to acute bleeding. The patient was at hypovolemic shock status: his blood pressure was 80/50 mmHg, pulse rate was 110/min and hemoglobin level was 4.4 g/dL. Because of the presence of antibody against high incidence antigen, we could not find any compatible blood for the patient. Nevertheless, 4 units of packed RBCs had to be transfused. Moderate hemolytic transfusion reaction was developed after transfusion. At endoscopic examination, blood was spurting from gastric cardiac varix. Endoscopic histoacryl injection was tried, and bleeding was successfully controlled. After bleeding stopped, he was managed for anemia using steroid and other medical therapy instead of transfusion. His hemoglobin level was improved to 7.7 g/dL at the time of discharge. Later he has been proved to have a Knull phenotype, which is very rare, and anti-Ku antibody. This report is the first case of anti-Ku in a Knull phenotype person in Korea, who experienced a moderate hemolytic transfusion reaction. PMID:19571622

Kang, Min Gu; Lim, Young Ae; Lee, Kee Myung

2009-06-01

133

Anemia  

MedlinePLUS

... several other rarer forms of anemia, such as thalassemia and anemias caused by defective hemoglobin. Risk factors ... also is used to treat sickle cell anemia. Thalassemia. This anemia may be treated with blood transfusions, ...

134

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

135

A deep intronic mutation in the ankyrin-1 gene causes diminished protein expression resulting in hemolytic anemia in mice.  

PubMed

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

2013-10-03

136

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–induced condition. Such therapy may prove useful in treatment of human disorders such as sideroblastic anemia, which SOD2 deficiency most closely resembles.

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

137

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

2012-10-31

138

Rituximab is an effective and safe therapeutic alternative in adults with refractory and severe autoimmune hemolytic anemia.  

PubMed

Rituximab-induced B-cell depletion has been proven to be a useful therapy for autoimmune hemolytic anemia (AIHA). The aim of this retrospective study was to evaluate the effectiveness of rituximab in the treatment of 36 patients with AIHA refractory to several treatments. These patients had received a median of four (one to eight) previous treatments, and 13 patients had undergone splenectomy. Rituximab was administered by intravenous infusion at a dose of 375 mg/m(2) once weekly for four doses in 29 patients, and 7 patients received one to six doses. Overall, 28 (77%) of 36 patients achieved response. Twenty-two patients (61%) reached a complete response (CR), and 6 patients (16%) obtained a partial response. All patients that reached CR (61%) were able to maintain the response during more than 6 months, with a median follow-up of 14 months (1-86 months). Sixteen patients maintained response for more than 1 year. The predictors of maintained response were achievement of CR and negative Coombs test result. Splenectomized patients showed a better response rate than those nonsplenectomized. Rituximab was well tolerated, and only one patient presented a transitory rash during infusion. Rituximab induced clinical responses in multitreated severe refractory both warm and cold AIHA patients with little toxicity, and consequently, this therapy should be considered as an early therapeutic option in this setting. PMID:20526716

Peñalver, Francisco Javier; Alvarez-Larrán, Alberto; Díez-Martin, Jose Luis; Gallur, Laura; Jarque, Isidro; Caballero, Dolores; Díaz-Mediavilla, Joaquín; Bustelos, Rosalía; Fernández-Aceñero, María Jesús; Cabrera, José Rafael

2010-06-05

139

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.

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

2013-01-01

140

Diverse point mutations in the human glucose-6-phosphate dehydrogenase gene cause enzyme deficiency and mild or severe hemolytic anemia.  

PubMed Central

Glucose-6-phosphate dehydrogenase (G6PD; EC 1.1.1.49) deficiency is a common genetic abnormality affecting an estimated 400 million people worldwide. Clinical and biochemical analyses have identified many variants exhibiting a range of phenotypes, which have been well characterized from the hematological point of view. However, until now, their precise molecular basis has remained unknown. We have cloned and sequenced seven mutant G6PD alleles. In the nondeficient polymorphic African variant G6PD A we have found a single point mutation. The other six mutants investigated were all associated with enzyme deficiency. In one of the commonest, G6PD Mediterranean, which is associated with favism among other clinical manifestations, a single amino acid replacement was found (serine----phenylalanine): it must be responsible for the decreased stability and the reduced catalytic efficiency of this enzyme. Single point mutations were also found in G6PD Metaponto (Southern Italy) and in G6PD Ilesha (Nigeria), which are asymptomatic, and in G6PD Chatham, which was observed in an Indian boy with neonatal jaundice. In G6PD "Matera," which is now known to be the same as G6PD A-, two separate point mutations were found, one of which is the same as in G6PD A. In G6PD Santiago, a de novo mutation (glycine----arginine) is associated with severe chronic hemolytic anemia. The mutations observed show a striking predominance of C----T transitions, with CG doublets involved in four of seven cases. Thus, diverse point mutations may account largely for the phenotypic heterogeneity of G6PD deficiency. Images

Vulliamy, T J; D'Urso, M; Battistuzzi, G; Estrada, M; Foulkes, N S; Martini, G; Calabro, V; Poggi, V; Giordano, R; Town, M

1988-01-01

141

Both Hemolytic Anemia and Malaria Parasite-Specific Factors Increase Susceptibility to Nontyphoidal Salmonella enterica Serovar Typhimurium Infection in Mice?  

PubMed Central

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.

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

2010-01-01

142

Conditional deletion of the Bcl-x gene from erythroid cells results in hemolytic anemia and profound splenomegaly.  

PubMed

Bcl-x is a member of the Bcl2 family and has been suggested to be important for the survival and maturation of various cell types including the erythroid lineage. To define the consequences of Bcl-x loss in erythroid cells and other adult tissues, we have generated mice conditionally deficient in the Bcl-x gene using the Cre-loxP recombination system. The temporal and spatial excision of the floxed Bcl-x locus was achieved by expressing the Cre recombinase gene under control of the MMTV-LTR. By the age of five weeks, Bcl-x conditional mutant mice exhibited hyperproliferation of megakaryocytes and a decline in the number of circulating platelets. Three-month-old animals suffered from severe hemolytic anemia, hyperplasia of immature erythroid cells and profound enlargement of the spleen. We demonstrate that Bcl-x is only required for the survival of erythroid cells at the end of maturation, which includes enucleated reticulocytes in circulation. The extensive proliferation of immature erythroid cells in the spleen and bone marrow might be the result of a fast turnover of late red blood cell precursors and accelerated erythropoiesis in response to tissue hypoxia. The increase in cell death of late erythroid cells is independent from the proapoptotic factor Bax, as demonstrated in conditional double mutant mice for Bcl-x and Bax. Mice conditionally deficient in Bcl-x permitted us for the first time to study the effects of Bcl-x deficiency on cell proliferation, maturation and survival under physiological conditions in an adult animal. PMID:11044408

Wagner, K U; Claudio, E; Rucker, E B; Riedlinger, G; Broussard, C; Schwartzberg, P L; Siebenlist, U; Hennighausen, L

2000-11-01

143

Assessment of the red cell proteome of young patients with unexplained hemolytic anemia by two-dimensional differential in-gel electrophoresis (DIGE).  

PubMed

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

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

2012-04-03

144

Livedo reticularis associated with autoimmune hemolytic anemia: prolonged remission induced by peripheral blood stem cell transplantation relapse after 10 years and restoration of hemoglobin levels by rituximab.  

PubMed

Autoimmune hemolytic anemia (AIHA) is a disease where patients produce antibodies against erythrocytes directed towards membrane glycoproteins adsorbed onto the erythrocyte surface. Drugs and other associations have been implicated. It is described and discussed a case of livedo reticularis associated with AIHA treated with peripheral blood stem cell transplantation (PBSCT) that went into full remission for 10 years. After that period the patient relapsed and was treated with antibody anti-CD20, rituximab, and is now in full remission. The role of PBSCT and rituximab in the treatment of AIHA will be discussed. PMID:22286652

Ferreira, Eurípedes; Feitosa, Andrezza; Hamerschlak, Nelson; Scheinberg, Morton Aaron

145

Coinfection of hepatitis A virus genotype IA and IIIA complicated with autoimmune hemolytic anemia, prolonged cholestasis, and false-positive immunoglobulin M anti-hepatitis E virus: a case report  

PubMed Central

A 37-year-old male presented with fever and jaundice was diagnosed as hepatitis A complicated with progressive cholestasis and severe autoimmune hemolytic anemia. He was treated with high-dose prednisolone (1.5 mg/kg), and eventually recovered. His initial serum contained genotype IA hepatitis A virus (HAV), which was subsequently replaced by genotype IIIA HAV. Moreover, at the time of development of hemolytic anemia, he became positive for immunoglobulin M (IgM) anti-hepatitis E virus (HEV). We detected HAV antigens in the liver biopsy specimen, while we detected neither HEV antigen in the liver nor HEV RNA in his serum. This is the first report of hepatitis A coinfected with two different genotypes manifesting with autoimmune hemolytic anemia, prolonged cholestasis, and false-positive IgM anti-HEV.

Kim, Hee-Sup; Jang, Je-Hyuck; Myung, Hyung-Joon; Kim, Jin-Wook; Bang, Soo-Mee; Song, Sang Hoon; Kim, Haeryoung; Yun, Hae Sun

2011-01-01

146

Inborn Anemias in Mice: (Annual Report, 1981-1982).  

National Technical Information Service (NTIS)

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 hemolyti...

S. E. Bernstein

1982-01-01

147

A case of acute hemolytic transfusion reaction due to anti-Dia antibody -A case report-  

PubMed Central

Many medical institutions in Korea have recently been performing an antibody screening test as one of the essential elements of a pre-transfusion test. The Dia antigen is well known as one of the antigens with low incidence among Caucasians; however, it has been discovered with a relatively higher incidence among Mongoloid populations. The frequency of the Dia antigen among the Korean population is estimated to be 6.4-14.5%. But in Korea, a screening panel of cells from abroad without Dia positive cells has been commonly used when a patient has an unexpected antibody screening test. Here we report a case of acute hemolytic transfusion reaction due to Anti-Dia antibody. To prevent other transfusion reaction by anti-Dia antibody, addition of Dia positive cells as unexpected antibody screening test is recommended.

Lee, Seung Hwan; No, Min Young

2012-01-01

148

Mutation of a highly conserved residue of betaI spectrin associated with fatal and near-fatal neonatal hemolytic anemia.  

PubMed Central

We studied an infant with severe nonimmune hemolytic anemia and hydrops fetalis at birth. His neonatal course was marked by ongoing hemolysis of undetermined etiology requiring repeated erythrocyte transfusions. He has remained transfusion-dependent for more than 2 yr. A previous sibling born with hemolytic anemia and hydrops fetalis died on the second day of life. Peripheral blood smears from the parents revealed rare elliptocytes. Examination of their erythrocyte membranes revealed abnormal mechanical stability as well as structural and functional abnormalities in spectrin. Genetic studies revealed that the proband and his deceased sister were homozygous for a mutation of betaIsigma1 spectrin, L2025R, in a region of spectrin that is critical for normal function. The importance of leucine in this position of the proposed triple helical model of spectrin repeats is highlighted by its evolutionary conservation in all beta spectrins from Drosophila to humans. Molecular modeling demonstrated the disruption of hydrophobic interactions in the interior of the triple helix critical for spectrin function caused by the replacement of the hydrophobic, uncharged leucine by a hydrophilic, positively charged arginine. This mutation must also be expressed in the betaIsigma2 spectrin found in muscle, yet pathologic and immunohistochemical examination of skeletal muscle from the deceased sibling was unremarkable.

Gallagher, P G; Petruzzi, M J; Weed, S A; Zhang, Z; Marchesi, S L; Mohandas, N; Morrow, J S; Forget, B G

1997-01-01

149

dRTA and hemolytic anemia: first detailed description of SLC4A1 A858D mutation in homozygous state.  

PubMed

Mutations in the anion exchanger 1 (AE1) gene encoding the erythroid and kidney anion (chloride-bicarbonate) exchanger 1 may result in familial distal renal tubular acidosis (dRTA) in association with membrane defect hemolytic anemia. Seven children presenting with hyperchloremic normal anion gap metabolic acidosis, failure to thrive, and compensated hemolytic anemia were studied. Analysis of red cell AE1/Band 3 surface expression by Eosin 5'-maleimide (E5M) was performed in patients and their family members using flow cytometry. Genetic studies showed that all patients carried a common SLC4A1 mutation, c.2573C>A; p.Ala858Asp in exon 19, found as homozygous (A858D/A858D) mutation in the patients and heterozygous (A858D/N) in the parents. Analysis by flowcytometry revealed a single uniform fluorescence peak, with the mean channel fluorescence (MCF) markedly reduced in cases with homozygous mutation, along with a left shift of fluorescence signal but was only mildly reduced in the heterozygous state. Red cell morphology showed striking acanthocytosis in the homozygous state [patients] and only a mild acanthocytosis in heterozygous state [parents]. In conclusion, this is the first description of a series of homozygous cases with the A858D mutation. The E5M flowcytometry test is specific for reduction in the Band 3 membrane protein and was useful in conjunction with a careful morphological examination of peripheral blood smears in our patient cohort. PMID:22126643

Fawaz, Naglaa A; Beshlawi, Ismail O; Al Zadjali, Shoaib; Al Ghaithi, Hamed K; Elnaggari, Mohamed A; Elnour, Ibtisam; Wali, Yasser A; Al-Said, Bushra B; Rehman, Jalil U; Pathare, Anil V; Knox-Macaulay, Huxley; Alkindi, Salam S

2012-01-04

150

Nomimmune hydrops fetalis due to Diamond-Blackfan anemia.  

PubMed

We describe case report of a baby with Diamond-Blackfan anemia, who presented as non-immune hydrops fetalis. The diagnosis was confirmed by measurement of red cell adenosine deaminase activity which is increased in Diamond-Blackfan anemia. At 2 years of age he is dependent on small dose of alternate day steroid to maintain his hemoglobin. PMID:15004307

Saladi, S M; Chattopadhyay, T; Adiotomre, P N

2004-02-01

151

Immune hemolytic anemia  

MedlinePLUS

... Transfusion of blood from a donor with a blood type that does not match When antibodies form against ... disease Past blood transfusions Pregnancy (if the baby's blood type is different from the mother's) Risk factors are ...

152

Postpartum aplastic anemia presenting as pancytopenia due to malarial infection.  

PubMed

Pancytopenia is a condition with decreased numbers of all cell lines. Aplastic anemia is a common cause although malarial infection causing lysis of RBCs may also partly mimic this condition. The infection may also damage the patient's bone marrow resulting in pancytopenia as well. We present the case of a post-partum female patient who reported with fever, body aches and shortness of breath one month after the delivery of her baby. All blood cell counts were decreased and peripheral blood smear showed malarial parasites. Anti-malarial treatment was initiated following which the fever subsided but, despite regular transfusions, the blood counts remained low. Bone marrow biopsy report revealed P. falciparum pigments along with hypocellularity characteristic of severe aplastic anemia. Consequently, bone marrow transplantation was advised as a therapeutic measure. This case report highlights the increased susceptibility of pregnant women to malaria in endemic areas and subsequent aplastic anemia. PMID:24169391

Shah, Muhammad Usman; Sundhu, Murtaza Ali; Hussain, Muhammad Zahid

2013-11-01

153

Hemolytic anemia and distal renal tubular acidosis in two Indian patients homozygous for SLC4A1/AE1 mutation A858D  

PubMed Central

Familial distal renal tubular acidosis (dRTA) can be caused by mutations in the Cl?/HCO3? exchanger of the renal Type A intercalated cell, kidney AE1/SLC4A1. dRTA-associated AE1 mutations have been reported in families from North America, Europe, Thailand, Malaysia, Papua-New Guinea, Taiwan, and the Philipines, but not India. The dRTA mutation AE1 A858D has been detected only in the context of compound heterozygosity. We report here two unrelated Indian patients with combined hemolytic anemia and dRTA who share homozygous A858D mutations of the AE1/SLC4A1 gene. The mutation creates a novel restriction site that is validated for diagnostic screening.

Shmukler, Boris E.; Kedar, Prabhakar S.; Warang, Prashant; Desai, Mukesh; Madkaikar, Manisha; Ghosh, Kanjaksha; Colah, Roshan B.; Alper, Seth L.

2012-01-01

154

False-positive human immunodeficiency virus antibody test and autoimmune hemolytic anemia in a patient with angioimmunoblastic T-cell lymphoma.  

PubMed

A 44-year-old woman was admitted with generalized lymphadenopathy, which was diagnosed as angioimmunoblastic T-cell lymphoma (AITL). The patient showed autoimmune hemolytic anemia (AIHA), polyclonal hypergammaglobulinemia and a high antinuclear antibody titer. Moreover, a human immunodeficiency virus (HIV)-1/2 screening test using the particle agglutination method was reactive. After chemotherapy for AITL, the AIHA was eliminated, and the false-positive HIV results were no longer detected. Autoimmunity associated with AITL is the likely cause of the cross-reaction with HIV and the AIHA. It is important to recognize that the cross-reaction with HIV can be a potential complication in AITL as well as AIHA. PMID:22001471

Shida, Seiji; Takahashi, Naoto; Fujishima, Naohito; Kameoka, Yoshihiro; Nara, Miho; Fujishima, Masumi; Saitoh, Hirobumi; Tagawa, Hiroyuki; Hirokawa, Makoto; Ichinohasama, Ryo; Sawada, Kenichi

2011-10-15

155

Inborn Anemias in Mice. Progress Report, 1 May 1976--31 July 1977.  

National Technical Information Service (NTIS)

Hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, and the a...

E. S. Russell S. E. Bernstein

1977-01-01

156

Inborn Anemias in Mice. Progress Report, 1 May 1977--31 July 1978.  

National Technical Information Service (NTIS)

Hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, and the a...

S. E. Bernstein E. S. Russell

1978-01-01

157

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

Microsoft Academic Search

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

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

2005-01-01

158

Studies on Erythrocyte Destruction Due to Strenuous Muscular Exercise Which Causes 'Sport Anemia,' and Analysis of Causes of 'March Hematuria.'.  

National Technical Information Service (NTIS)

It is well known that a temporary anemia occurs frequently in the early period of training to the strenuous physical exercise. This anemia is named 'sports anemia'. Yamada clarified its cause as due to an increased fragility of the red blood cells. In oth...

K. Hirakawa H. Yoshimura

1968-01-01

159

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

PubMed Central

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

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

2013-01-01

160

Lack of evidence of a beneficial effect of azathioprine in dogs treated with prednisolone for idiopathic immune-mediated hemolytic anemia: a retrospective cohort study  

PubMed Central

Background Azathioprine is used as an immunosuppressant in canine immune-mediated hemolytic anemia (IMHA), but this potentially toxic and carcinogenic drug has not been proven to be beneficial. The aim of this study was to determine the difference in outcome and survival of dogs with idiopathic IMHA treated with a protocol that included azathioprine and prednisolone versus a protocol that included prednisolone alone. Results The study included 222 dogs with a hematocrit lower than 0.30 L/L and either a positive Coombs' test or spherocytosis and no evidence of diseases that could trigger IMHA. The clinical and laboratory data at the time of diagnosis and the response to therapy and survival were compared in dogs treated according to the prednisolone and azathioprine protocol (AP protocol; n = 149) and dogs treated according to the prednisolone protocol (P protocol; n = 73). At study entry, the two groups were comparable, except that thrombocyte counts were significantly lower and clinical signs had been present significantly longer in the AP protocol group. No significant difference in survival was found between the two groups: the 1-year survival was 64% (95% CI 54 - 77%) in the P protocol group and 69% (95% CI 59-80%) in the AP protocol group, respectively. Conclusions Azathioprine would appear not to be beneficial as standard treatment for all cases of IMHA; however, a blinded, randomized clinical trial is needed to establish whether outcome is different with the two treatment protocols.

2011-01-01

161

Development of flow cytometry for detection and quantitation of red cell bound immunoglobulin G in autoimmune hemolytic anemia with negative direct Coombs test.  

PubMed

About 2-10% of patients with warm-antibody autoimmune hemolytic anemia (WAIHA) exhibit a negative direct Coombs test (DAT), requiring more sensitive tests, including detection of RBC-bound immunoglobulins by flow cytometry, for diagnosis. In this study, the optimal conditions for detection and quantitation of RBC-bound IgG by flow cytometry were studied using blood samples from six patients with AIHA and two healthy individuals. Quantitation of RBC-bound IgG was performed using quantum simply cellular (QSC) beads coated with goat anti-mouse IgG antibodies. For detection of RBC bound IgG, a 60-minute incubation of all blood samples with 40 microl of 1:10 dilution of FITC-conjugated mouse anti-human IgG gave mean fluorescent intensity (MFI) values comparable to experiments using larger amounts or higher concentrations of the anti-human IgG. The acquired antibody binding capacity (ABC) values (or IgG molecules) for each QSC bead level, at 40 microl of 1:5 and 1:10 dilution of anti-human IgG for 60 minutes were close to the manufacturer-assigned ABC values. The IgG molecules per RBC in all six patients with positive DAT of 4+, 3+, 2+, 1+, trace and negative DAT were 31,725, 3,823, 1,753, 524, 260 and 88 respectively and in two healthy individuals with negative DAT they were 104 and 78. PMID:22299318

Thedsawad, Anchalee; Taka, Orathai; Wanachiwanawin, Wanchai

2011-12-01

162

[Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjögren's syndrome].  

PubMed

A 73-year-old woman with Sjögren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×10(9)/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sjögren's syndrome. PMID:21821986

Shimoji, Sonoko; Takiuchi, Yohko; Maruoka, Hayato; Inoue, Daichi; Kimura, Takaharu; Mori, Minako; Nagai, Yuya; Togami, Katuhiro; Tabata, Sumie; Kurata, Masayuki; Matsushita, Akiko; Nagai, Kenichi; Takahashi, Takayuki

2011-07-01

163

[Hemolytic-uremic syndrome in a young woman following the use of ovulation inhibitors].  

PubMed

After prolonged intake of oral contraceptives a 28-year-old woman developed a hemolytic uremic syndrome with microangiopathic hemolytic anemia, renal failure, malignant hypertension and blindness due to papillary congestion. Renal biopsy revealed widespread arteriolar alterations with massive intimal thickening, narrowing of the lumina and fibrinoid necrosis. During treatment with anticoagulation, antihypertensives and hemodialysis the hemolysis disappeared and vision improved, but the renal failure persisted. The possible relation between oral contraceptives and hemolytic uremic syndrome is mentioned and the literature is reviewed. PMID:1215910

Blumberg, A; Studer, U; Briner, J

1975-10-11

164

[Outbreak of subclinical mastitis due to beta hemolytic group L streptococci (S. dysgalactiae ssp. equisimilis) in an Austrian dairy herd].  

PubMed

This study is reporting an outbreak of subclinical mastitis due to beta-hemolytic group L streptococci in an Austrian dairy herd with a history of high somatic cell count. At the first survey 16 of 33 lactating cows (28 quarters of 132) were cultured positive for beta-hemolytic, CAMP and esculin negative cocci that grew on Columbia blood agar with small grey catalase negative colonies. With the commercial API 20 Strep system (bioMerieux, F) isolates were classified as members of streptococci group L. All tested strains (eight of 28) produced acid from ribose, lactose, trehalose, amidon and glycogen; they hydrolysed hippurate and showed beta-glucuronidase, beta-galactosidase, alkaline phosphatase, leucinaminopeptidase and arginindehydrolase activity. Isolates were sensitive to bacitracin but resistant to tetracycline. Using phenotypic characterisation as well as sequence analysis of the 16S-23S intergenic spacer region of a representative strain, recovered isolates were identified as Streptococcus (S.) dysgalactiae ssp. equisimilis. Mastitis was characterized by normal milk secretions and absence of clinical abnormalities but high elevations of somatic cell count. Based on the characteristics of the strains and on the observations during the first herd survey, contagious transmission during milking as a result of poor milking hygiene was assumed. The mastitis was controlled through implementation of a strict hygiene protocol including use of single-use udder towels, post milking teat desinfection and cluster disinfection between milking cows in combination with antibiotic treatment of infected udders. PMID:22059292

Baumgartner, Martina; Giffinger, Friederike; Hoppe, Jan Christoph; Spergser, Joachim

165

Unrelated umbilical cord blood transplantation in infancy for mucopolysaccharidosis type IIB (Hunter syndrome) complicated by autoimmune hemolytic anemia  

Microsoft Academic Search

This report describes unrelated umbilical cord blood transplantation for a 10-month-old infant boy with mucopolysaccharidosis IIB (Hunter syndrome), an X-linked metabolic storage disorder due to deficiency of iduronate sulfatase. Two years after transplant ?55% normal plasma enzyme activity has been restored and abnormal urinary excretion of glycosaminoglycans has nearly completely resolved. The boy has exhibited normal growth and development after

CA Mullen; JN Thompson; LA Richard; KW Chan

2000-01-01

166

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.

2011-01-01

167

Fatal hemolytic transfusion reaction due to anti-Ku in a Knull patient.  

PubMed

A fatal transfusion reaction due to anti-Ku in a Knull (Ko) patient is reported. The patient was transfused with 34 units of incompatible RBCs during 44 days of hospitalization. Apart from the first transfusion, all subsequent transfusions failed to raise the patient's Hb. No serum antibody was identified until he was transferred to another hospital for dialysis. A compatibility test demonstrated a weak antibody and autocontrol reacting at room temperature by a manual polybrene method. The antibody was considered to be a "cold agglutinin." A blood sample was sent to a reference laboratory where the patient was found to be Knull and the antibody was identified as anti-Ku. PMID:15373542

Lin, M; Wang, C L; Chen, F S; Ho, L H

2003-01-01

168

Case Report: Severe Hemolytic Disease of the Newborn Due to anti-Dib Treated with Phototherapy and Intravenous Immunoglobulin  

Microsoft Academic Search

The Di b antigen usually occurs with high incidence, except in certain Asian and South American Indian populations. In general, hemolysis caused by anti-Dib is not severe and its clinical course is benign. We report a Korean neonate with severe hemolytic disease of the newborn caused by anti-Dib. The phenotype and genotype of the Diego blood group system of the

Eun-Jee Oh; Dong Wook Jekarl; Hyun-Sik Jang; Hae-Il Park; Yeon-Joon Park; Hyun Ah Choi; Chung-Sik Chun; Yonggoo Kim; Hyung Hoi Kim

169

Prevalence of Anemia in Children with Congestive Heart Failure due to Dilated Cardiomyopathy  

PubMed Central

Introduction. Anemia is prevalent in adult heart failure patients and appears to be an independent risk factor for morbidity and mortality. The purpose of this work is to determine the prevalence of anemia in children with heart failure from dilated cardiomyopathy (DCM) and to evaluate its influence on morbidity and mortality. Methods. A homogenous group of 58 children with congestive heart failure from DCM was evaluated for heart failure symptoms, appearance of anemia, hospitalization, age of first clinical appearance, necessity of transfusion, and death during medical attendance. Anemic and nonanemic patients were analyzed for differences in age distribution, morbidity, and mortality. Results. Anemia was present in 64% of DCM patients. Hospitalization secondary to heart failure was significantly elevated in heart failure patients with anemia (mean 35.1 ± 40.5 versus 9.97 ± 9.65 days per year, P < 0.05). However, mortality was not elevated. Significant relations of age and prevalence of anemia or age and severity of anemia did not appear. Conclusion. Anemia is prevalent in pediatric patients with congestive heart failure from DCM and appears in all age classes. Hospitalization as a surrogate of morbidity is elevated in heart failure patients developing anemia, but mortality risk did not increase.

Mueller, Goetz Christoph; Schlueter, Emmy Lou; Arndt, Florian; Dodge-Khatami, Ali; Weil, Jochen; Mir, Thomas S.

2012-01-01

170

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.

Wallerstein, Ralph O.

1987-01-01

171

A Potential Strategy for Preventing Complications of Hemolytic ...  

Center for Biologics Evaluation and Research (CBER)

Text Version... The effects of cell-free, unbound Hb observed in hemolytic anemia, malaria, and SCD include high blood pressure in the arteries of the lung ... More results from www.fda.gov/downloads/biologicsbloodvaccines/scienceresearch

172

Sickle Cell Anemia: A Review  

Microsoft Academic Search

Sickle cell anemia is caused by a single mutation on chromosome 11 resulting in the production of abnormal hemoglobin. When deoxygenated, sickle hemoglobin forms polymers that distort the red blood cell and prevent its passage through small vessels. Both the hemoglobin and erythrocyte are damaged by repeated episodes of sickling, resulting in hemolytic anemia and vascular occlusion. The severity of

Holly E. Arnold

173

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

174

Posttransplant Autoimmune Hemolytic Anemia and Other Autoimmune Cytopenias are Increased in Very Young Infants Undergoing Unrelated Donor Umbilical Cord Blood Transplantation  

PubMed Central

Autoimmune cytopenias are a recognized complication of hematopoietic stem cell transplant (HSCT), and are considered to be a feature of chronic graft-versus-host disease (cGVHD). We report on a cohort of very young infants (?3 months of age) receiving HSCT from unrelated donor umbilical cord blood for genetic disorders who developed posttransplant autoimmune cytopenias at an increased rate compared to older aged controls. These infants received a conditioning regimen consisting of busulfan, cyclophosphamide, and antithymocyte globulin (ATG). All infants received HLA mismatched unrelated umbilical cord blood as graft source. GVHD prophylaxis was either cyclosporine + methylprednisolone (n = 16) or cyclosporine + mycophenolate mofetil (n =3). Engraftment, acute GVHD (aGVHD) and cGVHD, survival, treatment-related mortality (TRM), and deaths were evaluated. Ten patients developed cGVHD manifesting as autoimmune cytopenias at a median 247 days posttransplant with a cumulative incidence of 44% (95% confidence interval [CI] 21%–68%) and 56% (95% CI 32%–80%) at 1 and 2 years, respectively. In 6 of 10 patients developing autoimmune cytopenias, cGVHD presented as autoimmune cytopenia de novo. The cytopenias observed included anemia (n =4), thrombocytopenia (n =1), anemia with thrombocytopenia (n =3), and pancytopenia (n =2). No graft factors were identified as being significant to development of cGVHD. All patients responded to treatment with methylprednisolone, azithioprine ± rituximab. One patient required splenectomy. We hypothesize that posttransplant immunosuppression interferes with normal immune ontogeny creating immune dysregulation and graft directed cell destruction. Alternative strategies to prevent GVHD should be considered for this unique patient population.

Page, Kristin M.; Mendizabal, Adam M.; Prasad, Vinod K.; Martin, Paul L.; Parikh, Suhag; Wood, Susan; Sempowski, Gregory D.; Szabolcs, Paul; Kurtzberg, Joanne

2013-01-01

175

Blueberry muffin rash, hyperbilirubinemia, and hypoglycemia: A case of hemolytic disease of the fetus and newborn due to anti-Kpa  

Microsoft Academic Search

Hemolytic disease of the fetus and newborn occurs when maternal IgG antibodies cross the placenta and cause hemolysis of fetal red blood cells. Kpa is a low frequency red blood cell antigen that has rarely been implicated in hemolytic disease of the fetus and newborn. The few reported cases attributed to anti-Kpa have typically had minimal clinical consequences. We report

J E Brumbaugh; S Morgan; J C Beck; N Zantek; S Kearney; C M Bendel; K D Roberts

2011-01-01

176

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, Emília; Miguel, Natalina; Vizcaíno, Ramón; Barbot, José; Pereira, Fernando

2013-05-01

177

Anemia in Elderly Koreans  

PubMed Central

Recently, the geriatric population in Korea has grown to comprise approximately 10% of the total population, and anemia has become a significant problem among elderly patients. Many elderly patients have anemia due to nutritional deficiency, chronic inflammation, or comorbid diseases; however, in a significant fraction of the patients with anemia, the cause remains obscure. Anemia of any degree is recognized as a significant independent contributor to morbidity and mortality in elderly patients. This article summarizes the patterns of anemia in Korean geriatric patients.

2011-01-01

178

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

179

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

PubMed Central

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

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

2013-01-01

180

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

181

Shared Copy Number Variation in Simultaneous Nephroblastoma and Neuroblastoma due to Fanconi Anemia  

PubMed Central

Concurrent emergence of nephroblastoma (Wilms Tumor; WT) and neuroblastoma (NB) is rare and mostly observed in patients with severe subtypes of Fanconi anemia (FA) with or without VACTER-L association (VL). We investigated the hypothesis that early consequences of genomic instability result in shared regions with copy number variation in different precursor cells that originate distinct embryonal tumors. We observed a newborn girl with FA and VL (aplasia of the thumbs, cloacal atresia (urogenital sinus), tethered cord at L3/L4, muscular ventricular septum defect, and horseshoe-kidney with a single ureter) who simultaneously acquired an epithelial-type WT in the left portion of the kidney and a poorly differentiated adrenal NB in infancy. A novel homozygous germline frameshift mutation in PALB2 (c.1676_c1677delAAinsG) leading to protein truncation (pGln526ArgfsX1) inherited from consanguineous parents formed the genetic basis of FA-N. Spontaneous and induced chromosomal instability was detected in the majority of cells analyzed from peripheral lymphocytes, bone marrow, and cultured fibroblasts. Bone marrow cells also showed complex chromosome rearrangements consistent with the myelodysplastic syndrome at 11 months of age. Array-comparative genomic hybridization analyses of both WT and NB showed shared gains or amplifications within the chromosomal regions 11p15.5 and 17q21.31-q25.3, including genes that are reportedly implicated in tumor development such as IGF2, H19, WT2, BIRC5, and HRAS.

Serra, A.; Eirich, K.; Winkler, A.K.; Mrasek, K.; Gohring, G.; Barbi, G.; Cario, H.; Schlegelberger, B.; Pokora, B.; Liehr, T.; Leriche, C.; Henne-Bruns, D.; Barth, T.F.; Schindler, D.

2012-01-01

182

Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura\\/hemolytic-uremic syndrome (TTP\\/HUS)  

Microsoft Academic Search

Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura\\/hemolytic-uremic syndrome (TTP\\/HUS).BackgroundThrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are now considered to be variants of one single syndrome called thrombotic thrombocytopenic purpura\\/hemolytic-uremic syndrome (TTP\\/HUS). Key features are thrombocytopenia, hemolytic anemia, and subsequently impaired function of different organs, especially the kidneys and the central nervous system (CNS). One possible reason is

CHRISTOPH LICHT; LUDWIG STAPENHORST; THORSTEN SIMON; ULRICH BUDDE; REINHARD SCHNEPPENHEIM; BERND HOPPE

2004-01-01

183

Development of an experimental hemolytic uremic syndrome in rats  

Microsoft Academic Search

Escherichia coli strains producing Shiga toxins (Stxs) colonize the lower gastrointestinal tract and cause watery diarrhea, hemorrhagic colitis,\\u000a and hemolytic-uremic syndrome (HUS). HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure.\\u000a Oliguria associated with acute tubular necrosis and microangiopathic thrombosis has been reported as the most common cause\\u000a of renal failure in Argentinean children. Our study was undertaken

Elsa Zotta; Nestor Lago; Federico Ochoa; Horacio A. Repetto; Cristina Ibarra

2008-01-01

184

[Enzyme deficient non-spherocytic hemolytic anemias].  

PubMed

Enzymopathies are described concerning the enzymes of the oxidative pentose phosphate pathway including the glutathion system, of the majority of glycolytic enzymes as well as of the ATPase, adenylate kinase and pyrimidine-5'-nucleotidase. The distribution and the frequency of the enzymopathies differ strongly in the various regions of the world. Glucose-6-phosphate dehydrogenase and pyruvate kinase show the highest frequency. The detected polymorphism of the pathological enzyme variants is one of the reasons for the fact that no correlation between the decrease of the catalytic activity and the severity of the anaemias has been found. For the identification of risk-groups more precise methods are necessary. Till now the detailed relationships between enzymopathy and non-spherocytic haemolytic anaemias are not clarified. Furthermore the molecular mechanism of the instability of pathological enzyme variants is not yet clear. PMID:676410

Jacobasch, G

1978-05-01

185

[Diagnosis of hemolytic anemia with unstable hemoglobin].  

PubMed

Heinz-body haemolytic anaemia represents a rarely occurring kind of hereditary defect of haemoglobin, G-6-PDH or glutathion reductase. The course of disease observed in two patients with non-spherocytic haemolytic anaemia was very serious as compared with other cases with haemoglobin variants and enzyme defects of G-6-PDH described in literature. The course of disease could not be influenced by splenectomy, substitution therapy, and long-term therapy with desferrioxamin. Exitus occurred at an age 22 or 41 as a consequence of severe haemosiderosis. PMID:6162731

Sakalová, A; Hrubisko, M

1980-01-01

186

Early erythropoietin reduced the need for red blood cell transfusion in childhood hemolytic uremic syndrome—a randomized prospective pilot trial  

Microsoft Academic Search

Childhood hemolytic uremic syndrome (HUS) is most often caused by enterohemorrhagic Escherichia coli (EHEC). Due to severe hemolysis, red blood cell (RBC) transfusions are often necessary, and anemia is aggravated by low erythropoietin\\u000a (EPO) levels caused by acute renal failure. In a single center, prospective study, we randomized ten children with EHEC-positive\\u000a HUS into two therapeutic groups: one receiving EPO

Lars Pape; Thurid Ahlenstiel; Martin Kreuzer; Jens Drube; Kerstin Froede; Doris Franke; Jochen H. H. Ehrich; Marion Haubitz

2009-01-01

187

A New Alkaline pH-Adjusted Medium Enhances Detection of ?-Hemolytic Streptococci by Minimizing Bacterial Interference Due to Streptococcus salivarius  

PubMed Central

A new selective medium (CNA-P) that reduces or eliminates the inhibitory activity of bacteriocin-producing Streptococcus salivarius against ?-hemolytic streptococci has been developed and compared with sheep blood agar (SBA) for the sensitive detection of small numbers of ?-hemolytic streptococci in clinical specimens. CNA-P has as its basis a commercial medium (Difco Columbia CNA agar) supplemented with 5% (vol/vol) sheep blood, and the CNA is further modified by addition of 100 mM PIPES buffer [piperazine-N,N?-bis(2-ethanesulfonic acid)] (pH 7.5) to maintain cultures at an alkaline pH during incubation. CNA-P was shown to inhibit the production and/or release of four different types of S. salivarius bacteriocins or bacteriocin-like inhibitory molecules. The efficacies of CNA-P and SBA for detection of ?-hemolytic streptococci in 1,352 pharyngeal samples from 376 children were compared. The ?-hemolytic streptococcal isolates recovered from the samples included 314 group A (S. pyogenes), 61 group G, 33 group B, and 5 group C streptococci. Of 314 samples that yielded S. pyogenes, 300 were positive on CNA-P (96%) and 264 (86%) were positive on SBA. A significantly greater number of S. pyogenes isolates from these samples were recovered only on CNA-P (50 of 314) compared with the number of isolates recovered only on SBA (14 of 314). In addition, the degree of positivity, a measure of the total numbers of S. pyogenes isolates on the plate, was significantly higher on CNA-P than on SBA (2.40 versus 2.07; P < 0.001). Interestingly, CNA-P was also found to enhance the hemolytic activity of streptolysin O, allowing detection of streptolysin S-deficient S. pyogenes strains which might otherwise go undetected on SBA and other isolation media.

Dierksen, Karen P.; Ragland, Nancy L.; Tagg, John R.

2000-01-01

188

Differentiation and Treatment of Anemia in HIV Disease  

Microsoft Academic Search

Anemia is a frequent complication of HIV disease that contributes to decreased quality of life and increased morbidity and mortality. The three major categories of anemia in HIV disease are anemia due to impaired red blood cell production, anemia due to increased red blood cell destruction, and anemia due to increased red blood cell loss. Although anemia of chronic illness

Kenneth D. Phillips; Maureen Groer

2002-01-01

189

A Case of Alloimmune Thrombocytopenia, Hemorrhagic Anemia-Induced Fetal Hydrops, Maternal Mirror Syndrome, and Human Chorionic Gonadotropin-Induced Thyrotoxicosis  

PubMed Central

Fetal/neonatal alloimmune thrombocytopenia (FNAIT) can be a cause of severe fetal thrombocytopenia, with the common presentation being intracranial hemorrhage in the fetus, usually in the third trimester. A very unusual case of fetal anemia progressed to hydrops. This was further complicated by maternal Mirror syndrome and human chorionic gonadotropin–induced thyrotoxicosis. Without knowledge of etiology, and possibly due to associated cardiac dysfunction, fetal transfusion resulted in fetal demise. Subsequent testing revealed FNAIT as the cause of severe hemorrhagic anemia. In cases with fetal anemia without presence of red blood cell antibodies, FNAIT must be ruled out as a cause prior to performing fetal transfusion. Fetal heart may adapt differently to acute hemorrhagic anemia compared with a more subacute hemolytic anemia.

Jain, Venu; Clarke, Gwen; Russell, Laurie; McBrien, Angela; Hornberger, Lisa; Young, Carmen; Chandra, Sujata

2013-01-01

190

A case of alloimmune thrombocytopenia, hemorrhagic anemia-induced fetal hydrops, maternal mirror syndrome, and human chorionic gonadotropin-induced thyrotoxicosis.  

PubMed

Fetal/neonatal alloimmune thrombocytopenia (FNAIT) can be a cause of severe fetal thrombocytopenia, with the common presentation being intracranial hemorrhage in the fetus, usually in the third trimester. A very unusual case of fetal anemia progressed to hydrops. This was further complicated by maternal Mirror syndrome and human chorionic gonadotropin-induced thyrotoxicosis. Without knowledge of etiology, and possibly due to associated cardiac dysfunction, fetal transfusion resulted in fetal demise. Subsequent testing revealed FNAIT as the cause of severe hemorrhagic anemia. In cases with fetal anemia without presence of red blood cell antibodies, FNAIT must be ruled out as a cause prior to performing fetal transfusion. Fetal heart may adapt differently to acute hemorrhagic anemia compared with a more subacute hemolytic anemia. PMID:23943709

Jain, Venu; Clarke, Gwen; Russell, Laurie; McBrien, Angela; Hornberger, Lisa; Young, Carmen; Chandra, Sujata

2013-01-25

191

[Beta-hemolytic streptococcal bacteremia in adults].  

PubMed

The serogroups A (Streptococcus pyogenes), B (Streptococcus agalactiae) and S. dysgalactiae subsp. equisimilis (group C and G) are generally defined as beta-hemolytic streptococci. Beta-hemolytic streptococci cause a variety of infections ranging from skin and soft-tissue infections to severe invasive infections such as necrotizing fasciitis (NF) and streptococcal toxic shock syndrome (STSS). The case fatality rate due to bacteremias caused by beta-hemolytic streptococci is 15%. The use of clindamycin in combination with benzylpenicillin has been shown to be of benefit. The use of intravenous immunoglobulin is suggested in STSS in combination with antibiotics and surgery. PMID:23961606

Rantala, Sari

2013-01-01

192

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

193

An Elevated Fetal IL-6 Concentration Can Be Observed In Fetuses with Anemia Due To Rh Alloimmunization: Implications for the Understanding of the Fetal Inflammatory Response Syndrome  

PubMed Central

Objective The fetal inflammatory response syndrome (FIRS) has been described in the context of preterm labor and preterm PROM and is often associated with intra-amniotic infection/inflammation. This syndrome is characterized by systemic fetal inflammation and operationally-defined by an elevated fetal plasma interleukin (IL)-6. The objective of this study was to determine if FIRS can be found in fetuses with activation of their immune system, such as the one observed in Rh alloimmune-mediated fetal anemia. Methods Fetal blood sampling was performed in sensitized Rh-D negative women with suspected fetal anemia (n=16). Fetal anemia was diagnosed according to reference range nomograms established for the assessment of fetal hematologic parameters. An elevated fetal plasma IL-6 concentration was defined using a cutoff of >11 pg/mL. Concentrations of IL-6 were determined by immunoassay. Non-parametric statistics were used for analysis. Results 1) The prevalence of an elevated fetal plasma IL-6 was 25% (4/16); 2) there was an inverse relationship between the fetal hematocrit and IL-6 concentration - the lower the hematocrit, the higher the fetal IL-6 (r= ?0.68, p=0.004); 3) fetuses with anemia had a significantly higher plasma IL-6 concentration than those without anemia (3.74 pg/ml, interquartile range (IQR) 1.18–2.63 vs. 1.46 pg/ml, IQR 1.76–14.7; p=0.02); 4) interestingly, all fetuses with an elevated plasma IL-6 concentration had anemia (prevalence 40%, 4/10), while in the group without anemia, none had an elevated fetal plasma IL-6. Conclusions An elevation in fetal plasma IL-6 can be observed in a subset of fetuses with anemia due to Rh alloimmunization. This observation suggests that the hallmark of FIRS can be caused by non-infection-related insults. Further studies are required to determine whether the prognosis of FIRS caused by intra-amniotic infection/inflammation is different from that induced by alloimmunization.

Vaisbuch, Edi; Romero, Roberto; Gomez, Ricardo; Kusanovic, Juan Pedro; Mazaki-Tovi, Shali; Chaiworapongsa, Tinnakorn; Hassan, Sonia S.

2010-01-01

194

Thrombomodulin Mutations in Atypical Hemolytic-Uremic Syndrome  

PubMed Central

BACKGROUND The hemolytic–uremic syndrome consists of the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. The common form of the syndrome is triggered by infection with Shiga toxin–producing bacteria and has a favorable outcome. The less common form of the syndrome, called atypical hemolytic–uremic syndrome, accounts for about 10% of cases, and patients with this form of the syndrome have a poor prognosis. Approximately half of the patients with atypical hemolytic–uremic syndrome have mutations in genes that regulate the complement system. Genetic factors in the remaining cases are unknown. We studied the role of thrombomodulin, an endothelial glycoprotein with anticoagulant, antiinflammatory, and cytoprotective properties, in atypical hemolytic–uremic syndrome. METHODS We sequenced the entire thrombomodulin gene (THBD) in 152 patients with atypical hemolytic–uremic syndrome and in 380 controls. Using purified proteins and cell-expression systems, we investigated whether thrombomodulin regulates the complement system, and we characterized the mechanisms. We evaluated the effects of thrombomodulin missense mutations associated with atypical hemolytic–uremic syndrome on complement activation by expressing thrombomodulin variants in cultured cells. RESULTS Of 152 patients with atypical hemolytic–uremic syndrome, 7 unrelated patients had six different heterozygous missense THBD mutations. In vitro, thrombomodulin binds to C3b and factor H (CFH) and negatively regulates complement by accelerating factor I–mediated inactivation of C3b in the presence of cofactors, CFH or C4b binding protein. By promoting activation of the plasma procarboxypeptidase B, thrombomodulin also accelerates the inactivation of anaphylatoxins C3a and C5a. Cultured cells expressing thrombomodulin variants associated with atypical hemolytic–uremic syndrome had diminished capacity to inactivate C3b and to activate procarboxypeptidase B and were thus less protected from activated complement. CONCLUSIONS Mutations that impair the function of thrombomodulin occur in about 5% of patients with atypical hemolytic–uremic syndrome.

Delvaeye, Mieke; Noris, Marina; De Vriese, Astrid; Esmon, Charles T.; Esmon, Naomi L.; Ferrell, Gary; Del-Favero, Jurgen; Plaisance, Stephane; Claes, Bart; Lambrechts, Diether; Zoja, Carla; Remuzzi, Giuseppe; Conway, Edward M.

2012-01-01

195

Abnormal Phospholipid Metabolism in Spur Cell Anemia: Decreased Fatty Acid Incorporation Into Phosphatidylethanolamine and Increased Incorporation Into Acylcarnitine in Spur Cell Anemia Erythrocytes  

Microsoft Academic Search

Spur cell anemia is a hemolytic anemia seen in severe alco- holic cirrhosis that is characterized by unusual morphology and a decreased ratio of phospholipids to cholesterol in the erythrocyte membrane. We hypothesized that defective phospholipid repair may contribute to the red blood cell (RBC) phospholipid abnormalities of spur cell anemia. There- fore, we compared RBCs from normal control subjects

David W. Allen; Nancy Manning

1994-01-01

196

[A case report with hemolytic uremic syndrome by a oral contraceptive, and its clinical and pathological reviews (author's transl)].  

PubMed

A 37 year old female with acute renal failure following malignant hypertension and microangiopathic hemolytic anemia as a side effect of oral contraceptives is reported as a first from Japan. Although she had enough medical treatment including hemodialysis for 2 years from the onset of symptoms, the complete remission did not occur due to severe narrowing of the small renal arteries. 3 interval biopsies revealed considerable myxoid intimal thickening of small arcuated and interlobular arteries without evident histological regression. No fibrinoid necrosis is present in arterioles. Glomeruli show a wrinkling of GBM with a partial mesangial interposition and a translucent subendothelial deposit. A year after onset, both kidneys contracted slightly. Platelet factors which were probably released during the hemolytic uremic state in the kidney may play an important role in severe myxoid intimal thickening of the small renal arteries. (author's) PMID:7328888

Fukushima, K; Yamagata, Y; Taguchi, T; Matsuo, K; Koga, N; Takebayashi, S

1981-04-01

197

Posttransplant recurrence of atypical hemolytic uremic syndrome.  

PubMed

Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It is usually secondary to infections by strains of Escherichia coli (STEC) that produce Shiga-like toxin. In about 10% of patients, no STEC infections are reported. In these cases of atypical HUS (aHUS), mutations in genes encoding proteins of the complement system have been described. Atypical HUS is characterized by poor prognosis and by high risk of posttransplant recurrence which greatly depends on the specific gene mutation involved in the disease. Plasma therapy, eculizumab treatment and, in some cases, combined liver-kidney transplant have been used to prevent and/or treat posttransplant aHUS recurrences. PMID:22760880

Valoti, Elisabetta; Alberti, Marta; Noris, Marina

198

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 Müller; Christoph Licht; Marianne Wigger; Christine Skerka

2010-01-01

199

A case of splenic torsion with progressive anemia and thrombocytopenia  

PubMed Central

A 4-year-old male, castrated Saint Bernard was evaluated for acute onset of lethargy and collapse. Moderately severe anemia and splenomegaly were noted. Immune mediated hemolytic anemia was initially suspected. Abdominal ultrasound demonstrated an absence of splenic blood flow. Splenic torsion was confirmed on exploratory laparotomy and a splenectomy was performed.

Schnier, Lisa M.

2010-01-01

200

Hemolytic uremic syndrome in renal allografted patients treated with cyclosporin.  

PubMed

The classical triad of hemolytic uremic syndrome (microangiopathic hemolytic anemia, severe thrombopenia, and renal failure) developed de novo in three of our renal transplanted patients under cyclosporin A treatment. The predominant morphologic findings in the grafts consisted of glomerular and arteriolar thrombosis as well as arteriolonecrosis, all features of the syndrome. In one instance, ischemic bowel disease supervened after graft removal and was associated with persistent low grade microangiopathic process. De novo hemolytic uremic syndrome has been reported in patients treated with cyclosporin A following bone marrow or liver transplantation as well as in a few renal graft recipients. This peculiar form of cyclosporin A nephrotoxicity should not be confused with acute rejection of the renal transplant. PMID:3304591

Giroux, L; Smeesters, C; Corman, J; Paquin, F; Allaire, G; St-Louis, G; Daloze, P

1987-06-01

201

Anemia due to coadministration of renin-angiotensin-system inhibitors and PPAR? agonists in uncomplicated diabetic patients.  

PubMed

Therapy with either angiotensin converting enzyme inhibitors and angiotensin receptor blockers (ACEI/ARB) or thiazolidinediones (TZD) is associated with dose-dependent decrements in hematocrit and hemoglobin levels. We aimed to investigate the impact of the coadministration of TZD and ACEI/ARB on hematocrit and hemoglobin values in uncomplicated patients with type 2 diabetes mellitus and normal serum creatinine.Data from patients with type 2 diabetes currently followed, were reviewed and patients treated with ACEI/ARB and/or TZD were identified. For the purpose of this study the following 4 groups of 30 stable non-anemic diabetic patients each matched for age, gender, and BMI were formed. Group ACEI/ARB included patients on ACEI/ARB without TZD, group TZD included patients on TZD and antihypertensive agents other than ACEI/ARB, group ACEI/ARB/TZD consisted of patients on combined therapy with ACEI/ARB and TZD and the control group C included patients never exposed to ACEI/ARB or TZD. Clinical and laboratory data were collected prior to initiation of treatment and after 6 months.Neither hematocrit nor hemoglobin showed any significant change from baseline at the end of the study in group C. In both group ACEI/ARB and group TZD a small, but statistically significant reduction in hematocrit (~ 1% point) and hemoglobin levels (~ 0.3 g/dl) was seen. A greater statistically and clinically important reduction in hematocrit (~ 3% points) and hemoglobin (~ 1 g/dl) levels was observed in group ACEI/ARB/TZD. Furthermore, incident anemia at the end reached 7% in group TZD and 23% in group ACEI/ARB/TZD.Coadministration of RAS inhibitors and PPAR-? agonists should be considered in the differential diagnosis of hematocrit lowering and anemia in uncomplicated type 2 diabetic patients with normal serum creatinine. Further studies are required to clarify the mechanism(s), the cardiovascular consequences and the cost utility of anemia workup in such patients. PMID:22441720

Raptis, A E; Bacharaki, D; Mazioti, M; Marathias, K P; Markakis, K P; Raptis, S A; Dimitriadis, G D; Vlahakos, D V

2012-03-22

202

Hemolytic activity of Serratia marcescens  

Microsoft Academic Search

A cell-bound hemolytic activity was found in several strains of Serratia marcescens. One Serratia cell per ten erythrocytes was sufficient to cause complete lysis of human erythrocytes within 2 h in the liquid assay. The hemolytic activity resided in the membrane fraction and could be inactivated by incubating cells with proteases. The hemolytic activity was greatly enhanced in actively metabolizing

Volkmar Braun; Hannelore Günther; Burkard Neuß; Christiane Tautz

1985-01-01

203

Anemia of Chronic Disease (Anemia of Inflammation)  

Microsoft Academic Search

Mild-to-moderate anemia often develops in the setting of acute or chronic immune activation and is termed anemia of chronic disease (ACD) or anemia of inflammation. Anemia of chronic disease is the second most common type of anemia (after anemia of iron deficiency) and results in increased morbidity and mortality of the underlying disease. Anemia of chronic disease is mediated by

Neeraj Agarwal; Josef T. Prchal

2009-01-01

204

Differential microcyte anemia diagnosis using hierarchical soft computing  

Microsoft Academic Search

Anemia is the most common hematological disorder. It's difficult to discriminate either thalassemia or Iron deficiency anemia, due to the two subtype of microcytic anemia, which has the feature similarly with mean cell volume less than 80 fL (fluid ounces). The CBC is objective for physician to discriminate anemia between iron deficiency anemia with thalassemia. The disorder will be more

Jehn-Shan Yeh

2004-01-01

205

Adult hemolytic uremic syndrome associated with Streptococcus pneumoniae.  

PubMed

Hemolyitic uremic syndrome (HUS), characterized by triad of acute kidney injury, thrombocytopenia, and hemolytic anemia, has considerable morbidity and mortality and is known to be associated with diarrheal illness. It usually occurs after a diarrheal illness due to Shiga-toxin-producing Escherichia coli. Streptococcus pneumoniae is a rare but well recognized trigger for nondiarrhea associated HUS in children, but has not been reported in adults. We report a case of an adult presenting with pneumococcal pneumonia complicated by HUS and required renal replacement therapy.Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis characterized by mesangial deposits of IgM. IgM nephropathy presenting with proteinuria, especially nephrotic syndrome, frequently is steroid-dependent or steroidresistant and associated with reaching endstage renal disease after a 15-year follow-up. Because no long-term effective treatment is known for patients with IgM nephropathy, there is a clear need for therapeutic alternatives. We describe a patient with IgM nephropathy represented by recurrence of nephrotic syndrome who achieved longterm remission with interferon-? sustained treatment. PMID:23380391

Allen, Jennifer C; McCulloch, Thomas; Kolhe, Nitin V

2013-02-01

206

Glucose-6-phosphate dehydrogenase deficiency  

MedlinePLUS

G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency ... Churchill Livingston; 2008:chap 45. Golan DER. Hemolytic anemias: red cell membrane and metabolic defects. In: Goldman ...

207

Aplastic Anemia  

MedlinePLUS

... for more serious cases, and, in severe cases, bone marrow transplantation. Severe aplastic anemia, in which your blood cell ... In addition, not everyone is a candidate for transplantation or can find a ... can't undergo a bone marrow transplant or for those whose aplastic anemia may ...

208

Risk of hemolytic uremic syndrome after sporadic Escherichia coli O157:H7 infection: Results of a Canadian collaborative study  

Microsoft Academic Search

Objectives: The objectives of this study were to better estimate the age-specific risks of hemolytic uremic syndrome (HUS) and hemolytic anemia after Escherichia coli O157:H7 infection among a representative cohort of both referred and nonreferred children with documented illness from the province of Alberta and to compare this with the rates in children evaluated at referral centers in the rest

Peter C. Rowe; Elaine Orrbine; Hermy Lior; George A. Wells; Elizabeth Yetisir; Melissa Clulow; Peter N. McLaine

1998-01-01

209

[Risk of development of clinical and pathogenetic features of anemia on the background of basic therapy of inflammatory bowel disease].  

PubMed

Anemia in IBD is the result of a combination of iron deficiency and anemia of chronic disease. Therapy of IBD is relief of inflammation, but the drugs usage may cause the development hemolytic anemia and myelodysplastic syndrome. We studied the effect of basic therapy on the incidence of anemia and assess the impact of modern biological therapies on the main markers of AHZ. A total of 153 patients with ulcerative colitis (UC) and 53 patients with Crohn's disease (CD), which at the time of the study received basic anti-inflammatory therapy for at least 1 year. All patients underwent blood tests, iron metabolism parameters were determined by the level of erythropoietin and G-gepsidina C reactive protein. Modern biological therapy increases the effectiveness of the treatment of anemia in patients with IBD. The use of Remicade gives a quick positive response, which is due to the decrease of gepsidin negative influence on iron metabolism and unlocking the synthesis of erythropoietin. The use of MSCs does not inhibit the synthesis of erythropoietin, and is likely to stimulate erythropoiesis at the erythroblast precursors. PMID:22629693

Noskova, K K; Lishchinskaia, A A; Parfenov, A I; Kniazev, O V; Varvanina, G G; Drozdov, V N

2011-01-01

210

THE ELIMINATION OF IRON AND ITS DISTRIBUTION IN THE LIVER AND SPLEEN IN EXPERIMENTAL ANEMIA  

PubMed Central

Blood destruction due to a single injury, as by sodium oleate, or acting through a short period of time, as by toluylenediamine or hemolytic immune serum, is not characterized, in the absence of hemoglobinuria, by an increased elimination of iron in the urine. This holds, not only for the evanescent injury caused by sodium oleate, but also for the severe type caused by hemolytic immune serum, in which a progressive destruction of the blood may persist for 2 weeks or more with constant evidence of the disintegration of erythrocytes as shown by bile pigment in the urine. This finding is in accord with previous investigations of anemia in both man and animals. Likewise, no striking increase is evident, under such circumstances, in the percentage of iron excreted in the feces. The total amount of iron in the feces has been notably increased in two experiments with hemolytic serum, but as the percentage was not appreciably altered, the difference depends presumably on variations in the bulk of feces rather than upon increased elimination. This evidence of the power of the body to conserve the iron rephagocytosis is negligible, is to be fragmented one by one, while still circulating, to a fine, hemoglobin-containing dust. The cell fragments are rapidly removed from the blood, but their ultimate fate remains to be determined. The facts indicate that they are removed from the blood by the spleen, and under exceptional conditions, by the bone marrow.

Dubin, Harry; Pearce, Richard M.

1917-01-01

211

A neonate with Coombs-negative hemolytic jaundice with spherocytes but normal erythrocyte indices: a rare case of autosomal-recessive hereditary spherocytosis due to alpha-spectrin deficiency.  

PubMed

The diagnosis of hereditary spherocytosis (HS) in a newborn infant is generally made on the basis of a positive family history, spherocytes on blood film and Coombs-negative hemolytic jaundice of variable severity with an elevated mean corpuscular hemoglobin concentration (MCHC) and a low mean corpuscular volume (MCV). In general, sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE) quantification of erythrocyte membrane proteins is not needed to make the clinical diagnosis of HS. However, we observed that a neonate with no family history of HS, but with abundant spherocytosis on repeated blood films, Coombs-negative hemolytic jaundice and normal MCHC and MCV measurements, where SDS-PAGE revealed alpha-spectrin deficiency, a rare autosomal-recessive variety of HS that generally has a severe clinical phenotype. PMID:23624969

Yaish, H M; Christensen, R D; Agarwal, A

2013-05-01

212

Pathogenesis of Hemolytic Anemia in Homozygous Hemoglobin C Disease*  

PubMed Central

Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged ?6-lysyl groups and negatively charged groups on adjacent molecules. Red cells from patients with homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which suggest that the cells are more rigid than normal erythrocytes. They pass through membrane filters less readily than normal red cells do, and their viscosity is higher than that of normal cells. Differences from normal cells are exaggerated if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension in hypertonic salt solution. Increased rigidity of CC cells, by accelerating their fragmentation, may be responsible for formation of microspherocytes. These small dense cells are exceptionally rigid, and probably are even more susceptible to fragmentation and sequestration. Rigidity of CC cells can be attributed to a “precrystalline” state of intracellular hemoglobin, in which crystallization does not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates. Images

Charache, Samuel; Conley, C. Lockard; Waugh, David F.; Ugoretz, Richard J.; Spurrell, J. Richard

1967-01-01

213

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

214

In utero erythrocyte transfusion for fetal xerocytosis associated with severe anemia and non-immune hydrops fetalis.  

PubMed

Hereditary xerocytosis is a rare hemolytic anemia occurring secondary to a defect in cell membrane potassium flux. We report a case of severe fetal anemia and non-immune hydrops secondary to hereditary xerocytosis that was managed successfully with in utero erythrocyte and albumin transfusion. PMID:11483935

Ogburn, P L; Ramin, K D; Danilenko-Dixon, D; Fairbanks, V F; Ramsey, P S

2001-07-01

215

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

PubMed Central

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.

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

2013-01-01

216

About Anemia  

MedlinePLUS

... get anemia if: not enough RBCs are made too many RBCs are destroyed too many RBCs are lost (from bleeding) Not enough being ... marrow from being able to make enough RBCs. Too many being destroyed: If the life of a red ...

217

Assesment, Treatment and Prevention of Atypical Hemolytic Uremic Syndrome  

PubMed Central

Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

Nickavar, Azar; Sotoudeh, Kambiz

2013-01-01

218

Assesment, treatment and prevention of atypical hemolytic uremic syndrome.  

PubMed

Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS. PMID:23412906

Nickavar, Azar; Sotoudeh, Kambiz

2013-01-01

219

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

PubMed

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

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

2011-08-04

220

Management of cancer anemia.  

PubMed

Anemia complicates the course of disease in about 50% of patients with cancer, and negatively affects their quality of life. A correct approach to therapy should consider all the possible causes and patients need to be treated accordingly. The observation that erythropoietin production is often blunted offers new treatment possibilities. Fifty to 70% of anemic patients respond to rHuEpo, given in a three times or once-a-week schedule. The novel hyperglycosylated protein darbepoetin permits longer intervals between administrations, thanks to its longer half-life, and a once per cycle or once-a-month schedule is a reasonable target. Correction of anemia improves the quality of life, and it has been hypothesized that improvement of cognitive function may derive from a direct effect of Epo on CNS cells. Although anemia is an adverse prognostic factor in cancer, results of recent clinical trials have raised the question whether rHuEpo may favor neoplastic cell proliferation. Results are conflicting at the moment, and further studies are required before arriving at a conclusion. Data available so far do not indicate any negative effect of darbepoetin on the outcome of cancer disease, nor has the production of anti-darbepoetin antibodies or PRCA been reported, a complication observed in less than 200 patients with anemia due to renal insufficiency and treated with rHuEpo alpha. PMID:15688625

Grossi, A

2004-11-01

221

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.

2013-01-01

222

Hemolytic disease of the fetus and newborn caused by anti-E  

PubMed Central

Objective: Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother's red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs. Case summary: We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion. Conclusion: There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.

Usman, Adiyyatu Sa'idu; Mustaffa, Rapiaah; Ramli, Noraida; Diggi, Sirajo A.

2013-01-01

223

Severe Aplastic Anemia (SAA)  

MedlinePLUS

... Email this page Print this page Severe aplastic anemia (SAA) Tweet Aplastic anemia is a disease in which the bone marrow ... fight infection), and platelets (to control bleeding). Aplastic anemia is rare. In the United States, about 600- ...

224

THE RELATION OF THE SPLEEN TO BLOOD DESTRUCTION AND REGENERATION AND TO HEMOLYTIC JAUNDICE  

PubMed Central

1. In four animals with a bile duct-ureter anastomosis and without disturbance due to obstruction or absorption, the total quantity of bile pigment output during a day under normal conditions varied from 0.0618 to 0.0678 gm. These figures are practically identical with those of Stadelmann (9, 10) but lower than those given by Hooper and Whipple (7), who find that the average bile pigment excretion amounts to about 1 mg. per pound of body weight per 6 hours. 2. In all the experiments there is definite evidence of a decrease in bile pigment elimination after splenectomy. This is true not only of the elimination when no hemolytic agent is administered but also when excessive blood destruction is caused. Under the latter circumstances the amount of bile pigment is greatly increased but never reaches the high level of blood destruction before splenectomy. 3. These observations appear to show conclusively that the absence of the spleen influences the formation of bile pigment. To what extent the influence is mechanical, i.e., change in the course of the blood to the liver, and to what extent due to anemia, cannot be stated at present.

Goto, Kingo

1917-01-01

225

Pulmonary Hypertension in Hemolytic Disorders  

PubMed Central

The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. The pathogenesis of pulmonary hypertension in hemolytic disorders is likely multifactorial, including hemolysis, impaired nitric oxide (NO) bioavailability, chronic hypoxemia, chronic thromboembolic disease, chronic liver disease, and asplenia. In contrast to patients with traditional forms of pulmonary arterial hypertension, patients with hemolytic disorders have a mild-to-moderate degree of elevation in mean pulmonary pressures, with mild elevations in pulmonary vascular resistance. The hemodynamic etiology of pulmonary hypertension in these patients is multifactorial and includes pulmonary arterial hypertension, pulmonary venous hypertension, and pulmonary hypertension secondary to a hyperdynamic state. Currently, there are limited data on the effects of any specific treatment modality for pulmonary hypertension in patients with hemolytic disorders. It is likely that maximization of treatment of the primary hemoglobinopathy in all patients and treatment with selective pulmonary vasodilators and antiproliferative agents in patients with pulmonary arterial hypertension would be beneficial. However, there is still a major need for large multinational trials of novel therapies for this patient population.

Gladwin, Mark T.

2010-01-01

226

S-adenosylhomocysteine hydrolase, S-adenosylmethionine, S-adenosylhomocysteine: correlations with ribavirin induced anemia  

Microsoft Academic Search

Objective is to speculate on the ribavirin induced anemia by inhibiting S-adenosylhomocysteine (SAH)-hydrolase activity. The major toxicity associated with the use of ribavirin is hemolytic anemia. This adverse effect has been ascribed to the accumulation of ribavirin triphosphate in the erythrocyte, which interferes with erythrocyte function. Ribavirin has been found to inhibit SAH-hydrolase activity in erythrocyte. SAH is further hydrolyzed

Engin Altintas; Orhan Sezgin

2004-01-01

227

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

Microsoft Academic Search

Serum bilirubin levels have been associated with polymorphisms in the UGT1A1 promoter in normal populations and in patients with hemolytic anemias, including sickle cell anemia. When hemolysis occurs circulating heme increases, leading to elevated bilirubin levels and an increased incidence of cholelithiasis. We performed the first genome-wide association study (GWAS) of bilirubin levels and cholelithiasis risk in a discovery cohort

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

2012-01-01

228

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.

Cobo, Viviane de Almeida; Chapadeiro, Cibele Alves; Ribeiro, Joao Batista; Moraes-Souza, Helio; Martins, Paulo Roberto Juliano

2013-01-01

229

Iron refractory iron deficiency anemia.  

PubMed

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

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

2013-06-01

230

Post-transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation.  

PubMed

HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. While "typical" HUS is usually associated with Shiga toxin-producing Escherichia coli infections and recovers in the majority of cases, aHUS is caused by mutations of complement components or antibodies against CFH leading to uncontrolled activation of alternative complement pathway and often to ESRD. Recently, THBD gene mutations have been reported in aHUS. Theoretically, the risk of disease recurrence after renal transplantation should be low because THBD is primarily a membrane-bound protein expressed by endothelial cells; however, a small proportion of THBD is present as a soluble form in plasma. We report the case of a 19-yr-old man with aHUS secondary to a THBD mutation that relapsed twice after two renal transplantations performed 12 yr apart. Despite successful control of HUS with plasma exchange and eculizumab after the second transplantation, the graft was ultimately lost due to severe steroid-resistant cellular rejection. The present report suggests that THBD mutations may favor-relapse of aHUS after renal transplantation. PMID:24118826

Sinibaldi, Serena; Guzzo, Isabella; Piras, Rossella; Bresin, Elena; Emma, Francesco; Dello Strologo, Luca

2013-09-30

231

Parvovirus B19 infection in Tunisian patients with sickle-cell anemia and acute erythroblastopenia  

Microsoft Academic Search

BACKGROUND: Human parvovirus B19 is the etiologic agent of erythema infectiosum in children. It is also associated with other clinical manifestations in different target groups. Patients with chronic hemolytic anemia are at high risk of developing acute erythroblastopenia following infection by the virus. They usually become highly viremic and pose an increased risk of virus transmission. Close monitoring of such

Faouzi Regaya; Lassad Oussaief; Mohamed Bejaoui; Mongi Karoui; Mohamed Zili; Ridha Khelifa

2007-01-01

232

Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome.  

PubMed

Pathologic thrombosis is a major cause of mortality. Hemolytic-uremic syndrome (HUS) features episodes of small-vessel thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia and renal failure. Atypical HUS (aHUS) can result from genetic or autoimmune factors that lead to pathologic complement cascade activation. Using exome sequencing, we identified recessive mutations in DGKE (encoding diacylglycerol kinase ?) that co-segregated with aHUS in nine unrelated kindreds, defining a distinctive Mendelian disease. Affected individuals present with aHUS before age 1 year, have persistent hypertension, hematuria and proteinuria (sometimes in the nephrotic range), and develop chronic kidney disease with age. DGKE is found in endothelium, platelets and podocytes. Arachidonic acid-containing diacylglycerols (DAG) activate protein kinase C (PKC), which promotes thrombosis, and DGKE normally inactivates DAG signaling. We infer that loss of DGKE function results in a prothrombotic state. These findings identify a new mechanism of pathologic thrombosis and kidney failure and have immediate implications for treating individuals with aHUS. PMID:23542698

Lemaire, Mathieu; Frémeaux-Bacchi, Véronique; Schaefer, Franz; Choi, Murim; Tang, Wai Ho; Le Quintrec, Moglie; Fakhouri, Fadi; Taque, Sophie; Nobili, François; Martinez, Frank; Ji, Weizhen; Overton, John D; Mane, Shrikant M; Nürnberg, Gudrun; Altmüller, Janine; Thiele, Holger; Morin, Denis; Deschenes, Georges; Baudouin, Véronique; Llanas, Brigitte; Collard, Laure; Majid, Mohammed A; Simkova, Eva; Nürnberg, Peter; Rioux-Leclerc, Nathalie; Moeckel, Gilbert W; Gubler, Marie Claire; Hwa, John; Loirat, Chantal; Lifton, Richard P

2013-03-31

233

A case of late-onset systemic lupus erythematosus with severe anemia.  

PubMed

A 59-year-old woman was referred to our hospital because of severe anemia and leucopenia. Although she developed mild arthralgia without the typical symptoms of systemic lupus erythematosus (SLE), positivity for anti-Sm antibodies led us to a diagnosis of late-onset SLE. Autoimmune hemolytic anemia (AIHA) and suppression of reticulocyte production were considered to have been involved in the etiology of severe anemia. Administration of oral prednisolone (PSL) resulted in a marked improvement of the hematological abnormalities. As late-onset SLE is rare and patients tend to show the typical symptoms less frequently, close attention should be focused on latent symptoms and immunological findings. PMID:23925156

Matsumoto, Moeko; Kaieda, Shinjiro; Honda, Seiyo; Ida, Hiroaki; Hoshino, Tomoaki; Fukuda, Takaaki

2013-08-07

234

Postoperative hemolytic uremic syndrome with renal cortical necrosis following laparoscopic hemicolectomy.  

PubMed

Non-Shiga-like toxin-producing Escherichia coli (STEC) or atypical hemolytic uremic syndrome (aHUS) is observed in 5-10% of all hemolytic uremic syndrome (HUS) cases, and usually develops secondary to infections, malignancies, drugs, transplantation, pregnancy, and autoimmune disease. However, there has been no report on adult onset HUS initiated by surgical procedures except transplantation. We report a 66-year-old woman who incurred renal impairment on the first day after laparoscopic hemicolectomy. Hemolytic anemia, thrombocytopenia, absence of Shiga toxin associated disease, normal ADAMTS13 activity, and low serum C3 (not C4) were consistent with a diagnosis of aHUS. We performed plasma exchange with fresh frozen plasma. Nevertheless, deteriorated renal function was not recovered after the treatment. Although it is an uncommon postoperative complication, aHUS needs to be considered as a possible cause of acute kidney injury combined with thrombocytopenia and anemia after surgical procedures, considering its different treatment modality and poor outcomes. PMID:23560430

Lee, Jae-Won; Won, Nam Hee; Cho, Eunjung; Kim, Myung-Gyu; Jo, Sang-Kyung; Cho, Won Yong; Kim, Hyoung Kyu

2013-04-08

235

Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab.  

PubMed

Uncontrolled complement activation is central to the occurrence of atypical hemolytic uremic syndrome (aHUS) and can result in thrombotic microangiopathies (TMAs). These terms encompass a group of heterogenic inherited or acquired diseases that recent research suggests may be triggered by the complement cascade. Pathogenetic triggers of complement activation include immunologic disorders, genetics, infections, systemic diseases, pregnancy, drug administration, metabolic diseases, transplantation, or triggers of mixed cause. Hallmarks of aHUS and other TMAs include increased vascular endothelium thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, coagulation abnormalities, and vascular shear stress, whereas common end points of these mechanisms include hemolytic anemia, thrombocytopenia with microvascular infarction, and predisposition for decreased kidney function and other organ involvement. The central role of the complement cascade as a disease trigger suggests a possible therapeutic target. Eculizumab, a first-in-class humanized monoclonal anti-C5 antibody that has been successful in the treatment of paroxysmal nocturnal hemoglobinuria, a disorder of complement-induced hemolytic anemia, received approval for the treatment of aHUS in the United States and Europe in late 2011. We review the treatment of aHUS and other TMAs, focusing on the role of eculizumab, including its pharmacology, mechanism of action, and approved dosing recommendations and health economic considerations. Finally, the potential for future indications for eculizumab use in other complement-driven diseases is discussed. PMID:23141475

Schmidtko, Jan; Peine, Sven; El-Housseini, Youssef; Pascual, Manuel; Meier, Pascal

2012-11-07

236

Optimal management of pernicious anemia  

PubMed Central

Pernicious anemia (also known as Biermer’s disease) is an autoimmune atrophic gastritis, predominantly of the fundus, and is responsible for a deficiency in vitamin B12 (cobalamin) due to its malabsorption. Its prevalence is 0.1% in the general population and 1.9% in subjects over the age of 60 years. Pernicious anemia represents 20%–50% of the causes of vitamin B12 deficiency in adults. Given its polymorphism and broad spectrum of clinical manifestations, pernicious anemia is a great pretender. Its diagnosis must therefore be evoked and considered in the presence of neurological and hematological manifestations of undetermined origin. Biologically, it is characterized by the presence of anti-intrinsic factor antibodies. Treatment is based on the administration of parenteral vitamin B12, although other routes of administration (eg, oral) are currently under study. In the present update, these various aspects are discussed with special emphasis on data of interest to the clinician.

Andres, Emmanuel; Serraj, Khalid

2012-01-01

237

Hemolytic Uremic Syndrome after Bone Marrow Transplantation  

Microsoft Academic Search

A 16-year-old female developed a hemolytic uremic syndrome 9 months after undergoing bone marrow transplantation for acute lymphoblastic leukemia during second relapse. There appears to be no etiologic relationship between the post-transplant immune status and the hemolytic uremic syndrome. The patient succumbed later to recurrent leukemia.Copyright © 1982 S. Karger AG, Basel

W. E. Spruce; S. J. Forman; K. G. Blume; R. M. Bearman; H. Bixby; A. Ching; J. Drinkard; San Marco

1982-01-01

238

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

PubMed

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

2013-10-07

239

Early Complication in Sickle Cell Anemia Children due to A(TA)nTAA Polymorphism at the Promoter of UGT1A1 Gene  

PubMed Central

Aim. To determine the implication of the polymorphism, namely, A(TA)nTAA of UGT1A1 in lithogenesis for the first time in Tunisia among sickle cell anemia (SCA) children patients. Material and Methods. Our study was performed in 2010 and it involved 76 subjects chosen as control group characterized with normal hemoglobin status and presence of cholelithiasis and 102 SCA pediatric patients among whom 52 have cholelithiasis. We analyzed the polymorphism A(TA)nTAA at the UGT1A1 promoter and the relationships between the various A(TA)nTAA genotypes and alleles and bilirubin levels and occurrence of cholelithiasis. Results and Discussion. The repartition of genotypes found according to serum bilirubin level shows a significant association between genotypes carrying variant (TA)7 and hyperbilirubinemia (P < 0.05). We demonstrated the association of two genotypes with gallstones formation among SCA children patients: (TA)7/(TA)7 and (TA)7/(TA)8 with P = 8.1 × 10?8 and P = 0.01, respectively. (TA)7 and (TA)8 allele variants act as a risk factor for early gallstones formation in SCA patients with P = 5.8 × 10?9 and P = 0.01, respectively. As for the control group only the genotype (TA)7/(TA)7 presented a risk factor for gallstones formation. Conclusion. The novelty of this report is that it is the first time that a similar study was made on the Tunisian children sickle cell population and that the results show a clear association of (TA)7 variant in early gallstones formation in Tunisian SCA children. Interestingly our findings highlighted the association of (TA)8 variant as well, which was not found in previous studies.

Chaouch, Leila; Talbi, Emna; Moumni, Imen; Ben Chaabene, Arij; Kalai, Miniar; Chaouachi, Dorra; Mallouli, Fethi; Ghanem, Abderraouf; Abbes, Salem

2013-01-01

240

[Differential diagnosis of anemia].  

PubMed

Anemia is often encountered in clinical practice. It has to be noted that anemia is only a symptom of an underlying disease. Early, correct and consequent diagnosis is crucial in order to prevent late detection of fatal disease or to prescribe contraindicated therapy. The combined use of a morphologic and physiologic classification allows for understanding and correct classification of anemia in a specific patient. In this respect, the determination of reticulocyte counts is of special importance. Further targeted biochemical, morphological and immunological analysis leads to the definitive diagnosis of anemia. A large proportion of the anemias can be diagnosed and treated by the general practitioner. However, involvement of a haematologist is recommended in several clinical situations, such as newly diagnosed haemolytic anemia, as well as in patients with complex, equivocal or therapy-refractory anemia or certain normocytic hyporegenerative anemias. This paper presents a differentiated stepwise approach in diagnosing anemic disorders. PMID:15018393

Risch, L; Herklotz, R; Huber, A R

2004-02-01

241

Iron-Deficiency Anemia  

MedlinePLUS Videos and Cool Tools

... NHLBI Entire Site NHLBI Entire Site 1 Health Topics 2 News & Resources 3 Intramural Research 4 Home » Health Information for the Public » Health Topics » Iron-Deficiency Anemia » What Is ... Iron-Deficiency Anemia ...

242

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

243

Sickle Cell Anemia (SCD)  

MedlinePLUS

Sickle Cell Anemia (SCD) Sickle cell anemia (also called sickle cell disease or SCD) is an inherited disease in ... decisions Sickle cell trait More information References Sickle cell disease symptoms and diagnosis Normal healthy red blood ...

244

Genetics Home Reference: Anemia  

MedlinePLUS

... REN-related kidney disease sickle cell disease thiamine-responsive megaloblastic anemia syndrome X-linked sideroblastic anemia X- ... Reference. Links to web sites outside the Federal Government do not constitute an endorsement. See Selection Criteria ...

245

Literacy Measure B - Anemia  

Center for Drug Evaluation (CDER)

... Literacy Measure B - Anemia. ANEMIA. Frequency. Percent. Valid Percent. Cumulative Percent. Valid, Correct, 755, 83.5, 83.5, 83.5. ... More results from www.fda.gov/drugs/developmentapprovalprocess/developmentresources

246

Fanconi Anemia: Diagnosis  

MedlinePLUS

FA Diagnosis Fanconi anemia usually reveals itself before children are 12 years old, but in rare cases no symptoms are present until adulthood. Fanconi anemia patients are usually smaller than average. They may ...

247

Diagnosis and management of pernicious anemia.  

PubMed

Pernicious anemia is a macrocytic anemia due to cobalamin deficiency, which is the result of intrinsic factor deficiency. Pernicious anemia is associated with atrophic body gastritis, whose diagnostic criteria are based on the histologic evidence of gastric body atrophy associated with hypochlorhydria. Serological markers suggesting the presence of oxyntic mucosa damage are increased levels of fasting gastrin and decreased levels of Pepsinogen I. Without the now obsolete Schilling's test, intrinsic factor deficiency may not be proven, and gastric intrinsic factor output after pentagastric stimulation has been proposed. Intrinsic factor autoantibodies are useful surrogate markers of pernicious anemia. The management of patients with pernicious anemia should focus on the life-long replacement treatment with cobalamin and the monitoring to early diagnose an eventual onset of iron deficiency. Moreover, these patients should be advised about possible gastrointestinal long-term consequences, such as gastric cancer and carcinoids. PMID:21947876

Annibale, Bruno; Lahner, Edith; Fave, Gianfranco Delle

2011-12-01

248

The patient with anemia  

Microsoft Academic Search

Anemia is not a diagnosis but usually a sign of a systemic illness. The clinical manifestations of anemia depend on its rate of development and severity. This paper presents a simple pathophysiologic classification of the various types of anemia based on the patient's medical history, physical findings, peripheral blood morphology, and signs of bone marrow activity as reflected in the

Fred Rosner; Hans W Grünwald

1997-01-01

249

Hematologic Emergencies: Acute Anemia  

Microsoft Academic Search

Anemia can be seen in the emergency department both as a primary pathological process or secondary to both medical and surgical diseases. Moreover, acute anemia can occur in children who have been otherwise healthy, who have systemic disease, or who have known hematologic disorders. Anemia may indicate a disorder with a single hematopoietic cell line (eg, red blood cells) or

Samuel C. Blackman; Javier A. Gonzalez del Rey

2005-01-01

250

Iron Deficiency Anemia  

Microsoft Academic Search

The prevalence of iron deficiency anemia is 2 percent in adult men, 9 to 12 percent in non-Hispanic white women, and nearly 20 percent in black and Mexican-American women. Nine percent of patients older than 65 years with iron deficiency anemia have a gastrointestinal cancer when evaluated. The U.S. Preventive Services Task Force currently recommends screening for iron deficiency anemia

SHERSTEN KILLIP; JOHN M. BENNETT; MARA D. CHAMBERS

251

Anemia and heart failure  

Microsoft Academic Search

Over the past few years, anemia has emerged as a powerful independent predictor of adverse outcomes in chronic heart failure\\u000a (CHF). It affects up to 50% of patients with CHF, depending on the definition of anemia used and on the population studied.\\u000a Even small reductions in hemoglobin are associated with worse outcome. However, the causes of anemia in CHF remain

Eileen O’Meara; Clare Murphy; John J. V. McMurray

2004-01-01

252

Hypogonadism and anemia in an athlete.  

PubMed

We report the case of a highly trained endurance athlete (22-year-old) who developed anemia (Hb 9.5?mg/dl) over a period of 6 months. Iron deficient or haemolytic anemia, as well as chronic loss of blood, were excluded. Further, laboratory analyses revealed that this athlete exhibited very low levels of testosterone due to a partial hypogonadotropic hypogonadism. Following testosterone supplementation, red blood cell indices improved. Although hypogonadotropic hypogonadism is well known to be associated with reduced hematopoesis, it rarely causes anemia in athletes. This should be considered as a possible cause for anemia. Extreme training, unbalanced nutrition or the combination of both, have been shown to be causally involved in the development of secondary hypogonadotropic hypogonadism. PMID:22095327

Korsten-Reck, U; Seufert, J; Dickhuth, H-H; Schumacher, Y O; König, D

2011-11-17

253

Managing the hematologic side effects of antiviral therapy for chronic hepatitis C: anemia, neutropenia, and thrombocytopenia.  

PubMed

Hematologic abnormalities such as anemia, neutropenia, and thrombocytopenia are common during combination therapy with pegylated (or standard) interferon and ribavirin for chronic hepatitis C. Ribavirin-induced hemolytic anemia is a common cause of dose reduction or discontinuation. Bone marrow suppression also contributes to the anemia and is the predominant mechanism for interferon-induced neutropenia and thrombocytopenia. Although dose reduction or discontinuation of combination therapy can reverse these abnormalities, they may reduce virologic response. Hematopoietic growth factors may provide a useful alternative for managing these hematologic side effects without reducing the optimal dose of the combination antiviral regimen. Treatment of anemia also may improve patients' health-related quality of life and their adherence to combination antiviral therapy. The impact of growth factors on sustained virologic response and their cost-effectiveness in patients with chronic hepatitis C need further assessment. PMID:15468613

Ong, Janus P; Younossi, Zobair M

2004-05-01

254

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

255

Sickle Cell Anemia  

MedlinePLUS

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

256

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

257

Mitochondrial iron metabolism and sideroblastic anemia.  

PubMed

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 are caused by defects in genes present on the X chromosome (mutations in the ALAS2, ABCB7, or GRLX5 gene), genes on autosomal chromosomes, or mitochondrial genes. Acquired sideroblastic anemias are either primary (refractory anemia with ring sideroblasts, RARS, representing one subtype of the myelodysplastic syndrome) or secondary due to some drugs, toxins, copper deficiency, or chronic neoplastic disease. The pathogenesis of mitochondrial iron loading in developing erythroblasts is diverse. Ring sideroblasts can develop as a result of a heme synthesis defect in erythroblasts (ALAS2 mutations), a defect in iron-sulfur cluster assembly, iron-sulfur protein precursor release from mitochondria (ABCB7 mutations), or by a defect in intracellular iron metabolism in erythroid cells (e.g. RARS). PMID:19907149

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

2009-11-10

258

Prediction of fetal anemia by Doppler of the middle cerebral artery and descending thoracic aorta  

Microsoft Academic Search

Summary\\u000a Background  Despite significant advances, perinatal hemolytic disease has not been eradicated and is still associated with significant\\u000a morbidity and mortality, especially in developing countries. An accurate method for antenatal diagnosis and quantification\\u000a of fetal anemia is a crucial step prior to the performance of invasive procedures, which are not risk-free. The middle cerebral\\u000a artery peak systolic velocity (MCA-PSV) is currently

David Pares; Paulo A. Chinen; Luiz Camano; Antonio F. Moron; Maria R. Torloni

2008-01-01

259

Anemias of Bone Marrow Failure  

Microsoft Academic Search

he anemias of chronic bone marrow failure are disorders other than iron deficiency, folate deficiency, and vitamin B12 deficiency in which anemia is present and the reticulocyte count does not increase appropriately. These anemias are commonly (but not always) normochromic and nor- mocytic. The differential diagnosis of the anemias of bone marrow failure is presented in Table 1. Although chronic

Richard S. Stein; Stacey Goodman

260

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.

Roy, Cindy N.

2010-01-01

261

Impact of Iron Supplementation on Anemia During Pregnancy  

Microsoft Academic Search

Pregnancy is a time in which the risk for developing iron deficiency anemia is highest, due to increase of iron requirement. Maternal nutrition is often considered as an important regulator of human fetal growth. Objectives: To study the impact of iron supplementation on anemia during pregnancy . Salty rice flakes preparation was prepared. Sixty volunteered pregnant women in their III

Taru Agarwal; G. K. Kochar; Sonali Goel

262

Hemolytic uremic syndrome and Clostridium difficile colitis  

PubMed Central

Hemolytic uremic syndrome (HUS) can be associated with different infectious etiologies, but the relationship between pseudomembranous colitis and HUS was first described in the 1970s in some childhood patients. There is very limited published literature on Clostridium difficile-associated HUS. We report a case of C. difficile-related HUS in an adult patient and provide a review of the literature.

Keshtkar-Jahromi, Maryam; Mohebtash, Mahsa

2012-01-01

263

Atypical hemolytic uremic syndrome with membranoproliferative glomerulonephritis.  

PubMed

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

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

2013-08-01

264

[Cardiorenal anemia syndrome (review)].  

PubMed

Cardiorenal anemia syndrome (CRAS) refers to the simultaneous presence of anemia, heart failure (HF), and chronic kidney disease (CKD) that forms a pathologic triad with an observe impact on morbidity and mortality. Certain researches were made regarding the usage of erythropoietin (EPO) in patients with the above mentioned disorders. This leads to the improvement of left ventricular function, quality of life and physical tolerance with decreased risk of hospitalization. Despite successful anemia treatment with EPO in dialysis patients with CKD, HF and cardiorenal syndrome type 2, it should be important to reveal the target Hb level and role of EPO in this category of patients. According to European guidelines in 85% of hemodialysis patients targeted Hb level should be no more than 11g/dl, moreover, the treatment of anemia can be organized before dialysis and it will certainly increase the quality of life in this type of patients. PMID:23293227

Minasyan, A

2012-12-01

265

Sickle cell anemia  

MedlinePLUS

... Other treatments for sickle cell anemia may include: Hydroxyurea (Hydrea), a medicine that may help reduce the ... National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med . ...

266

Hereditary sideroblastic anemias: pathophysiology, diagnosis, and treatment.  

PubMed

Inherited sideroblastic anemia comprises several rare anemias due to heterogeneous genetic lesions, all characterized by the presence of ringed sideroblasts in the bone marrow. This morphological aspect reflects abnormal mitochondrial iron utilization by the erythroid precursors. The most common X-linked sideroblastic anemia (XLSA), due to mutations of the first enzyme of the heme synthetic pathway, delta-aminolevulinic acid synthase 2 (ALAS2), has linked heme deficiency to mitochondrial iron accumulation. The identification of other genes, such as adenosine triphosphate (ATP) binding cassette B7 (ABCB7) and glutaredoxin 5 (GLRX5), has strengthened the role of iron sulfur cluster biogenesis in sideroblast formation and revealed a complex interplay between pathways of mitochondrial iron utilization and cytosolic iron sensing by the iron-regulatory proteins (IRPs). As recently occurred with the discovery of the SLC25A38-related sideroblastic anemia, the identification of the genes responsible for as yet uncharacterized forms will provide further insights into mitochondrial iron metabolism of erythroid cells and the pathophysiology of sideroblastic anemia. PMID:19786205

Camaschella, Clara

2009-10-01

267

[Anemia in selected diseases of the gastrointestinal tract in children].  

PubMed

Anemia is a frequent symptom of diseases of alimentary tract, also in children. Among others, inflammatory bowel disease, celiac disease and Helicobacter pylori are most often complicated by anemia. Not infrequently these disorders are accompanied by more than one type of anemia and moreover its pathogenesis may be complex. In children with inflammatory bowel disease iron deficiency anemia is predominant, which is caused by the loss and insufficient supply of iron, but also in this group of diseases anemia of chronic diseases pose a problem. In patient with celiac disease, especially in small children, the main cause of anemia is malabsorption of iron, also its loss due to microdamage of the intestine mucosa has also been observed. In Helicobacter pylori infection the origin of anemia is still being discussed. The treatment of iron deficiency anemia (most frequent in the diseases of the alimentary tract) consists mainly of the treatment of underlying disease, supply of iron in food and in the form of drugs. Transfusions of blood ingredients are done only in severe anemia leading to hemodynamic disturbances. Iron may be supplemented either by oral or intravenous route. PMID:23894782

Krzesiek, Elzbieta; Iwa?czak, Barbara

2013-05-01

268

Diagnosis of Fanconi anemia in patients with bone marrow failure  

PubMed Central

Background Patients with bone marrow failure and undiagnosed underlying Fanconi anemia may experience major toxicity if given standard-dose conditioning regimens for hematopoietic stem cell transplant. Due to clinical variability and/or potential emergence of genetic reversion with hematopoietic somatic mosaicism, a straightforward Fanconi anemia diagnosis can be difficult to make, and diagnostic strategies combining different assays in addition to classical breakage tests in blood may be needed. Design and Methods We evaluated Fanconi anemia diagnosis on blood lymphocytes and skin fibroblasts from a cohort of 87 bone marrow failure patients (55 children and 32 adults) with no obvious full clinical picture of Fanconi anemia, by performing a combination of chromosomal breakage tests, FANCD2-monoubiquitination assays, a new flow cytometry-based mitomycin C sensitivity test in fibroblasts, and, when Fanconi anemia was diagnosed, complementation group and mutation analyses. The mitomycin C sensitivity test in fibroblasts was validated on control Fanconi anemia and non-Fanconi anemia samples, including other chromosomal instability disorders. Results When this diagnosis strategy was applied to the cohort of bone marrow failure patients, 7 Fanconi anemia patients were found (3 children and 4 adults). Classical chromosomal breakage tests in blood detected 4, but analyses on fibroblasts were necessary to diagnose 3 more patients with hematopoietic somatic mosaicism. Importantly, Fanconi anemia was excluded in all the other patients who were fully evaluated. Conclusions In this large cohort of patients with bone marrow failure our results confirmed that when any clinical/biological suspicion of Fanconi anemia remains after chromosome breakage tests in blood, based on physical examination, history or inconclusive results, then further evaluation including fibroblast analysis should be made. For that purpose, the flow-based mitomycin C sensitivity test here described proved to be a reliable alternative method to evaluate Fanconi anemia phenotype in fibroblasts. This global strategy allowed early and accurate confirmation or rejection of Fanconi anemia diagnosis with immediate clinical impact for those who underwent hematopoietic stem cell transplant.

Pinto, Fernando O.; Leblanc, Thierry; Chamousset, Delphine; Le Roux, Gwenaelle; Brethon, Benoit; Cassinat, Bruno; Larghero, Jerome; de Villartay, Jean-Pierre; Stoppa-Lyonnet, Dominique; Baruchel, Andre; Socie, Gerard; Gluckman, Eliane; Soulier, Jean

2009-01-01

269

[Heart failure and anemia].  

PubMed

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

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

2013-09-01

270

Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis  

Microsoft Academic Search

Extramedullary hematopoiesis (EMH) is a rare disorder, characterized by the appearance of hematopoietic elements outside\\u000a of the bone marrow, which occurs in patients with chronic myeloproliferative disorders or congenital hemolytic anemias. We\\u000a report on a 64-year-old man with hereditary spherocytosis, who presented with anemia, jaundice, intrathoracic EMH, and massive\\u000a hemothorax. The diagnosis of EMH was established after computer tomography (CT)-guided

N. Xiros; T. Economopoulos; E. Papageorgiou; G. Mantzios; S. Raptis

2001-01-01

271

[An uncommon etiology of anemia: copper deficiency].  

PubMed

A 58-year-old patient, without any notable medical history, except for alcoholism and treated hypertension, developed anemia and leukopenia with macrocytosis. Folate deficiency was diagnosed and subsequently treated. Despite folate supplementation, the hematological parameters did not normalize. Further diagnosis investigations were led to search for uncommon etiologies of anemia and leukoneutropenia. We diagnosed severe copper deficiency on the basis of decreased plasma levels of copper and ceruloplasmin. Copper supplementation improved blood counts within three months. This case illustrates hematological disorders due to copper deficiency, initially masked by an associated folate deficiency. The copper deficiency etiology was not identified in this case. PMID:23906580

Kouamou, Edwige; Stépanian, Alain; Khadra, Fadi; de Prost, Dominique; Teillet, France

272

Molecular pathogenesis in Diamond-Blackfan anemia.  

PubMed

Diamond-Blackfan anemia (DBA) is a congenital anemia and a broad spectrum of developmental abnormalities that presents soon after birth. The anemia is due to a failure of erythropoiesis with normal platelet and myeloid lineages. Approximately 10-20% of DBA cases are inherited. Genetic studies have identified heterozygous mutations in at least one of eight ribosomal protein genes in up to 50% of cases. Mutations in RPL5 and RPL11 are at a high risk for developing malformation. Especially, mutations in RPL5 are associated with multiple physical abnormalities, including cleft lip/plate and thumb and heart anomalies. Recently, the 5q- syndrome, a subtype of myelodysplastic syndrome characterized by a defect in erythroid differentiation, is caused by a somatically acquired deletion of chromosome 5q, which results in haploinsufficiency of RPS14. These data indicate that abnormalities in ribosome function are broadly implicated in both congenital and acquired bone marrow failure syndrome in humans. PMID:20882441

Ito, Etsuro; Konno, Yuki; Toki, Tsutomu; Terui, Kiminori

2010-09-30

273

The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe  

PubMed Central

The intensity of hemolytic anemia has been proposed as an independent risk factor for the development of certain clinical complications of sickle cell disease, such as pulmonary hypertension, hypoxemia and cutaneous leg ulceration. A composite variable derived from several individual markers of hemolysis could facilitate studies of the underlying mechanisms of hemolysis. In this study, we assessed the association of hemolysis with outcomes in sickle cell anemia. A hemolytic component was calculated by principal component analysis from reticulocyte count, serum lactate dehydrogenase, aspartate aminotransferase and total bilirubin concentrations in 415 hemoglobin SS patients. Association of this component with direct markers of hemolysis and clinical outcomes was assessed. As primary validation, both plasma red blood cell microparticles and cell-free hemoglobin concentration were higher in the highest hemolytic component quartile compared to the lowest quartile (P?0.0001 for both analyses). The hemolytic component was lower with hydroxyurea therapy, higher hemoglobin F, and alpha-thalassemia (P?0.0005); it was higher with higher systemic pulse pressure, lower oxygen saturation, and greater values for tricuspid regurgitation velocity, left ventricular diastolic dimension and left ventricular mass (all P<0.0001). Two-year follow-up analysis showed that a high hemolytic component was associated with an increased risk of death (hazard ratio, HR 3.44; 95% confidence interval, CI: 1.2–9.5; P=0.02). The hemolytic component reflects direct markers of intravascular hemolysis in patients with sickle cell disease and allows for adjusted analysis of associations between hemolytic severity and clinical outcomes. These results confirm associations between hemolytic rate and pulse pressure, oxygen saturation, increases in Doppler-estimated pulmonary systolic pressures and mortality (Clinicaltrials.gov identifier: NCT00492531).

Nouraie, Mehdi; Lee, Janet S.; Zhang, Yingze; Kanias, Tamir; Zhao, Xuejun; Xiong, Zeyu; Oriss, Timothy B.; Zeng, Qilu; Kato, Gregory J.; Gibbs, J. Simon R.; Hildesheim, Mariana E.; Sachdev, Vandana; Barst, Robyn J.; Machado, Roberto F.; Hassell, Kathryn L.; Little, Jane A.; Schraufnagel, Dean E.; Krishnamurti, Lakshmanan; Novelli, Enrico; Girgis, Reda E.; Morris, Claudia R.; Rosenzweig, Erika Berman; Badesch, David B.; Lanzkron, Sophie; Castro, Oswaldo L.; Goldsmith, Jonathan C.; Gordeuk, Victor R.; Gladwin, Mark T.

2013-01-01

274

Enhanced Hemolytic Antibody Response Following Thermal Injury  

Microsoft Academic Search

This paper presents evidence of a significantly enhanced humoral immune response to sheep red cells following thermal injury, as assayed by direct hemolytic antibody plaque formation. An increase in the number of 19 S IgM antibody-secreting cells from the spleen was observed in rats up to 16 days after a 30-percent total body surface area, third-degree, scald burn. Since this

R. F. Mortensen; K. Eurenius

1972-01-01

275

Facts about Diamond Blackfan Anemia  

MedlinePLUS

... message, please visit this page: About CDC.gov . Diamond Blackfan Anemia (DBA) Share Compartir Add this to... ... Favorites Delicious Digg Google Bookmarks Facts About DBA Diamond Blackfan anemia (DBA) is a rare blood disorder ...

276

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

277

Congenital dyserythropoietic anemias.  

PubMed

The congenital dyserythropoietic anemias (CDAs) are a group of relatively rare inherited anemias that share in common ineffective erythropoiesis and morphologic abnormalities of mature red blood cells and their precursors. Three major types of CDA and a number of variants have been described. The diagnosis and categorization of these disorders are facilitated by microscopic examination of the blood and bone marrow and by serologic testing. Management of patients currently consists of observation and supportive care. Because patients with CDAs may be at significant risk for secondary hemochromatosis, they require monitoring for this condition. Splenectomy may be of benefit in certain cases in which the anemia is particularly severe. Over the past few years advances have been made in understanding the pathogenesis of these disorders, and it now appears that CDA II results from enzymatic defects in the cellular glycosylation pathway. PMID:8571938

Marks, P W; Mitus, A J

1996-01-01

278

Nutritional anemia and its control  

Microsoft Academic Search

Available studies on prevalence of nutritional anemia in India show that 65% infant and toddlers, 60% 1–6 years of age, 88%\\u000a adolescent girls (3.3% had hemoglobin < 7.0 g\\/dl; severe anemia) and 85% pregnant women (9.9% having severe anemia) were anemic.\\u000a The prevalence of anemia was marginally higher in lactating women as compared to pregnancy. The commonest is iron deficiency

Deeksha Kapur; Kailash Nath Agarwal; Dev Kumari Agarwal

2002-01-01

279

Mechanisms of Anemia in CKD  

PubMed Central

Anemia is a common feature of CKD associated with poor outcomes. The current management of patients with anemia in CKD is controversial, with recent clinical trials demonstrating increased morbidity and mortality related to erythropoiesis stimulating agents. Here, we examine recent insights into the molecular mechanisms underlying anemia of CKD. These insights hold promise for the development of new diagnostic tests and therapies that directly target the pathophysiologic processes underlying this form of anemia.

Lin, Herbert Y.

2012-01-01

280

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.

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

281

Atypical hemolytic-uremic syndrome: the interplay between complements and the coagulation system.  

PubMed

Hemolytic-uremic syndrome (HUS) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and impaired renal function. A thrombotic microangiopathy underlies the clinical features of HUS. In the majority of cases, HUS follows an infection with toxin-producing bacteria such as verotoxin-producing Escherichia coli. In some cases, HUS is not preceded by a clinically apparent infection, and therefore, is named atypical HUS. The prognosis of atypical HUS is poor. While mortality approaches 25% during the acute phase, end-stage renal disease develops in nearly half of patients within a year. Evidence is accumulating that complement activation through the alternative pathway is at the heart of the pathophysiology leading to atypical HUS. Genetic abnormalities involving complement regulatory proteins and complement components form the molecular basis for complement activation. Since microvascular thrombosis is a quintessential feature of atypical HUS, complements and the coagulation system must work in tandem to give rise to the pathologic alterations observed in this condition. Here, a brief discussion of clinical and morphologic features of atypical HUS is followed by a concise presentation of the complement and coagulation systems. The interplay between complements and the coagulation system is graphically highlighted. Last but not least, conventional and emerging therapies for atypical HUS are outlined. PMID:24072143

Nayer, Ali; Asif, Arif

2013-09-01

282

Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis.  

PubMed

Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far. PMID:23557793

Povey, Hannah; Vundru, Rahul; Junglee, Naushad; Jibani, Mahdi

2013-04-01

283

Preservation of renal function in atypical hemolytic uremic syndrome by eculizumab: a case report.  

PubMed

Genetic mutations in complement components are associated with the development of atypical hemolytic uremic syndrome (aHUS), a rare disease with high morbidity rate triggered by infections or unidentified factors. The uncontrolled activation of the alternative pathway of complement results in systemic endothelial damage leading to progressive development of renal failure. A previously healthy 8-month-old boy was referred to our hospital because of onset of fever, vomiting, and a single episode of nonbloody diarrhea. Acute kidney injury with preserved diuresis, hemolytic anemia, and thrombocytopenia were detected, and common protocols for management of HUS were followed without considerable improvement. The persistent low levels of complement component C3 led us to hypothesize the occurrence of aHUS. In fact, the child carried a specific mutation in complement factor H (Cfh; nonsense mutation in 3514G>T, serum levels of Cfh 138 mg/L, normal range 350-750). Given the lack of response to therapy and the occurrence of kidney failure requiring dialysis, we used eculizumab as rescue therapy, a monoclonal humanized antibody against the complement component C5. One week from the first administration, we observed a significant improvement of all clinical and laboratory parameters with complete recovery from hemodialysis, even in the presence of systemic infections. Our case report shows that complement inhibiting treatment allows the preservation of renal function and avoids disease relapses during systemic infections. PMID:23027168

Giordano, Mario; Castellano, Giuseppe; Messina, Giovanni; Divella, Claretta; Bellantuono, Rosa; Puteo, Flora; Colella, Vincenzo; Depalo, Tommaso; Gesualdo, Loreto

2012-10-01

284

Manifestation of atypical hemolytic uremic syndrome caused by novel mutations in MCP.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Mutations in genes encoding regulators of the alternative complement pathway (CFH, MCP, C3, CFI, CFB, THBD, and CFHR1-5) are connected with this disease. Polymorphisms (SNPs) in these genes might also influence the manifestation of aHUS. We have analyzed the genes of CFH, CFI, MCP, and C3 in a cohort of 10 unrelated Czech patients with clinically diagnosed familial aHUS. Surprisingly, 4 patients had mutations only in MCP, without mutations in any of the other genes that cause aHUS. Mutations, as yet unpublished, were widely distributed over the gene (SCR2 domain, signal peptide, and cytoplasmic region). The phenotype of the patients and their close relatives (14 individuals) was also investigated. Functional examination of MCP was also provided and proved lower expression on granulocytes in all mutations. Severity of disease varied, but onset was never earlier than 5 years of age. Penetrance of disease was 50% among carriers. We found that the severity and recurrence of the disease within families varied and might also be dependent on SNPs. Mutations in the MCP gene seems to be a common etiology of aHUS in Czech patients. PMID:21706448

Provaznikova, Dana; Rittich, Simon; Malina, Michal; Seeman, Tomas; Marinov, Iuri; Riedl, Magdalena; Hrachovinova, Ingrid

2011-06-27

285

Unexplained Anemia in the Elderly  

PubMed Central

Among the elderly, anemia occurs with increasing frequency with each advancing decade. Unlike when anemia occurs in younger adults, the cause of anemia in the elderly is oftentimes not readily apparent or attributable to a single cause. However, this commonly observed form of anemia in the elderly (termed unexplained anemia [UA]) can generally be dissected to its root causes, which include renal insufficiency, inflammation, testosterone deficiency, and stem cell proliferative decline. Myelodysplasia (MDS) occurs commonly in this age group but can and should, for both diagnostic and therapeutic considerations, be distinguished from UA.

Makipour, Sasan; Kanapuru, Bindu; Ershler, William B.

2008-01-01

286

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 Gomollón; Javier P Gisbert

2009-01-01

287

Anemia ferropriva na infância  

Microsoft Academic Search

Objective: To present the aspects involved in iron deficiency anemia in children as well as the probabilities of minimizing its nutritional effects. Method: The authors made an extensive review of national and international literature, and associated findings to their own experi- ence in this area. The study led to an overview that included general aspects of iron metabolism, iron deficiency,

Suzana de Souza Queiroz; Marco A. de A. Torres

2000-01-01

288

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

289

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.

290

Anemia and Pregnancy  

MedlinePLUS

... red blood cells that carries oxygen to other cells in your body. Many women lack the sufficient amount of iron needed for the second and third trimesters. When your body needs more iron than it has available, you can become anemic. Mild anemia is normal during ...

291

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

PubMed

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 requires special clinical attention in terms of diagnosis and therapy. DEAP-HUS and other atypical forms of HUS share common features, such as microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. However, DEAP-HUS has the unique combination of an acquired factor in the form of autoantibodies to the complement inhibitor Factor H and a genetic factor which, in most cases, is the chromosomal deletion of a 84-kbp fragment within human chromosome 1 that results in the absence of the CFHR1 and CFHR3 proteins in plasma. Special attention is required to diagnose and treat DEAP-HUS patients. Most patients show a favorable response to the reduction of autoantibody titers by either plasma therapy, steroid treatment, and/or immunosuppression. In addition, in those DEAP-HUS patients with end-stage renal disease, the reduction of autoantibody titers prior to transplantation is expected to prevent post-transplant disease recurrence by aiming for full complement control at the endothelial cell surface in order to minimize adverse complement and immune reactions. PMID:20157737

Zipfel, Peter F; Mache, Christoph; Müller, Dominik; Licht, Christoph; Wigger, Marianne; Skerka, Christine

2010-02-16

292

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.

Gomollon, Fernando; Gisbert, Javier P

2009-01-01

293

Iron-Deficiency Anemia: Reexamining the Nature and Magnitude of the Public Health Problem An Analysis of Anemia and Child Mortality1,2  

Microsoft Academic Search

The relationship of anemia as a risk factor for child mortality was analyzed by using cross-sectional, longitudinal and case-control studies, and randomized trials. Five methods of estimation were adopted: 1) the proportion of child deaths attributable to anemia; 2) the proportion of anemic children who die in hospital studies; 3) the population-attributable risk of child mortality due to anemia; 4)

Bernard J. Brabin; Zulfiqarali Premji; Francine Verhoeff

294

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

295

Hemolytic uremic syndrome caused by enteroviral infection.  

PubMed

A 4-year-old boy presented with enteroviral infection complicated with atypical hemolytic uremic syndrome (aHUS). Enterovirus RNA was detected by reverse transcription polymerase chain reaction (RT-PCR) of both blood and kidney biopsy specimens. A survey of the complement system did not reveal a specific complement defect. Supportive therapy with blood components transfusion, plasma therapy, and immunosuppressants was administered, however, renal function did not recover. The results of this report demonstrate that the enterovirus is the cause of aHUS. PMID:23597514

Lee, Ming-Dar; Tzen, Chin-Yuan; Lin, Chun-Chen; Huang, Fu-Yuan; Liu, Hsi-Che; Tsai, Jeng-Daw

2012-12-14

296

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura  

Microsoft Academic Search

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis, and partial or complete obstruction of the vessel lumina. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different entities have been described: the hemolytic uremic syndrome

Piero Ruggenenti; Marina Noris; Giuseppe Remuzzi

2001-01-01

297

Hemolytic effects of crude venom from Aiptasia mutabilis nematocysts  

Microsoft Academic Search

The aim of this paper was to focus on the toxinological aspects of microbasic-mastigophore nematocysts isolated from acontia of Aiptasia mutabilis, an Anthozoan collected in the Strait of Messina, by performing hemolytic assay on human, chicken, and rabbit red blood cells in suspension. The hemolytic effects of single isolated nematocysts were achieved by checking the lytic pattern after discharge. Crude

A. Marino; G. Musci; G. La Spada

2004-01-01

298

Congenital dyserythropoietic anemia  

Microsoft Academic Search

Congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of rare hereditary disorders of erythropoiesis characterized\\u000a by morphologic abnormal erythroblasts in the bone marrow. Three types of the disease are known as type I, II and III, and\\u000a the variant type of CDA and several minor subgroups of CDA have been also reported since the first classification. Recently,\\u000a responsible genes for

Takahiro Kamiya; Atsushi Manabe

2010-01-01

299

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

300

Iron Deficiency: Beyond Anemia  

Microsoft Academic Search

Iron deficiency is the most common nutritional disorder affecting at least one third of world’s population. Though anemia\\u000a is common manifestation of iron deficiency, other effects of iron deficiency on various tissues, organs and systems are usually\\u000a under recognized. Impaired brain development and cognitive, behavioural and psychomotor impairment are most worrisome manifestations\\u000a of iron deficiency. Studies have demonstrated that some

Dinesh Yadav; Jagdish Chandra

2011-01-01

301

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

302

Parvovirus B19 infection reminiscent of myelodysplastic syndrome in three children with chronic hemolytic anemia.  

PubMed

The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus-B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of true clonal MDS. PMID:10989468

Yarali, N; Duru, F; Sipahi, T; Kara, A; Teziç, T

2000-09-01

303

A New Case of Phosphoglycerate Kinase Deficiency: PGK Creteil Associated With Rhabdomyolysis and Lacking Hemolytic Anemia  

Microsoft Academic Search

A new case of phosphoglycerate kinase (PGK) deficiency is described. The propositus displayed episodes of rhabdo- myolysis crises and acute renal failure but did not exhibit any sign of hemolysis. A severe deficiency in phospho- glycerate kinase was revealed in muscle and was also found in erythrocytes, white cells and platelets. A partial defect in the same enzyme was present

Raymonde Rosa; Claude George; Michel Fardeau; Marie-Claude Calvin; Maurice Rapin; Jean Rosa

1982-01-01

304

A Novel Hemotropic Mycoplasma (Hemoplasma) in a Patient With Hemolytic Anemia and Pyrexia  

PubMed Central

A patient with chronic moderate neutropenia, acute hemolysis, and pyrexia was found to be infected with a novel hemoplasma species. A clinical response to doxycyline was noted, and moxifloxacin was added subsequently to aid infection clearance. This represents the first report of hemolysis in association with confirmed hemoplasma infection in a human.

Steer, Jane A.; Barker, Emily N.; Jensen, J?rgen; Mitchell, Joanne; Stocki, Teresa; Chalker, Victoria J.; Hamon, Mike

2011-01-01

305

Rapid capping in alpha-spectrin-deficient MEL cells from mice afflicted with hereditary hemolytic anemia  

PubMed Central

A spectrin-based membrane skeleton is important for the stability and organization of the erythrocyte. To study the role of spectrin in cells that possess complex cytoskeletons, we have generated alpha-spectrin- deficient erythroleukemia cell lines from sph/sph mice. These cells contain beta-spectrin, but lack alpha-spectrin as determined by immunoblot and Northern blot analyses. The effects of alpha-spectrin deficiency are apparent in the cells' irregular shape and fragility in culture. Capping of membrane glycoproteins by fluorescent lectin or antibodies occurs more rapidly in sph/sph than in wild-type erythroleukemia cells, and the caps appear more concentrated. The data support the idea that spectrin plays an important role in organizing membrane structure and limiting the lateral mobility of integral membrane glycoproteins in cells other than mature erythrocytes.

1994-01-01

306

Lethal autoimmune hemolytic anemia in CD47-deficient nonobese diabetic (NOD) mice  

Microsoft Academic Search

The glycoprotein CD47 (integrin-associ- ated protein, IAP) is present on the sur- face of virtually all cells, including red blood cells (RBCs). CD47 acts like a marker of self by ligating the macrophage inhibitory receptor signal regulatory pro- tein (SIRP). In this manner mild reactiv- ity of wild-type RBCs with macrophage phagocytic receptors is tolerated, whereas otherwise identical CD47-deficient RBCs

Per-Arne Oldenborg; Hattie D. Gresham; Yongmei Chen; Shozo Izui; Frederik P. Lindberg

2002-01-01

307

Anemia of chronic disease.  

PubMed

Anemia of chronic disease (ACD) or inflammation may be secondary to infections, autoimmune disorders, chronic renal failure, or malignancies. It is characterized by an immune activation with an increase in inflammatory cytokines and resultant increase in hepcidin levels. In addition, inappropriate erythropoietin levels or hyporesponsiveness to erythropoietin and reduced red blood cell survival contribute to the anemia. Hepcidin being the central regulator of iron metabolism plays a key role in the pathophysiology of ACD. Hepcidin binds to the iron export protein, ferroportin, present on macrophages, hepatocytes, and enterocytes, causing degradation of the latter. This leads to iron trapping within the macrophages and hepatocytes, resulting in functional iron deficiency. Production of hepcidin is in turn regulated by iron stores, inflammation, and erythropoiesis via the BMP-SMAD and JAK-STAT signaling pathways. Treatment of anemia should primarily be directed at the underlying disease, and conventional therapy such as red blood cell transfusions, iron, erythropoietin, and novel agents targeting the hepcidin-ferroportin axis and signaling pathways (BMP-SMAD, JAK-STAT) involved in hepcidin production also may be considered. PMID:23953340

Gangat, Naseema; Wolanskyj, Alexandra P

2013-07-01

308

Anemia in Heart Failure Patients  

PubMed Central

Heart failure is a very common disease, with severe morbidity and mortality, and a frequent reason of hospitalization. Anemia and a concurrent renal impairment are two major risk factors contributing to the severity of the outcome and consist of the cardio renal anemia syndrome. Anemia in heart failure is complex and multifactorial. Hemodilution, absolute or functional iron deficiency, activation of the inflammatory cascade, and impaired erythropoietin production and activity are some pathophysiological mechanisms involved in anemia of the heart failure. Furthermore other concomitant causes of anemia, such as myelodysplastic syndrome and chemotherapy, may worsen the outcome. Based on the pathophysiology of cardiac anemia, there are several therapeutic options that may improve hemoglobin levels, tissues' oxygenation, and probably the outcome. These include administration of iron, erythropoiesis-stimulating agents, and blood transfusions but still the evidence provided for their use remains limited.

Alexandrakis, Michael G.; Tsirakis, George

2012-01-01

309

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

PubMed Central

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

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

2012-01-01

310

Ocular Findings in Aplastic Anemia  

Microsoft Academic Search

Objective: To analyze the ocular findings in aplastic anemia. Design: Eighteen patients with aplastic anemia were examined. Results: Ocular findings included cotton wool spots (38%), nerve fiber layer or preretinal hemorrhages (67%), vitreous hemorrhages (13%), a picture resembling central retinal vein occlusion (13%) and optic disk edema (6%). Preretinal hemorrhages were the presenting sign of aplastic anemia in 2 patients

Ahmad M. Mansour; Haytham I. Salti; Dennis P. Han; Albert Khoury; Scott M. Friedman; Ziad Salem; Khaled Ibrahim; Ali Bazerbachi; Nagi Saghir

2000-01-01

311

Cobalamin deficiency associated with erythroblastic anemia and methylmalonic aciduria in a border collie.  

PubMed

Anemia due to cobalamin deficiency is a rare genetic disorder that has been recognized in dogs only recently. This report concerns a 14-month-old border collie that presented for chronic, nonregenerative anemia. Cytological examination of a peripheral blood smear showed the presence of erythroblasts. Serum cobalamin levels were below reference ranges reported for clinically normal dogs. A methylmalonic aciduria was found on urinalysis. These signs are consistent with the anemia in Imerslund-Graesbeck syndrome reported in humans. Anemia due to cobalamin deficiency responds to parenteral vitamin B12 therapy, and affected animals have a good prognosis for recovery. PMID:10493414

Morgan, L W; McConnell, J

312

21 CFR 866.5490 - Hemopexin immunological test system.  

Code of Federal Regulations, 2010 CFR

...various hematologic disorders, such as hemolytic anemia (anemia due to shortened in vivo survival of mature...for their decreased life span) and sickle cell anemia. (b) Classification. Class II (special controls). The...

2010-04-01

313

21 CFR 866.5490 - Hemopexin immunological test system.  

Code of Federal Regulations, 2010 CFR

...various hematologic disorders, such as hemolytic anemia (anemia due to shortened in vivo survival of mature...for their decreased life span) and sickle cell anemia. (b) Classification. Class II (special controls). The...

2009-04-01

314

Expression of hemolytic activity by Plesiomonas shigelloides.  

PubMed Central

More than 90% of the Plesiomonas shigelloides strains that we tested produced a beta-hemolysin, as judged by the results of agar overlay and contact-dependent hemolysis assays. The hemolysin was cell associated, was active against the erythrocytes of various animal species, and was synthesized at both 25 and 35 degrees C. Activity was lost after thermal or proteolytic treatments or after preincubation in the presence of gentamicin; hemolytic activity did not appear to correlate with the previously established 50% lethal doses for seven of these strains. The hemolysin may play a role in iron acquisition in vivo via the lysis of erythrocytes, liberating hemoglobin, or, alternatively, may be involved in gastrointestinal disease.

Janda, J M; Abbott, S L

1993-01-01

315

Complement disorders and hemolytic uremic syndrome  

PubMed Central

Purpose of review Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. Recent findings In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. Summary Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver–kidney transplantation offer promise for improved prognosis.

Joseph, Catherine; Gattineni, Jyothsna

2013-01-01

316

Clinical grand rounds: atypical hemolytic uremic syndrome.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS. PMID:22517061

Hodgkins, Kavita S; Bobrowski, Amy E; Lane, Jerome C; Langman, Craig B

2012-04-18

317

Congenital dyserythropoietic anemia.  

PubMed

Congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of rare hereditary disorders of erythropoiesis characterized by morphologic abnormal erythroblasts in the bone marrow. Three types of the disease are known as type I, II and III, and the variant type of CDA and several minor subgroups of CDA have been also reported since the first classification. Recently, responsible genes for type I (CDAN1) and type II (SEC23B) have been identified and the molecular pathogenesis of the disease is currently being explored. Although CDAs rarely transform to myelodysplastic syndrome or leukemia, the disease is important to understand the mechanism of hemopoiesis in humans. PMID:20820969

Kamiya, Takahiro; Manabe, Atsushi

2010-09-07

318

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.

Stamper, Debra L.

2000-01-01

319

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

320

CardioRenal Anemia Syndrome  

Microsoft Academic Search

Heart failure (HF) is a systemic disease that also involves organs and tissues other than the heart and the vasculature. Between 25 and 50% of patients with HF are also affected by some degree of kidney disease. Anemia may be present in patients with HF, particularly if the kidney is also affected. Anemia is observed in about 20% of patients

Stephan von Haehling; Stefan D. Anker

2011-01-01

321

Association between hemolysis and albuminuria in adults with sickle cell anemia  

PubMed Central

Studies have questioned whether renal dysfunction in sickle cell disease is linked to hemolysis-associated vasculopathy. We have investigated renal function and markers of hemolysis in a cohort of 424 adult African-British patients with sickle cell disease. While significant associations were found in HbSS and HbS?0 (sickle cell anemia) patients with and without controlling for covariates between hemolytic markers and albuminuria, the associations were not significant in patients with HbSC. Estimated glomerular filtration rate, a marker of renal function, correlated significantly with reticulocyte count and bilirubin. Alpha thalassemia, present in 34% of the sickle cell anaemia patients, had a protective effect against albuminuria in this group. Altogether, the incidence of hyperfiltration was 71% and microalbuminuria 37%, making nephropathy a common complication of sickle cell anemia.

Day, Thomas G.; Drasar, Emma R.; Fulford, Tony; Sharpe, Claire C.; Thein, Swee Lay

2012-01-01

322

nm1054: a spontaneous, recessive, hypochromic, microcytic anemia mutation in the mouse  

PubMed Central

Hypochromic, microcytic anemias are typically the result of inadequate hemoglobin production because of globin defects or iron deficiency. Here, we describe the phenotypic characteristics and pathogenesis of a new recessive, hypochromic, microcytic anemia mouse mutant, nm1054. Although the mutation nm1054 is pleiotropic, also resulting in sparse hair, male infertility, failure to thrive, and hydrocephaly, the anemia is the focus of this study. Hematologic analysis reveals a moderately severe, congenital, hypochromic, microcytic anemia, with an elevated red cell zinc protoporphyrin, consistent with functional erythroid iron deficiency. However, serum and tissue iron analyses show that nm1054 animals are not systemically iron deficient. From hematopoietic stem cell transplantation and iron uptake studies in nm1054 reticulocytes, we provide evidence that the nm1054 anemia is due to an intrinsic hematopoietic defect resulting in inefficient transferrin-dependent iron uptake by erythroid precursors. Linkage studies demonstrate that nm1054 maps to a genetic locus not previously implicated in microcytic anemia or iron phenotypes.

Ohgami, Robert S.; Campagna, Dean R.; Antiochos, Brendan; Wood, Emily B.; Sharp, John J.; Barker, Jane E.; Fleming, Mark D.

2005-01-01

323

[Megaloblastic-vitamin B12 deficiency anemia in childhood].  

PubMed

Megaloblastic anemias are basically caused by vitamin B(12) and/or folic acid deficiency. Childhood vitamin B(12) deficiency is extremely rare. There are congenital and acquired forms of vitamin B(12)-deficiency anemias. The article captures findings of 10 year observation of 3 patients with Imerslund-Gräsbeck Syndrome (congenital chronic megaloblastic anemia with proteinuria), in which the diagnosis was established by us in early childhood and due to correct treatment and prevention complete clinical-laboratory remission is kept so far. We have also observed rare case of acquired megaloblastic anemia - 14 years old vegetarian patient, who was diagnosed with vitamin B(12)-deficiency anemia based on history, clinical and para-clinical data. It was caused by strict vegetarianism of the patient. Therefore first of all the diet was corrected. In 5 days of specific treatment with vitamin B(12) "reticulocyte crisis" was manifested (proving the correctness of diagnosis and treatment) and complete clinical-hematological remission was achieved in 2 weeks. The given cases are interesting as megaloblastic anemias in childhood are both rare and difficult to diagnose. In such cases timely diagnosis, treatment and prevention tactics should be based on cause-and-effect relation of disease. PMID:19556642

Mtvarelidze, Z G; Kvezereli-Kopadze, A N; Kvezereli-Kopadze, M A

2009-05-01

324

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

325

Anemia of aging: the role of chronic inflammation and cancer  

PubMed Central

Aging is associated with increased incidence and prevalence of anemia that is associated with a number of adverse health outcomes. These include death, functional dependence, increased risk of therapeutic complications, falls, and dementia. In approximately 30% of cases, anemia in older individuals is due to either relative or absolute erythropoietin deficiency. Absolute erythropoietin deficiency may be primary or secondary to declining renal function. Relative erythropoietin deficiency is due to an age-related pro-inflammatory status that reduces the sensitivity of erythropoietic precursors to erythropoietin. Despite this condition of erythropoietin deficiency, the management of anemia of aging with erythropoiesis stimulating factors (ESA) is controversial, unless the anemia is due to renal insufficiency. The main concern related to this treatment arises from eight studies of ESAs in cancer, suggesting that ESAs may reduce patient survival, in addition to increasing the risk of deep vein thrombosis. The results of these studies contrast with a host of other trials showing the safety of ESA. The discrepancy may be explained in part by the fact that, in the trials suggesting a detrimental effect of ESAs, the goal was to obtain hemoglobin levels higher than 12g/dl. Because of this concern, correction of anemia in elderly individuals with relative erythropoietin insufficiency should not be attempted outside clinical trials.

Ferrucci, Luigi; Balducci, Lodovico

2009-01-01

326

Anemia and iron deficiency in heart failure.  

PubMed

Anemia is a common comorbidity in heart failure (HF), and is associated with increased morbidity and mortality. However, it remains unclear whether anemia is merely a marker of poor prognosis or whether anemia itself confers risk. The pathogenesis of anemia in HF is multifactorial. Iron deficiency also confers risk in HF, either with or without associated anemia, and treatment of iron deficiency improves the functional status of patients with HF. An ongoing large clinical trial studying the use of darbepoetin-alfa in patients with anemia and systolic HF is expected to provide information that should improve our understanding of anemia in HF. PMID:22940847

Gunawardena, Shanti; Dunlap, Mark E

2012-12-01

327

[Management, prevention and control of pernicious anemia].  

PubMed

Pernicious anemia is the most frequent cause of megaloblastic anemia in our area, and it is the result of a vitamin B12 deficiency due, itself, to the decrease or absence of intrinsic factor (IF) because of gastric mucosa atrophy or autoimmune destruction of IF-producing parietal cells. With the existence of a severe gastric atrophy, there is a decrease in acid and IF production and a further change in vitamin B12 absorption. Fifty percent of the cases are associated to anti-IF antibodies, which presence in other autoimmune diseases is exceptional. In patients with pernicious anemia, measurement of anti-IF antibodies has high specificity (95%); however, measurement of anti-parietal cells antibodies has low specificity. The first-choice treatment is administration of vitamin B12 intramuscularly. The regimen is the administration of 1 mg of vitamin B12 daily for one week, weekly thereafter for one month and, then, every 2-3 months for life. PMID:16335029

De Paz, R; Hernández-Navarro, F

328

Pseudomoyamoya in sickle cell anemia.  

PubMed

Sickle cell (drepanocytic) anemia is a hereditary blood disease occurring very rarely in Mexico. A 13-year-old Mexican boy with sickle cell anemia eventually died of a cerebrovascular accident of the brain stem, as shown by computerized tomography (CT). A characteristic moyamoya-like angiographic pattern was demonstrated on bilateral carotid and left vertebral arteriograms. Moyamoya disease has no known etiology, but the characteristic angiographic features of moyamoya have been observed in conjunction with some other disease of known origin (including sickle cell anemia). It is therefore my belief that these latter cases should be referred to as pseudomoyamoya and not true moyamoya. PMID:7163962

Garza-Mercado, R

1982-12-01

329

How Is Aplastic Anemia Treated?  

MedlinePLUS

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

330

Mouse model for hemolytic uremic syndrome induced by outer membrane vesicles of Escherichia coli O157:H7.  

PubMed

Hemolytic uremic syndrome (HUS) is characterized by acute renal failure in children and is typically complicated with thrombocytopenia and hemolytic anemia. Although mouse models of HUS have been evaluated using Shiga toxin (STx) combined with or without lipopolysaccharide (LPS), no HUS model has been tested using purified outer membrane vesicles (OMVs) from STx-producing Escherichia coli (STEC) O157:H7. Accordingly, we investigated whether OMVs of STEC O157:H7 conveying STx2 and LPS can cause HUS-like symptoms in mice inoculated intraperitoneally. Three types of OMVs differing in LPS acylation status and STx2 amount were used to compare their ability to induce HUS-like symptoms. Native OMVs (nOMV) with fully hexa-acylated LPS caused HUS-like symptoms at 72-96 h after dually divided injections of 1 ?g nOMV per animal. However, elevated doses of modified OMVs (mOMV) carrying mainly penta-acylated LPS were required to induce similar HUS signs. Moreover, mitomycin-C-induced OMVs (mcOMV) that were enriched with STx2 induced HUS-like symptoms similar to those of nOMV at the same dose. These results suggest that the OMVs of STEC O157:H7 potentiated with STx2 and fully hexa-acylated LPS can be utilized for inducing HUS-like symptoms in mice and could be the causative material involved in the development of HUS. PMID:22029600

Kim, Sang-Hyun; Lee, Yong-Hoon; Lee, Sang-Ho; Lee, Sang-Rae; Huh, Jae-Won; Kim, Sun-Uk; Chang, Kyu-Tae

2011-10-04

331

Severe hemolysis due to cloth wear 23 years after aortic valve replacement on a Starr–Edwards ball valve model 2320  

Microsoft Academic Search

Despite iron substitution therapy, a patient developed severe hemolytic anemia 23 yr after insertion of a cloth-covered Starr–Edwards model 2320 aortic valve prosthesis. The prosthesis showed no sign of significant dysfunction. Upon removal, it showed extensive cloth wear on the inner surface of all three struts; one strut was completely denuded of its cloth covering. Hemolysis immediately resolved after replacement

Mikiko Murakami; Hiroyuki Tanaka; Masazumi Watanabe; Masato Shimizu; Makoto Sunamori

2002-01-01

332

Administration of Ricin Induces a Severe Inflammatory Response via Nonredundant Stimulation of ERK, JNK, and P38 MAPK and Provides a Mouse Model of Hemolytic Uremic Syndrome  

PubMed Central

Recent interest in the health consequences of ricin as a weapon of terrorism has led us to investigate the effects of ricin on cells in vitro and in mice. Our previous studies showed that depurination of the 28S rRNA by ricin results in the inhibition of translation and the coordinate activation of the stress-activated protein kinases JNK and p38 MAPK. In RAW 264.7 macrophages, ricin induced the activation of ERK, JNK, and p38 MAPK, the accumulation of mRNA encoding tumor necrosis factor (TNF)-?, interleukin (IL)-1, the transcription factors c-Fos, c-Jun, and EGR1, and the appearance of TNF-? protein in the culture medium. Using specific inhibitors of MAPKs, we demonstrated the nonredundant roles of the individual MAPKs in mediating proinflammatory gene activation in response to ricin. Similarly, the intravenous administration of ricin to mice led to the activation of ERK, JNK, and p38 MAPK in the kidneys, and increases in plasma-borne TNF-?, IL-1?, and IL-6. Ricin-injected mice developed the hallmarks of hemolytic uremic syndrome, including thrombotic microangiopathy, hemolytic anemia, thrombocytopenia, and acute renal failure. Microarray analyses demonstrated a massive proinflammatory transcriptional response in the kidneys, coincidental with the symptoms of hemolytic uremic syndrome. Therapeutic management of the inflammatory response may affect the outcome of intoxication by ricin.

Korcheva, Veselina; Wong, John; Corless, Christopher; Iordanov, Mihail; Magun, Bruce

2005-01-01

333

Treatment of iron deficiency anemia associated with gastrointestinal tract diseases  

Microsoft Academic Search

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 pa- tients' signs and symptoms. While red blood cell trans- fusions may be required in hemodynamically unstable patients, transfusions should be avoided in chronically anemic patients due to their potential side effects and

Ulas D Bayraktar; Soley Bayraktar; Yusuf Bayraktar

2010-01-01

334

Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes.  

PubMed

Evaluation and management of patients with suspected thrombotic thrombocytopenic purpura (TTP) continue to be a critical challenge for hematologists. The diagnostic criteria are not precise, often causing uncertainty about whether it is appropriate to initiate plasma exchange (PEX), the essential treatment for TTP. Initiation of PEX remains a clinical decision; severe ADAMTS13 (< 10% activity) deficiency alone is neither sufficiently sensitive nor specific for the diagnosis of TTP. However, patients who do have severe acquired ADAMTS13 deficiency define the characteristic clinical features of TTP, the response to treatment, and the long-term outcomes. Patients with severe acquired ADAMTS13 deficiency are predominantly young women and the relative frequency of blacks is increased. Patients may present with only microangiopathic hemolytic anemia and thrombocytopenia, neurologic and renal abnormalities are often not present, fever rarely occurs; the complete "pentad" of these clinical features almost never occurs in current practice. Response to PEX is typically rapid but may not be sustained when PEX is stopped. Use of corticosteroids and rituximab has decreased the number of PEX treatments required to achieve a remission and has resulted in fewer PEX-related major complications. Relapse (in approximately 40% of patients) may be the most apparent risk after recovery, but long-term health outcomes are also very important. Minor cognitive abnormalities are common, the frequency of depression is increased, and the frequency of hypertension is increased. Careful long-term follow-up of TTP patients is essential. PMID:23233641

George, James N; Al-Nouri, Zayd L

2012-01-01

335

Peginesatide for the treatment of anemia in the nephrology setting.  

PubMed

Anemia is a major complication in patients with chronic kidney disease, as the damaged kidney is unable to produce enough erythropoietin. Peginesatide (formerly known as Hematide™) is a synthetic, peptide-based erythropoiesis-stimulating agent linked to polyethylene glycol. Based on extensive preclinical and clinical data substantiating the efficacy and safety of this agent, it was approved in the U.S. in March 2012 for the treatment of anemia due to chronic kidney disease in adult patients on dialysis. Peginesatide (Omontys®) was launched in the U.S. in April 2012. PMID:22745925

Graul, A I

2012-06-01

336

Sleep alterations and iron deficiency anemia in infancy  

PubMed Central

Iron-deficiency anemia (IDA) continues to be the most common single nutrient deficiency in the world. An estimated 20-25% of the world’s infants have IDA, with at least as many having iron deficiency without anemia. Infants are at particular risk due to rapid growth and limited dietary sources of iron. We found that infants with IDA showed different motor activity patterning in all sleep-waking states and several differences in sleep states organization. Sleep alterations were still apparent years after correction of anemia with iron treatment in the absence of subsequent IDA. We suggest that altered sleep patterns may represent an underlying mechanism that interferes with optimal brain functioning during sleep and wakefulness in former IDA children.

Peirano, Patricio D.; Algarin, Cecilia R.; Chamorro, Rodrigo A.; Reyes, Sussanne C.; Duran, Samuel A.; Garrido, Marcelo I.; Lozoff, Betsy

2013-01-01

337

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.

Moreno Chulilla, Jose Antonio; Romero Colas, Maria Soledad; Gutierrez Martin, Martin

2009-01-01

338

Peripheral gangrene in children with atypical hemolytic uremic syndrome.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy with severe clinical manifestation, frequent recurrence, and poor long-term prognosis. It is usually caused by abnormalities in complement regulation. We report 2 cases of children affected by a catastrophic extrarenal complication. A 4-year-old Indian girl developed gangrene of the finger tips 2 days after initial presentation of aHUS. Factor H autoantibodies were identified. Renal function continued to decline despite daily plasma exchanges, and she was started on peritoneal dialysis 5 days after admission. The distal tips of the left hand remained gangrenous with a line of demarcation. Three weeks later, she did not return for follow-up and died at home because of dialysis-related complications. An Arabic girl developed end-stage renal disease due to aHUS in the fourth month after birth. A de novo activating C3 mutation was found. At age 9 months, she suddenly developed ischemic changes in fingers of both hands and several toes. The lesions progressed, and several finger tips became gangrenous despite intense plasma exchange therapy. The decision was made to administer complement blocking therapy with the C5 antibody eculizumab. All nonnecrotic digits rapidly regained perfusion. The 3 already gangrenous fingers healed with loss of the end phalanges. During maintenance, eculizumab aHUS activity subsided completely and some late recovery of renal function was observed. aHUS may present by thrombotic macroangiopathy of small peripheral arteries. Eculizumab appears effective in preserving tissue viability if administered before gangrene occurs and should be considered as first-line rescue therapy in such cases. PMID:23230076

Malina, Michal; Gulati, Ashima; Bagga, Arvind; Majid, Mohammad A; Simkova, Eva; Schaefer, Franz

2012-12-10

339

Anemia Linked to Dementia Among Seniors  

MedlinePLUS

... Tools NLM Director’s Comments Transcript Anemia Linked to Dementia Among Seniors : 09/23/2013 To use the ... with anemia may have an increased risk of dementia, suggests a comprehensive study recently published in Neurology . ...

340

Genetics Home Reference: Dyserythropoietic anemia and thrombocytopenia  

MedlinePLUS

... undergo a form of programmed cell death called apoptosis. A lack of immature red blood cells results ... help with understanding dyserythropoietic anemia and thrombocytopenia? anemia ; apoptosis ; blood clotting ; cell ; chromosome ; clotting ; congenital ; differentiation ; DNA ; ...

341

Fanconi Anemia and its Diagnosis  

PubMed Central

Fanconi anemia (FA) is a genetically and phenotypically heterogeneous recessive disorder characterized by diverse congenital malformations, progressive pancytopenia, and predisposition to both hematologic malignancies and solid tumors. Congenital anomalies vary from patient to patient and may affect skeletal morphogenesis as well as any of the major organ systems. Although this highly variable phenotype makes accurate diagnosis on the basis of clinical manifestations difficult in some patients, laboratory study of chromosomal breakage induced by diepoxybutane (DEB) or other crosslinking agents provides a unique cellular marker for the diagnosis of the disorder either prenatally or postnatally. Diagnosis based on abnormal response to DNA crosslinking agents can be used to identify the pre-anemia patient as well as patients with aplastic anemia or leukemia who may or may not have the physical stigmata associated with the syndrome. This overview will present our present knowledge regarding the varied phenotypic manifestations of FA and procedures for diagnosis based upon abnormal DNA damage responses.

Auerbach, Arleen D.

2009-01-01

342

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.

Koshy, Susan M.

2007-01-01

343

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; Ezgü, Fatih S; Okur, Ilyas; Tümer, Leyla; Cakmak, Alpay; Nazarenko, Irina; Yu, Chunli; Clavero, Sonia; Bishop, David F; Desnick, Robert J

2010-11-20

344

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.

Hasanoglu, A; Balwani, M; Kasapkara, CS; Ezgu, FS; Okur, I; Tumer, L; Cakmak, A; Nazarenko, I; Yu, C; Clavero, S; Bishop, DF; Desnick, RJ

2011-01-01

345

Anemia and Heart Failure: A Community Study  

Microsoft Academic Search

PURPOSE: Anemia is an important comorbidity in heart failure and has been associated with increased mortality. The goals of this study were to define the prevalence of anemia in a community population with heart failure, examine trends in prevalence over time, and evaluate the role of anemia in patients with heart failure with preserved and reduced ejection fraction. METHODS: Two

Shannon M. Dunlay; Susan A. Weston; Margaret M. Redfield; Jill M. Killian; Véronique L. Roger

2008-01-01

346

Inborn Anemias of Mice: Terminal Progress Report.  

National Technical Information Service (NTIS)

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 an...

S. E. Bernstein

1987-01-01

347

Feline Nonregenerative Anemia: Diagnosis and Treatment  

Microsoft Academic Search

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

Carrie White; Nyssa Reine

348

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

PubMed

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; Waldorf, Kristina M Adams; Rajagopal, Lakshmi

2013-05-27

349

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.

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

1994-01-01

350

Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome  

PubMed Central

Background and objectives: Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway. Design, setting, participants, & measurements: We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab. Results: Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure. Conclusions: In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.

Acham-Roschitz, Birgit; Fremeaux-Bacchi, Veronique; Kirschfink, Michael; Zipfel, Peter F.; Roedl, Siegfried; Vester, Udo; Ring, Ekkehard

2009-01-01

351

Childhood Hemolytic Uremic Syndrome, United Kingdom and Ireland  

Microsoft Academic Search

We conducted prospective surveillance of childhood hemolytic uremic syndrome (HUS) from 1997 to 2001 to describe disease incidence and clinical, epidemiologic and microbiologic characteristics. We compared our findings, where possible, with those of a previous study conducted from 1985 to 1988. The average annual incidence of HUS for the United Kingdom and Ireland (0.71\\/100,000) was unchanged from 1985 to 1988.

Richard M. Lynn; Sarah J. O'Brien; C. Mark Taylor; Goutam K. Adak; Henrik Chart; Tom Cheasty; John E. Coia; Iain A. Gillespie; Mary E. Locking; William J. Reilly; Henry R. Smith; Aoife Waters; Geraldine A. Willshaw

2005-01-01

352

Green teeth in a premature infant following hemolytic jaundice.  

PubMed

Green staining of the dentition is a phenomenon associated with the deposition of bilirubin in the matrix of hard tissue during formation. This article presents a case of green teeth in a patient born 28 weeks premature with a medical history of hemolytic jaundice and grade IV intraventricular hemorrhage at birth. PMID:23823340

Rammal, M; Meador, M; Rodriguez, M; Lish, B

2013-07-01

353

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

SciTech Connect

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

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

1987-06-01

354

Characterization of the hemolytic activity of Haemophilus ducreyi.  

PubMed Central

H. ducreyi is the causative agent of chancroid, a genital ulcer disease most prevalent in developing countries. Chancroid enhances the heterosexual transmission of human immunodeficiency virus and is identified in focal outbreaks in the United States, but little is known about its pathogenesis. We studied the hemolysin produced by H. ducreyi because this molecule might be an important virulence factor in the pathogenesis of chancroid. Ten strains of H. ducreyi were tested on newly devised blood agar plates and were found to have hemolytic activity. We examined the hemolytic activity of H. ducreyi 35000 further and found that it was heat labile, cell associated, greatest at pH 7.0, and produced in logarithmic- but not stationary-phase cultures. Using transposons Tn916 and Tn1545-delta 3, we have isolated three classes of transposon mutants of strain 35000: those with no detectable hemolytic activity, those with reduced hemolytic activity, and those with enhanced hemolytic activity. Transposon insertions in the nonhemolytic mutants were located in a DNA sequence which hybridized to the Proteus mirabilis hemolysin gene. Analysis of clones containing overlapping sections of this region served to further localize the H. ducreyi hemolysin gene and allow its expression in Escherichia coli and complementation of the nonhemolytic defect in an H. ducreyi mutant. These experiments indicate that H. ducreyi 35000 produces a hemolysin that is related to the calcium-independent hemolysin produced by P. mirabilis. Further experiments are needed to define the similarity of the H. ducreyi hemolysin to other calcium-independent hemolysins and to determine its role in the pathogenesis of chancroid.

Totten, P A; Norn, D V; Stamm, W E

1995-01-01

355

Cardio-renal anemia syndrome.  

PubMed

Heart failure (HF) is a systemic disease that also involves organs and tissues other than the heart and the vasculature. Between 25 and 50% of patients with HF are also affected by some degree of kidney disease. Anemia may be present in patients with HF, particularly if the kidney is also affected. Anemia is observed in about 20% of patients with ambulatory HF, but its prevalence may increase to 60% or more in patients with advanced disease or significant co-morbidities. Cardio-renal anemia syndrome (CRAS) represents a pathological triangle in which the primary failing organ is either the heart or the kidney, and the dysfunction of one leads to dysfunction of the other. Mortality rates increase with only anemia or kidney disease being present in patients with HF. The full clinical picture of CRAS is present in about 20% of all patients hospitalized for HF. In such patients, a steep increase in mortality rates has been observed. This article describes the suggested classification systems of CRAS, its clinical significance, and potential therapeutic avenues. PMID:21625123

von Haehling, Stephan; Anker, Stefan D

2011-05-23

356

Erythema nodosum and pernicious anemia.  

PubMed

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

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

2013-07-14

357

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.

358

Radiosensitivity in Fanconi's anemia patients  

Microsoft Academic Search

The risks of radiation therapy in patients with Fanconi's anemia who have cancer are not clear. Possible toxicity was reported in six of 14 patients: 1\\/1 with vaginal cancer, 4\\/10 with head and neck or esophageal cancer, and 1\\/3 with oral cancer following bone marrow transplant.

Blanche P Alter

2002-01-01

359

Aplastic Anemia: Pathogenesis and Treatment  

Microsoft Academic Search

This review highlights some of the contributions that have appeared in the literature in the past decade on the pathogenesis and treatment of aplastic anemia (AA). This summary is brief because the field is vast, spaning from stem cell biology to stem cell disorders, from autoimmunity to transplantation, from graft- versus-host disease to late effects. The immune pathogenesis of AA

Andrea Bacigalupo; Grover C. Bagby; Blanche P. Alter

2007-01-01

360

Deafferentation anemia in splenectomized animals  

Microsoft Academic Search

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

M. I. Pekarskii

1970-01-01

361

Association of mild anemia with hospitalization and mortality in the elderly: the Health and Anemia population-based study  

PubMed Central

Background Mild anemia is a frequent laboratory finding in the elderly usually disregarded in everyday practice as an innocent bystander. The aim of the present population-based study was to prospectively investigate the association of mild grade anemia with hospitalization and mortality. Design and Methods A prospective population-based study of all 65 to 84 year old residents in Biella, Italy was performed between 2003 and 2007. Data from a total of 7,536 elderly with blood tests were available to estimate mortality; full health information available to evaluate health-related outcomes was available for 4,501 of these elderly subjects. Mild grade anemia was defined as a hemoglobin concentration between 10.0 and 11.9 g/dL in women and between 10.0 and 12.9 g/dL in men. Results The risk of hospitalization in the 3 years following recruitment was higher among the mildly anemic elderly subjects than among subjects who were not anemic (adjusted hazard ratio: 1.32; 95% confidence interval: 1.09–1.60). Mortality risk in the following 3.5 years was also higher among the mildly anemic elderly (adjusted hazard ratio: 1.86; 95% confidence interval: 1.34–2.53). Similar results were found when slightly elevating the lower limit of normal hemoglobin concentration to 12.2 g/dL in women and to 13.2 g/dL in men. The risk of mortality was significantly increased in mild anemia of chronic disease but not in that due to ?-thalassemia minor. Conclusions After controlling for many potential confounders, mild grade anemia was found to be prospectively associated with clinically relevant outcomes such as increased risk of hospitalization and all-cause mortality. Whether raising hemoglobin concentrations can reduce the risks associated with mild anemia should be tested in controlled clinical trials.

Riva, Emma; Tettamanti, Mauro; Mosconi, Paola; Apolone, Giovanni; Gandini, Francesca; Nobili, Alessandro; Tallone, Maria Vittoria; Detoma, Paolo; Giacomin, Adriano; Clerico, Mario; Tempia, Patrizia; Guala, Adriano; Fasolo, Gilberto; Lucca, Ugo

2009-01-01

362

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.

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

2013-01-01

363

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

364

Epidemiology of Anemia in Older Adults  

PubMed Central

Anemia is a common, multifactorial condition among older adults. The World Health Organization (WHO) definition of anemia (hemoglobin concentration <12 g/dL in women and <13 g/dL in men) is most often used in epidemiologic studies of older adults. More than 10% of community-dwelling adults age 65 years and older has WHO-defined anemia. After age 50 years, prevalence of anemia increases with advancing age and exceeds 20% in those 85 years and older. In nursing homes, anemia is present in 48–63% of residents. Incidence of anemia in older adults is not well characterized. Among older adults with anemia, approximately one-third have evidence of iron, folate, and/or vitamin B12 deficiency, another third have renal insufficiency and/or chronic inflammation, and the remaining third have anemia that is unexplained. Several studies demonstrate that anemia is associated with poorer survival in older adults. This review details the distribution and consequences of anemia in older adults and identifies future epidemiologic research needs.

Patel, Kushang V.

2008-01-01

365

Clinical and genetic characteristics of congenital sideroblastic anemia: comparison with myelodysplastic syndrome with ring sideroblast (MDS-RS).  

PubMed

Sideroblastic anemia is characterized by anemia with the emergence of ring sideroblasts in the bone marrow. There are two forms of sideroblastic anemia, i.e., congenital sideroblastic anemia (CSA) and acquired sideroblastic anemia. In order to clarify the pathophysiology of sideroblastic anemia, a nationwide survey consisting of clinical and molecular genetic analysis was performed in Japan. As of January 31, 2012, data of 137 cases of sideroblastic anemia, including 72 cases of myelodysplastic syndrome (MDS)-refractory cytopenia with multilineage dysplasia (RCMD), 47 cases of MDS-refractory anemia with ring sideroblasts (RARS), and 18 cases of CSA, have been collected. Hemoglobin and MCV level in CSA are significantly lower than those of MDS, whereas serum iron level in CSA is significantly higher than those of MDS. Of 14 CSA for which DNA was available for genetic analysis, 10 cases were diagnosed as X-linked sideroblastic anemia due to ALAS2 gene mutation. The mutation of SF3B1 gene, which was frequently mutated in MDS-RS, was not detected in CSA patients. Together with the difference of clinical data, it is suggested that genetic background, which is responsible for the development of CSA, is different from that of MDS-RS. PMID:22983749

Ohba, Rie; Furuyama, Kazumichi; Yoshida, Kenichi; Fujiwara, Tohru; Fukuhara, Noriko; Onishi, Yasushi; Manabe, Atsushi; Ito, Etsuro; Ozawa, Keiya; Kojima, Seiji; Ogawa, Seishi; Harigae, Hideo

2012-09-16

366

Anemia of Inflammation Is Related to Cognitive Impairment among Children in Leyte, The Philippines  

Microsoft Academic Search

BackgroundMany studies have addressed the relationship between iron deficiency anemia (IDA) and cognitive impairment, but none have evaluated the role of non-iron deficiency anemia (NIDA). One of the main causes of NIDA in developing countries is AI, largely due to infectious diseases, whereby iron is shunted away from bio-available forms to storage forms, making it less accessible for use by

Courtney L. Olson; Luz P. Acosta; Natasha S. Hochberg; Remigio M. Olveda; Mario Jiz; Stephen T. McGarvey; Jonathan D. Kurtis; David C. Bellinger; Jennifer F. Friedman

2009-01-01

367

Risk factors for development of hemolytic uremic syndrome in a cohort of adult patients with STEC 0104:H4 infection.  

PubMed

The outbreak of Shiga toxin producing E.coli O104:H4 in northern Germany in 2011 was one of the largest worldwide and involved mainly adults. Post-diarrheal hemolytic uremic syndrome (HUS) occurred in 22% of STEC positive patients. This study's aim was to assess risk factors for HUS in STEC-infected patients and to develop a score from routine hospital parameters to estimate patient risks for developing HUS. In a cohort analysis, adult patients with STEC infection were included in five participating hospitals in northern Germany between May and July 2011. Clinical data were obtained from questionnaires and medical records, laboratory data were extracted from hospitals' electronic data systems. HUS was defined as thrombocytopenia, hemolytic anemia and acute renal dysfunction. Random forests and multivariate logistic regression were used to identify risk factors for HUS and develop a score using the estimated coefficients as weights. Among 259 adults with STEC infection, vomiting (OR 3.48,95%CI 1.88-6.53), visible blood in stools (OR 3.91,95%CI1.20-16.01), age above 75 years (OR 3.27, 95%CI 1.12-9.70) and elevated leukocyte counts (OR 1.20, 95%CI 1.10-1.31, per 1000 cells/mm(3)) were identified as independent risk factors for HUS. A score using these variables has an area under the ROC curve of 0.74 (95%CI 0.68-0.80). Vomiting, visible blood in stools, higher leukocyte counts, and higher age indicate increased risk for developing HUS. A score using these variables might help to identify high risk patients who potentially benefit from aggressive pre-emptive treatment to prevent or mitigate the devastating consequences of HUS. PMID:23533606

Zoufaly, Alexander; Cramer, Jakob P; Vettorazzi, Eik; Sayk, Friedhelm; Bremer, Jan P; Koop, Irmtraut; de Weerth, Andreas; Schmiedel, Stefan; Jordan, Sabine; Fraedrich, Katharina; Asselborn, Niels H; Nitschke, Martin; Neumann-Grutzeck, Christine; Magnus, Tim; Rüther, Christoph; Fellermann, Klaus; Stahl, Rolf K; Wegscheider, Karl; Lohse, Ansgar W

2013-03-22

368

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

PubMed

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

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

2007-01-01

369

Pulmonary Hemorrhage Complicating a Typical Hemolytic-Uremic Syndrome  

Microsoft Academic Search

We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All

M. Piastra; A. Ruggiero; A. Langer; E. Caresta; A. Chiaretti; S. Pulitanò; G. Polidori; R. Riccardi

2004-01-01

370

Reversible MR Findings of Hemolytic Uremic Syndrome with Mild Encephalopathy  

Microsoft Academic Search

Summary: We report the reversible MR findings in a 7-year-old girl with hemolytic uremic syndrome and mild encephalopathy. The splenium of the corpus callosum showed isointense to low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, representing local edema. These findings returned to near normal on MR images obtained 1 week later. The patient recovered without

Hiroshi Ogura; Makoto Takaoka; Masashi Kishi; Masahide Kimoto; Takeshi Shimazu; Toshiharu Yoshioka; Hisashi Sugimoto

371

Atypical relapse of hemolytic uremic syndrome after transplantation  

Microsoft Academic Search

Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of immediate recurrent HUS. Prophylactic plasma exchange before and after transplantation was used. Two months after transplantation, concomitant with

Karolien H. Olie; Sandrine Florquin; Jaap W. Groothoff; René Verlaak; Lisa Strain; Timothy H. J. Goodship; Jan J. Weening; Jean-Claude Davin

2004-01-01

372

Autosomal dominant hemolytic uremic syndrome: variable phenotypes and transplant results  

Microsoft Academic Search

Autosomal dominant hemolytic uremic syndrome (ADHUS) is a rare disorder with a poor prognosis that was considered to present\\u000a mainly in adults. Recurrent episodes of ADHUS were also thought to be uncommon. However, increasing reports suggest that children\\u000a are often affected, that recurrent episodes may occur pre-transplantation, and that post-transplant recurrences occur in about\\u000a 50% of cases. We describe the

B. S. Kaplan; M. B. Leonard

2000-01-01

373

Peripheral gangrene complicating idiopathic and recessive hemolytic uremic syndromes  

Microsoft Academic Search

Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred\\u000a in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the\\u000a second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more\\u000a than 1

Bernard S. Kaplan; Clotilde D. Garcia; Russell W. Chesney; William E. Segar; Katia Giugno; Roberto Chem

2000-01-01

374

Low serum selenium is associated with anemia among older adults in the United States  

PubMed Central

Objective We hypothesized that low serum selenium was associated with anemia in humans. Subjects A total of 2092 adults aged 65 and older, in the third National Nutrition Examination Survey, Phase 2 (1991–1994) (NHANES III). Methods Examination of the relationship between serum selenium and hematological indices in NHANES III. Results Anemia, defined by World Health Organization criteria, was present in 12.9%. Mean serum selenium among non-anemic and anemic adults was 1.60 and 1.51 ?mol l?1 (P=0.0003). The prevalence of anemia among adults in the lowest to highest quartiles of serum selenium was 18.3, 9.5, 9.7 and 6.9%, respectively (P=0.0005). The proportion of adults in the lowest quartile of selenium among those who were non-anemic or who had anemia due to nutritional causes, chronic inflammation, renal disease or unexplained anemia was 9.9, 27.5, 17.5, 24.0 and 15.4%, respectively. An increase in loge selenium was associated with a reduced risk of anemia (odds ratio per one standard deviation increase 0.75, 95% confidence interval 0.58–0.97, P=0.03), adjusting for age, race, education, body mass index and chronic diseases. Conclusion Low serum selenium is independently associated with anemia among older men and women in the United States.

Semba, RD; Ricks, MO; Ferrucci, L; Xue, Q-L; Guralnik, JM; Fried, LP

2009-01-01

375

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.

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

1995-01-01

376

Serratamolide is a Hemolytic Factor Produced by Serratia marcescens  

PubMed Central

Serratia marcescens is a common contaminant of contact lens cases and lenses. Hemolytic factors of S. marcescens contribute to the virulence of this opportunistic bacterial pathogen. We took advantage of an observed hyper-hemolytic phenotype of crp mutants to investigate mechanisms of hemolysis. A genetic screen revealed that swrW is necessary for the hyper-hemolysis phenotype of crp mutants. The swrW gene is required for biosynthesis of the biosurfactant serratamolide, previously shown to be a broad-spectrum antibiotic and to contribute to swarming motility. Multicopy expression of swrW or mutation of the hexS transcription factor gene, a known inhibitor of swrW expression, led to an increase in hemolysis. Surfactant zones and expression from an swrW-transcriptional reporter were elevated in a crp mutant compared to the wild type. Purified serratamolide was hemolytic to sheep and murine red blood cells and cytotoxic to human airway and corneal limbal epithelial cells in vitro. The swrW gene was found in the majority of contact lens isolates tested. Genetic and biochemical analysis implicate the biosurfactant serratamolide as a hemolysin. This novel hemolysin may contribute to irritation and infections associated with contact lens use.

Shanks, Robert M. Q.; Stella, Nicholas A.; Lahr, Roni M.; Wang, Shaoru; Veverka, Tara I.; Kowalski, Regis P.; Liu, Xinyu

2012-01-01

377

A simple microassay for computing the hemolytic potency of drugs.  

PubMed

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

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

1994-12-01

378

Individualization of pharmacological anemia management using reinforcement learning.  

PubMed

Effective management of anemia due to renal failure poses many challenges to physicians. Individual response to treatment varies across patient populations and, due to the prolonged character of the therapy, changes over time. In this work, a Reinforcement Learning-based approach is proposed as an alternative method for individualization of drug administration in the treatment of renal anemia. Q-learning, an off-policy approximate dynamic programming method, is applied to determine the proper dosing strategy in real time. Simulations compare the proposed methodology with the currently used dosing protocol. Presented results illustrate the ability of the proposed method to achieve the therapeutic goal for individuals with different response characteristics and its potential to become an alternative to currently used techniques. PMID:16109475

Gaweda, Adam E; Muezzinoglu, Mehmet K; Aronoff, George R; Jacobs, Alfred A; Zurada, Jacek M; Brier, Michael E

379

Light and scanning electron microscopic studies on chromium-induced anemia in a murine model.  

PubMed

Blood hemoglobin level, hematocrit value and erythrocyte count were reduced by 17.5, 17.4 and 15.9%, respectively, as compared to the controls, in Swiss mice treated intraperitoneally with hexavalent chromium (4 mg of potassium dichromate per Kg for 5 day per week) for 2 weeks. Echinocytic transformation of 33.8% erythrocytes, as revealed by both light and scanning electron microscopy, indicated the anemia to be hemolytic in nature. Leucopenia was apparent after 2 weeks (mean leucocyte count: 4.91 thousand c mm(-1)), but not 1 week of treatment (mean count: 6.43 thousand c mm(-1)), However, cytochemical studies indicated that chromium did not interfere with iron utilization for hemoglobin synthesis and also, did not cause denaturation of already synthesized hemoglobin. The study hints to the necessity of periodic monitoring of blood in workers of chromium-dependent tanneries of Kolkata, India. PMID:22080295

Ray, Rina Rani; Sarkar, Nirmal Kumar

2011-11-12

380

Anemia in rheumatoid arthritis: high prevalence of iron-deficiency anemia in Indian patients  

Microsoft Academic Search

Anemia in rheumatoid arthritis (RA) is multi-factorial. We studied the prevalence and type of anemia and its correlation with disease variables in RA patients. Among patients with RA anemia was defined as hemoglobin ? 11 g\\/dl in females and ? 12 g\\/dl in males. Iron-deficiency anemia (IDA) was defined as serum ferritin ? 50 ?g\\/dl. Disease activity was assessed by modified Disease Activity Score (DAS28). Of the 214 patients

Sumeet Agrawal; Ramnath Misra; Amita Aggarwal

2006-01-01

381

Coexistence of Megaloblastic Anemia and Iron Deficiency Anemia in a Young Woman with Chronic Lymphocytic Thyroiditis  

Microsoft Academic Search

Pernicious anemia is a megaloblastic anemia caused by vitamin B12 deficiency, and is the end-stage of autoimmune gastritis\\u000a that typically affects persons older than 60 years. It is the most common cause of vitamin B12 deficiency. Pernicious anemia\\u000a can also be diagnosed concurrently with other autoimmune diseases. We report the occurrence of megaloblastic anemia in a 22-year-old\\u000a woman with chronic

Shih-Hsiang Chen; Chia-Sui Hung; Chao-Ping Yang; Fu-Sung Lo; Hsun-Hui Hsu

2006-01-01

382

The usefulness of soluble transferrin receptor (sTfR) in differentiating anemia occurring in young children.  

PubMed

We evaluated the usefulness of soluble transferrin receptor (sTfR) and of the sTfR/log ferritin index(sTfR/logF) in the differentiation of anemia in young children. 96 children, aged 6-36 months, were examined.From these, four groups were distinguished: 1 - (IDA): 33 children with anemia due to iron deficiency; 2 -(IA): 19 children with infectious anemia without iron deficiency; 3 - (IA + ID): 16 children with infectious anemia and iron deficiency; and 4 - a comparator group (CG): 28 healthy children without iron deficiency. The soluble transferrin receptor, hematological indices and iron balance were evaluated and the sTfR/logF was calculated for each examined child. It was proved that the mean values of sTfR and sTfR/logF were substantially higher in children with anemia due to iron deficiency, and in those with infectious anemia and iron deficiency,vs. those with infectious anemia or in healthy children. This suggests that both sTfR and the sTfR/logF are good indicators of iron deficiency and could be useful in the differential diagnostics of anemia, especially in young children. PMID:23042282

Kamer, Barbara; Dó?ka, El?bieta; Pasowska, Renata; ?wi?tkowska, Ewa

2012-10-08

383

Child with aplastic anemia: Anesthetic management  

PubMed Central

Aplastic anemia is a rare heterogeneous disorder of hematopoietic stem cells causing pancytopenia and marrow hypoplasia with the depletion of all types of blood cells. This results in anemia, neutropenia and thrombocytopenia, which pose a challenge to both surgical and anesthetic management of such cases. We report a child with aplastic anemia who sustained traumatic ulcer on the arm and underwent split-thickness skin grafting under general anesthesia. There are only two case reports on anesthetic considerations in aplastic anemia patients in the literature. The anesthetic management is challenging because of the rarity of the disease, associated pancytopenia and immunosuppression.

Kaur, Manpreet; Gupta, Babita; Sharma, Aanchal; Sharma, Sanjeev

2012-01-01

384

Anemia increases the risk of renal cortical and medullary hypoxia during cardiopulmonary bypass.  

PubMed

INTRODUCTION: Anemia is an independent predictor of acute kidney injury (AKI) following cardiopulmonary bypass (CPB), possibly due to inadequate renal oxygen delivery. The objective of this study was to investigate the effects of CPB and anemia on tissue oxygen tension (pO2) and blood flow in the renal cortex and medulla. METHODS: Rats (n=6/group) underwent 1hr of normothermic cardiopulmonary bypass (CPB), with target hemoglobin concentrations (Hb) of 10g/dL (CPB) or 6.5g/dL (anemia-CPB). Renal blood flow (RBF) and tissue PO2 were measured before, during and after 1hr of CPB. To confirm the observed differences in renal cortical and medullary PO2, HIF-1? (ODD) luciferase mice were exposed to 8% O2 (hypoxia) and HIF-1? dependent luminescence was measured in the renal cortex and medulla (n=5). RESULTS: Renal tissue PO2 values decreased initially and returned towards baseline, however, values at the end of CPB. Anemia-CPB resulted in a significant increase in both renal cortical and medullary blood flow, PO2 remained significantly reduced throughout anemia-CPB. Renal medullary HIF-1?-dependent luminescence confirmed a greater degree of hypoxia in the renal medulla.Discussion:During CPB, renal O2 delivery was transiently jeopardized, but recovered after 1hr. Anemia-CPB resulted in a dramatic and sustained reduction in renal cortical and medullary PO2, which suggests an increased risk of renal hypoxic injury with anemia. CONCLUSION: The clear difference in the degree of hypoxia in the renal cortex and medulla may be useful in understanding the progress of medullary hypoxia during CPB with anemia and the potential development of AKI. Further studies should aim at identifying early markers of medullary hypoxia and potential agents that may decrease the work and O2 consumption in the renal medulla to reduce the risk of hypoxic damage during CPB and anemia. PMID:23719516

Darby, Pj; Kim, N; Hare, Gmt; Tsui, A; Wang, Z; Harrington, A; Mazer, Cd

2013-05-29

385

Partial characterization of the hemolytic activity of the nematocyst venom from the jellyfish Cyanea nozakii Kishinouye  

Microsoft Academic Search

Using a recently developed technique to extract jellyfish venom from nematocysts, the present study investigated the hemolytic activity of Cyanea nozakii Kishinouye nematocyst venom on chicken erythrocytes. Venom extract caused a significant concentration-dependent hemolytic effect. The extract could retain its activity at ?80°C but was unstable when kept at 4°C and ?20°C for 2 days. The hemolytic activity was inhibited

Jinhua Feng; Huahua Yu; Ronge Xing; Song Liu; Lin Wang; Shengbao Cai; Pengcheng Li

2010-01-01

386

Factors influencing hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye  

Microsoft Academic Search

In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU50 of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40?g\\/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and

Huahua Yu; Cuiping Li; Ronggui Li; Ronge Xing; Song Liu; Pengcheng Li

2007-01-01

387

Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome  

PubMed Central

The hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Genetic studies demonstrate that heterozygous mutations of membrane cofactor protein (MCP;CD46) predispose to atypical HUS (aHUS), which is not associated with exposure to Shiga toxin (Stx). Among the initial 25 MCP mutations in patients with aHUS were 2, R69W and A304V, that were expressed normally and for which no dysfunction was found. The R69W mutation is in complement control protein module 2, while A304V is in the hydrophobic transmembrane domain. In addition to 3 patients with aHUS, the A304V mutation was identified in 1 patient each with fatal Stx-HUS, the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome, and glomerulonephritis with C3 deposits. A major goal was to assess if these putative mutations lead to defective complement regulation. Permanent cell lines expressing the mutated proteins were complement “challenged,” and membrane control of C3 fragment deposition was monitored. Both the R69W and A304V MCP mutations were deficient in their ability to control the alternative pathway of complement activation on a cell surface, illustrating the importance of modeling transmembrane proteins in situ.

Fang, Celia J.; Fremeaux-Bacchi, Veronique; Liszewski, M. Kathryn; Pianetti, Gaia; Noris, Marina; Goodship, Timothy H. J.

2008-01-01

388

Tailored eculizumab therapy in the management of complement factor H-mediated atypical hemolytic uremic syndrome in an adult kidney transplant recipient: a case report.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury (AKI) which frequently progresses to end-stage renal disease (ESRD). In 50% of affected patients, mutations in complement regulatory proteins cause inappropriate complement activation with endothelial injury. Complement factor H (CFH) mutations cause 25% of aHUS cases; these patients have an 80% recurrence risk after kidney transplantation. Eculizumab, an anti-C5 antibody, is effective in limiting hemolysis episodes in patients with aHUS, but less is known about preventing recurrence after kidney transplantation. Herein we report the use of prophylactic eculizumab in an adult with aHUS who underwent kidney transplantation. A 31-year-old female presented with aHUS and progressive AKI associated with low complement 3 level leading to ESRD despite plasmapheresis and corticosteroids. She had a heterozygous nonsense mutation in CFH and reduced plasma CFH levels. She was given preoperative plasmapheresis and eculizumab and underwent living unrelated renal transplantation. Postoperatively, eculizumab was dosed to achieve low functional complement 5 levels and low soluble membrane attack complex levels and she has maintained excellent graft function without aHUS recurrence. We propose that eculizumab with titrated dosing should be used in CFH-mediated aHUS patients who are at a high risk of recurrence. PMID:23195022

Xie, L; Nester, C M; Reed, A I; Zhang, Y; Smith, R J; Thomas, C P

2012-12-01

389

Amiodarone-induced hypothyroidism with EPO-resistant anemia in a patient with chronic renal failure.  

PubMed

The overall incidence of amiodarone-induced thyroid dysfunction ranges from 2% to 24%. One third to half of patients with hypothyroidism have anemia due to some decrease in normal red blood cell mass and erythropoietin (EPO) resistance. Therefore, for patients with chronic renal disease under medication with amiodarone, early regular thyroid function test should be checked in order to avoid amiodarone-induced hypothyroidism and EPO-resistant anemia. If amiodarone-induced hypothyroidism and EPO-resistant anemia occur in patients with chronic renal failure, early thyroxine should be given instead of waiting for spontaneous recovery by amiodarone discontinuation only. Here, we report a patient with chronic renal failure who developed EPO-resistant anemia after amiodarone treatment for arrhythmia. The hemoglobin level responded to EPO therapy rapidly after thyroxine administration and amiodarone discontinuation. PMID:19015056

Chang, Peter M S; Ng, Yee-Yung

2008-11-01

390

[Regional differences in prevalence of anemia found by periodic health checkups at workplaces in Japan].  

PubMed

Anemia-related blood examinations are included in examinations for periodic health checkups at workplaces designated by the Industrial Safety and Health Law in Japan. The aim of this study was to determine whether there were regional differences in the prevalence of anemia in workers and, if so, to investigate possible reasons for the differences. Relationships between prevalence of anemia found by periodic health checkups and some common factors related to anemia in each prefecture of Japan were investigated by ecological regression analysis using Spearman's rank correlation coefficient. There were regional differences in the prevalence of anemia in the prefectures of Japan (5.2-11.7%), and high prevalence was observed in prefectures in the northeastern district, such as Iwate, Akita and Yamagata Prefectures, and in Fukui, Shimane and Nagasaki Prefectures. Prevalence of anemia in each prefecture was significantly correlated with the prevalence of hypertension, dyslipidemia, liver dysfunction, abnormality in ECG, hyperglycemia or glucosuria at health checkups in each prefecture. Prevalence of anemia in each prefecture was significantly correlated with the percentage of patients receiving therapy for anemia in each prefecture but not with the prevalence of myoma uteri, endometriosis uteri or mortality of uterus cancer in each prefecture. There was also no significant correlation of the prevalence of anemia with the prevalence of iron-deficiency anemia or dietary iron intake in each prefecture. The prevalence of anemia in each prefecture showed significant positive correlations with the ratio of female population to total population and the ratio of female workers to total workers in each prefecture; it also showed a significant negative correlation with the ratio of the number of large-sized workplaces (300 or more workers) to the number of workplaces with 50 or more workers in each prefecture. A considerable regional difference in the prevalence of anemia was found by periodic health checkups at workplaces, and we consider that this difference is not due to regional differences in the incidence of diseases causing genital bleeding in women but to regional differences in the ratio of female workers to total workers and the status of health control at the workplace, which depends on size of the workplace. PMID:19942817

Shimomura, Tomoko; Wakabayashi, Ichiro

2009-11-27

391

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

Microsoft Academic Search

Manganese superoxide dismutase 2 (SOD2) is a critical component of the mitochondrial path- way for detoxification of O 2 2 , and targeted disruption of this locus leads to embryonic or neo- natal 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

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

392

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

PubMed

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

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

2013-06-03

393

Diverse point mutations in the human glucose-6-phosphate dehydrogenase gene cause enzyme deficiency and mild or severe hemolytic anemia  

SciTech Connect

Glucose-6-phosphate dehydrogenase deficiency is a common genetic abnormality affecting an estimated 400 million people worldwide. Clinical and biochemical analyses have identified many variants exhibiting a range of phenotypes, which have been well characterized from the hematological point of view. However, until now, their precise molecular basis has remained unknown. The authors have cloned and sequenced seven mutant G6PD alleles. In the nondeficient polymorphic African variant G6PD A they have found a single point mutation. The other six mutants investigated were all associated with enzyme deficiency. The mutations observed show a striking predominance of C {yields} T transitions, with CG doublets involved in four of seven cases. Thus, diverse point mutations may account largely for the phenotypic heterogeneity of G6PD deficiency.

Vulliamy, T.J.; D'Urso, M.; Battistuzzi, G.; Estrada, M.; Foulkes, N.S.; Martini, G.; Calabro, V.; Poggi, V.; Giordano, R.; Town, M.; Luzzatto, L.; Persico, M.G. (Royal Postgraduate Medical School, London (England))

1988-07-01

394

Membrane-localized pyruvate kinase of red blood cells in hemolytic anemia associated with pyruvate kinase deficiency  

Microsoft Academic Search

Zusammenfassung Die Pyruvatkinaseaktivität von Erythrozytenmembranen, die normalerweise maskiert ist, wurde nach mechanischer Zerstörung der Membranen bei normalen Personen und bei drei homozygoten Patienten mit Pyruvatkinasemangel bestimmt. Obgleich die Patienten 1 und 2, die Geschwister sind, relativ hohe Enzymaktivitäten in ihren Hämolysaten haben, litten sie unter der schwersten Form der Erkrankung. Die Enzymaktivitäten ihrer Membranfragmente waren auf 7% der Fragmente normaler

W. Schröter; W. Tillmann

1975-01-01

395

Alteraciones dermatológicas en pacientes con anemias carenciales  

Microsoft Academic Search

Objectives: To determine the frequency of anemia-related dermatological abnormalities and severity. Materials and Methods: A sample of 100 subjects older than 18 years with a diagnosis of nutritional anemia who were admitted at 2 de Mayo Hospital between January and November, 2004 was studied. In each subject we measured total CBC and serum iron, ferritin, folate, and cobalamin levels. Results:

Oscar Ruiz; Luz Bardales; David Díaz; Carlos Galarza; Carlos Delgado; Oscar Castillo; Manuela Marangoni; Carlos Montenegro

2006-01-01

396

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

397

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

398

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

399

Anemia Management in Oncology and Hematology  

Microsoft Academic Search

Anemia is frequent in cancer patients and its incidence increases with chemotherapy. The probability of re- quiring transfusions also increases with chemotherapy. Anemia negatively impacts survival and accentuates fatigue in cancer patients. Cancer promotes inflam- matory cytokine production, which suppresses eryth- ropoiesis and erythropoietin (EPO) production. Erythropoiesis-stimulating agents (ESAs) improve erythropoiesis and reduce transfusion needs in anemic cancer patients receiving

JERRY L. SPIVAK

400

9 CFR 311.34 - Anemia.  

Code of Federal Regulations, 2010 CFR

...Animal Products 2 2009-01-01 2009-01-01 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...

2009-01-01

401

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

402

Anemia during pregnancy in a Chinese population  

Microsoft Academic Search

Objectives: To study prevalence, risk factors, and birth outcomes of women with anemia during pregnancy in a Chinese population. Methods: A retrospective cohort study was performed based on 16936 pregnancies delivered between January 1989 and December 1990 in Suzhou, China. Anemia was defined as hemoglobin less than 10 g\\/dl. Multivariate logistic regression was used to estimate odds ratio, 95% confidence

X. Xiong; P. Buekens; W. D. Fraser; Z. Guo

2003-01-01

403

Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis  

PubMed Central

X-linked sideroblastic anemia with ataxia (XLSA/A) is a rare syndromic form of inherited sideroblastic anemia associated with spinocerebellar ataxia, and is due to mutations in the mitochondrial ATP-binding cassette transporter Abcb7. Here, we show that Abcb7 is essential for hematopoiesis and formally demonstrate that XLSA/A is due to partial loss of function mutations in Abcb7 that directly or indirectly inhibit heme biosynthesis.

Pondarre, Corinne; Campagna, Dean R.; Antiochos, Brendan; Sikorski, Lindsay; Mulhern, Howard

2007-01-01

404

Enzymatic and hemolytic properties of Propionibacterium acnes and related bacteria.  

PubMed Central

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

Hoeffler, U

1977-01-01

405

Hypothesis: Hemolytic Transfusion Reactions Represent an Alternative Type of Anaphylaxis  

PubMed Central

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

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

2009-01-01

406

Strategies for Surveillance of Pediatric Hemolytic Uremic Syndrome: Foodborne Diseases Active Surveillance Network (FoodNet), 2000-2007  

PubMed Central

Background.?Postdiarrheal hemolytic uremic syndrome (HUS) is the most common cause of acute kidney failure among US children. The Foodborne Diseases Active Surveillance Network (FoodNet) conducts population-based surveillance of pediatric HUS to measure the incidence of disease and to validate surveillance trends in associated Shiga toxin–producing Escherichia coli (STEC) O157 infection. Methods.?We report the incidence of pediatric HUS, which is defined as HUS in children <18 years. We compare the results from provider-based surveillance and hospital discharge data review and examine the impact of different case definitions on the findings of the surveillance system. Results.?During 2000–2007, 627 pediatric HUS cases were reported. Fifty-two percent of cases were classified as confirmed (diarrhea, anemia, microangiopathic changes, low platelet count, and acute renal impairment). The average annual crude incidence rate for all reported cases of pediatric HUS was 0.78 per 100 000 children <18 years. Regardless of the case definition used, the year-to-year pattern of incidence appeared similar. More cases were captured by provider-based surveillance (76%) than by hospital discharge data review (68%); only 49% were identified by both methods. Conclusions.?The overall incidence of pediatric HUS was affected by key characteristics of the surveillance system, including the method of ascertainment and the case definitions. However, year-to-year patterns were similar for all methods examined, suggesting that several approaches to HUS surveillance can be used to track trends.

Ong, Kanyin L.; Apostal, Mirasol; Comstock, Nicole; Hurd, Sharon; Webb, Tameka Hayes; Mickelson, Stephanie; Scheftel, Joni; Smith, Glenda; Shiferaw, Beletshachew; Boothe, Effie

2012-01-01

407

Anemia of acute infection in hospitalized children-no evidence of hemolysis.  

PubMed

The objective of the study is to examine the assumption that a process of hemolysis plays a role in anemia of acute infection in children. The study was comprised of febrile pediatric patients, who had a positive blood or urine culture. Complete blood count measures were compared between hospitalization and prehospitalization or posthospitalization values. Children admitted to the hospital for elective surgical procedures served as controls. Blood parameters of hemolysis were investigated in some of the patients. Of the 70 patients studied, 49 (70%) were diagnosed with pyelonephritis and 21 (30%) had bacteremia. Mean (+/-SD) hemoglobin (Hgb) on hospital admission was 10.9+/-1.27 g/L as compared with 12.1+/-1.03 g/L of the controls, P<0.0001. Compared with normal-for-age Hgb values as a standard, 42 (60%) cases were identified as anemic. Compared with hospitalization values, Hgb and hematocrit (Hct) were significantly higher in prehospitalization or posthospitalization, whereas WBC values were significantly lower. All parameters of hemolysis, namely reticulocytes, bilirubin, lactate dehydrogenase (LDH), and haptoglobin, were normal. Bacteremia and pyelonephritis are accompanied by a significant drop in Hgb level. There is no evidence of hemolytic anemia in these patients. PMID:19755924

Ballin, Ami; Lotan, Amir; Serour, Francis; Ovental, Amit; Boaz, Mona; Senecky, Yehuda; Rief, Shimon

2009-10-01

408

The Bacillus thuringiensis cyt Genes for Hemolytic Endotoxins Constitute a Gene Family  

Microsoft Academic Search

In the same way that cry genes, coding for larvicidal delta endotoxins, constitute a large and diverse gene family, the cyt genes for hemolytic toxins seem to compose another set of highly related genes in Bacillus thuringiensis. Although the occurrence of Cyt hemolytic factors in B. thuringiensis has been typically associated with mosquitocidal strains, we have recently shown that cyt

ALEJANDRA GUERCHICOFF; ARMELLE DELECLUSE; CLARA P. RUBINSTEIN

2001-01-01

409

Anemia  

MedlinePLUS

... anemic, he or she will probably take a blood sample and send it to a lab for analysis. This will determine, among other things, the number, size, and shape of your red blood cells, the percentage of your blood that is ...

410

Anemia  

MedlinePLUS

... This is mostly a problem for children, young women who follow “fad” diets and people who don’t eat meat.Inability ... drink a lot of cow's milk, have a diet low in iron, or already had iron deficiency as an infant are also at risk.Pregnancy. Women who are pregnant or who are breastfeeding need ...

411

Anemia  

MedlinePLUS

... in the center. They carry oxygen and remove carbon dioxide (a waste product) from your body. These ... NHLBI updates Health Topics articles on a biennial cycle based on a thorough review of research findings ...

412

Anemia  

MedlinePLUS

Home Fact Sheet Categories Internet Bookmarks on AIDS Have Questions? Printing & Downloading Fact Sheets Permission to Use Fact Sheets Sponsors and Advertising Privacy Policy Project Staff Contact Us This site complies with the HONcode standard for trustworthy health information: verify here . Site ...

413

S-adenosylhomocysteine hydrolase, S-adenosylmethionine, S-adenosylhomocysteine: correlations with ribavirin induced anemia.  

PubMed

Objective is to speculate on the ribavirin induced anemia by inhibiting S-adenosylhomocysteine (SAH)-hydrolase activity. The major toxicity associated with the use of ribavirin is hemolytic anemia. This adverse effect has been ascribed to the accumulation of ribavirin triphosphate in the erythrocyte, which interferes with erythrocyte function. Ribavirin has been found to inhibit SAH-hydrolase activity in erythrocyte. SAH is further hydrolyzed to adenosine and homocysteine by SAH-hydrolase. The formation of S-adenosylmethionine (SAM) is then demethylated to SAH. SAH, the metabolite of SAM, on the other hand is a powerful inhibitor of methyltransferase enzymes, competing for the SAM binding site. A concurrent decrease in SAM and an increase in SAH levels would inhibit methylation of many tissue components including proteins, DNA, RNA, phospholipids and other small molecules. The enzyme protein carboxyl methyltransferase type II has been recently shown to play a crucial role in the repair of damaged proteins. SAM is the methyl donor of the reaction, and its demethylated product, SAH is the natural inhibitor of this reaction, as well as of most SAM-dependent methylations. The biological function of this transmethylation reaction is related to the repair or degradation of age-damaged proteins. Methyl ester formation in erythrocyte membrane proteins has been used as a marker reaction to tag these abnormal residues and to monitor their increase associated with erythrocyte ageing diseases. Liver disease is complicated by cholesterol deposition in hepatic and extrahepatic membranes. Erythrocyte membrane fluidity has been improved with the administration of SAM and correlated with the cholesterol/phospholipid ratio of the membranes. The levels of SAH-hydrolase activity were also found to undergo a sharp decrease with red cell ageing. The similarity of these alterations with certain morphofunctional characteristics of erythrocyte in some conditions as chronic renal failure, liver disease and hereditary spherocytosis makes it possible to hypothesize that the inhibition of SAH-hydrolase could constitute at least a part of ribavirin induced hemolytic anemia. PMID:15488656

Altintas, Engin; Sezgin, Orhan

2004-01-01

414

Severe hemolysis due to cloth wear 23 years after aortic valve replacement on a Starr-Edwards ball valve model 2320.  

PubMed

Despite iron substitution therapy, a patient developed severe hemolytic anemia 23 yr after insertion of a cloth-covered Starr-Edwards model 2320 aortic valve prosthesis. The prosthesis showed no sign of significant dysfunction. Upon removal, it showed extensive cloth wear on the inner surface of all three struts; one strut was completely denuded of its cloth covering. Hemolysis immediately resolved after replacement with a St Jude aortic prosthesis. PMID:12044439

Murakami, Mikiko; Tanaka, Hiroyuki; Watanabe, Masazumi; Shimizu, Masato; Sunamori, Makoto

2002-06-01

415

[Sensitivity of beta-hemolytic streptococci to antibiotics].  

PubMed

Susceptibility of 64 beta-hemolytic streptococcal strains isolated from the patients with sore throat was studied by the method of serial dilutions in fluid nutrient medium (Konikov broth). Heterogenecity with respect to the sensitivity was investigated in 34 strains among separate populations of the microbes (10 to 15 in every strain). The MIC of benzylpenicillin, oxacillin and erythromycin ranged within 0.007--0.24 U/ml, 0.02--0.36 gamma/ml and 0.005--0.1 gamma/ml respectively. The MIC of benzylpenicillin with respect to separate populations most sensitive to it was 0.007--0.015 U/ml, while that with respect to the lease sensitive populations ranged from 0.015 to 0.24 U/ml. The respective values for oxacillin were 0.02--0.12 and 0.18--0.36 gamma/ml and those for erythromycin were 0.005--0.025 and 0.05--0.1 gamma/ml. Therefore, the beta-hemolytic streptococci isolated from the patients with sore throat were characterized by a rather high sensitivity to the antibiotics which was important precondition for their efficiency in treatment of the patients with the above disease. PMID:354509

Liashenko, Iu I; Khodyrev, A P

1978-06-01

416

Isolation of a variant of subtilosin A with hemolytic activity.  

PubMed

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

2009-07-24

417

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.

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

2009-01-01

418

Atypical hemolytic uremic syndrome (aHUS): making the diagnosis.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. aHUS results from chronic, uncontrolled activity of the alternative complement pathway. In most patients, this defect is related to a genetic deficiency in one or more soluble and/or membrane-bound complement regulatory proteins. Complement factor H is most frequently implicated. Clinically, aHUS is often indistinguishable from the other TMAs: Shiga toxin–producing Escherichia coli (STEC) hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (TTP). TTP and aHUS are associated with high morbidity and mortality. aHUS has a distinct pathology from TTP. In nearly all patients, aHUS can be distinguished from TTP on the basis of an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) enzyme activity measurement. It is essential that aHUS and TTP be differentiated quickly, as they require markedly divergent treatments. The standard treatment for TTP is plasma exchange, a therapy that has no role for patients with a diagnosis of aHUS established by ADAMTS13 activity levels. PMID:23187605

Laurence, Jeffrey

2012-10-01

419

Covalent binding and hemolytic activity of complement proteins.  

PubMed Central

We report the inactivation of the third component of complement (C3) by hydroxylamine. C3 hemolytic and covalent binding activities decline with identical kinetics, demonstrating a direct correlation between the two activities. We conclude that covalent, surface-bound C3b is hemolytically active. The inactivation of C3 is first order with respect to hydroxylamine. We also studied C3 inactivation with [14C]methylamine. The inactivation corresponds quantitatively with the labeling of C3 in the C3d domain. The data obtained support the following hypothesis: there is an internal thioester within C3 which becomes highly reactive on activation to C3b, and C3b binds to receptive surfaces by transfer of the acyl function of the thioester to a hydroxyl group on the receptive surface. This proposed model for the reaction of C3 with receptive surfaces also applies to C4, which binds to membrane surfaces covalently and is able to be inactivated by hydroxylamine and methylamine. C5, on the other hand, is not inactivated by treatment with the amines. Images

Law, S K; Lichtenberg, N A; Levine, R P

1980-01-01

420

Anemia as a risk factor for cardiovascular disease  

Microsoft Academic Search

Anemia as a risk factor for cardiovascular disease. In the present review we examine the physiologic response to chronic anemia and describe potential adverse effects of anemia on myocardial and large arterial remodeling. We present observational data demonstrating that anemia is a risk factor for cardiovascular disease (CVD) outcomes in patients with chronic kidney disease and patients with heart failure.

Arema A. Pereira; Mark J. Sarnak

2003-01-01

421

Anemia and heart failure: A community study  

PubMed Central

Purpose Anemia is an important comorbidity in heart failure, and has been associated with increased mortality. The goals of this study were to define the prevalence of anemia in a community heart failure population, examine trends in prevalence over time, and evaluate the role of anemia in heart failure patients with preserved and reduced ejection fraction. Methods Two cohorts of Olmsted County residents with heart failure were examined. The retrospective cohort included incident heart failure cases from 1979–2002 (n=1063). The prospective cohort included active heart failure cases from 2003–2006 (n=677). Clinical characteristics were collected. Anemia was defined by WHO criteria. Results The prevalence of anemia was 40% in the retrospective and 53% in the prospective cohorts. Anemia prevalence increased by an estimated 16% between 1979 and 2002 (p=0.008), and was higher in those with preserved (?50%), vs. reduced (<50%) ejection fraction (58% vs. 48%, respectively, p<0.001) from 2003–2006. Anemia was associated with a large increase in the risk of death (p<0.001 both cohorts). The relationship between mortality and hemoglobin followed a J-shaped curve, with increased mortality with hemoglobin below 14mg/dL and above 16mg/dL. In the prospective cohort, after adjustment for clinical characteristics, the HR(95%CI) for death were 3.07(1.26–6.82) in those with hemoglobin ?16mg/dL and 2.39(1.37–4.27) in those with hemoglobin<10mg/dL using hemoglobin 14–16mg/dL as the referent. Conclusions In the community half of heart failure patients are anemic, and the prevalence of anemia increased over time. Anemia is more prevalent in heart failure with preserved ejection fraction and is associated with a large increase in mortality.

Dunlay, Shannon M; Weston, Susan A.; Redfield, Margaret M.; Killian, Jill M.; Roger, Veronique L.

2008-01-01

422

Vulvar squamous cell carcinoma associated with Fanconi's anemia.  

PubMed

Fanconi's anemia is a rare autosomal recessive disorder which is rarely associated with squamous cell carcinoma (SCC) of the vulva. We report a 23-year-old virgin female with Fanconi's anemia and diabetes mellitus who presented with a history of 6-month ulcerative lesions of the vulva. Gynecologic examination disclosed a 1 x 2 cm ulcerated tumor lesion at the right labia minor near to the urethra. The biopsy showed a high-grade vulvar intraepithelial neoplasia (VIN III). She underwent wide local excision for this lesion. Pathologic examination of the surgically removed specimen revealed SCC of the vulva. Therefore, radical vulvectomy and bilateral inguino-femoral lymphadenectomy were performed. Due to the involvement of right inguinal lymph node, radiotherapy with a total dose of 45 Gy was delivered to mid-pelvis through antero-posterior/postero-anterior fields with 18 mV photon energies. Until her last follow-up about 1 year after the treatment, the patient was free of disease or any recurrence at the site of operation. Patients with Fanconi's anemia have the risk of developing SCC of the genital tract. Radical vulvectomy and lymphadenectomy along with radiotherapy were associated with a satisfactory outcome in 1-year follow-up period in the presented patient. PMID:20217286

Mousavi, Azamsadat; Abbasi, Fatemeh; Abadi, Akram Ghahghai Nezam; Hashemi, Farnaz Amouzegar

2010-03-10

423

Regulation of Erythropoietin and Burst-Promoting Activity Production in Patients with Aplastic Anemia and Iron Deficiency Anemia  

Microsoft Academic Search

To clarify the control mechanism of production of erythropoietic growth factors in anemic states, we compared erythropoietin (Epo) and burst-promoting activity (BPA) in patients with aplastic anemia and iron deficiency anemia, using in vitro erythroid progenitor assays. Although serum levels of Epo activity increased in the presence of anemia, the rise was more marked in patients with aplastic anemia. BPA

Naohisa Takeichi; Tsukuru Umemura; Junji Nishimura; Seiji Motomura; Mitsuo Kozuru; Hiroshi Ibayashi

1988-01-01

424

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

2012-12-12

425

Anemia in Kidney Disease and Dialysis  

MedlinePLUS

... disease or an inflammatory problem. At one time, aluminum poisoning contributed to anemia in people with kidney ... caused by kidney failure were antacids that contained aluminum. But aluminum-free alternatives are now widely available. ...

426

What Happens After Treatment for Aplastic Anemia?  

MedlinePLUS

... have had aplastic anemia learn to live with uncertainty. Our document Living With Uncertainty: The Fear of Cancer Recurrence , gives more detailed ... very stressful. It has its own type of uncertainty. Our document When Cancer Doesn’t Go Away , ...

427

Factors influencing hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye.  

PubMed

In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU(50) of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40 microg/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and physical factors such as divalent cations, EDTA, (NH(4))(2)SO(4), pH and temperature. In the presence of Mg(2+), Cu(2+), Zn(2+), Fe(2+), Ca(2+) (>or=2 mM), Mn(2+) ((>or=1 mM), EDTA ((>or=2 mM) and (NH(4))(2)SO(4), the hemolytic activity of RFV was reduced. RFV had strong hemolytic activity at the pH 6-10 and the hemolytic ratios were 0.95-1.19. Hemolytic activity was temperature-sensitive and when RFV was pre-incubated at temperatures over 40 degrees C, it was sharply reduced. PMID:17306433

Yu, Huahua; Li, Cuiping; Li, Ronggui; Xing, Ronge; Liu, Song; Li, Pengcheng

2007-01-11

428

Factor I Autoantibodies in Patients with Atypical Hemolytic Uremic Syndrome: Disease-Associated or an Epiphenomenon?  

PubMed Central

Summary Background and objectives Atypical hemolytic uremic syndrome is a disease associated with mutations in the genes encoding the complement regulators factors H and I. In addition, factor H autoantibodies have been reported in ?10% of patients with atypical hemolytic uremic syndrome. This study searched for the presence of factor I autoantibodies in atypical hemolytic uremic syndrome. Design, setting, participants, & measurements This study screened 175 atypical hemolytic uremic syndrome patients for factor I autoantibodies using ELISA with confirmatory Western blotting. Functional studies using purified immunoglobulin from one patient were subsequently undertaken. Results Factor I autoantibodies were detected in three patients. In one patient with a high titer of autoantibody, the titer was tracked over time and was found to have no association with disease activity. This study found evidence of an immune complex of antibody and factor I in this patient, but purified IgG, isolated from current serum samples, had only a minor effect on fluid phase and cell surface complement regulation. Genetic analysis of the three patients with factor I autoantibodies revealed that they had two copies of the genes encoding factor H–related proteins 1 and 3 and therefore, did not have a deletion commonly associated with factor H autoantibodies in atypical hemolytic uremic syndrome. Two patients, however, had functionally significant mutations in complement factor H. Conclusions These findings reinforce the concept of multiple concurrent risk factors being associated with atypical hemolytic uremic syndrome but question whether autoantibodies per se predispose to atypical hemolytic uremic syndrome.

Kavanagh, David; Pappworth, Isabel Y.; Anderson, Holly; Hayes, Christine M.; Moore, Iain; Hunze, Eva-Maria; Bennaceur, Karim; Roversi, Pietro; Lea, Susan; Strain, Lisa; Ward, Roy; Plant, Nick; Nailescu, Corina; Goodship, Timothy H. J.

2012-01-01

429

Molecular Basis for Group B ? -hemolytic Streptococcal Disease  

NASA Astrophysics Data System (ADS)

Group B ? -hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin.

Hellerqvist, Carl G.; Sundell, Hakan; Gettins, Peter

1987-01-01

430

Hepcidin in anemia of chronic heart failure  

PubMed Central

Anemia is a common finding among patients with chronic heart failure. Although co-morbidities, such as kidney failure, might contribute to the pathogenesis of anemia, many patients with heart failure do not have any other obvious etiology for their anemia. We investigated whether anemia in heart failure is associated with an elevation in hepcidin concentration. We used time-of-flight mass spectrometry to measure hepcidin concentration in urine and serum samples of patients with heart failure and in control subjects. We found that the concentration of hepcidin was lower in urine samples of patients with heart failure compared to those of control subjects. Serum hepcidin was also reduced in heart failure but was not significantly lower than that in controls. There were no significant differences between hepcidin levels in patients with heart failure and anemia compared to patients with heart failure and normal hemoglobin. We concluded that hepcidin probably does not play a major role in pathogenesis of anemia in patients with chronic heart failure.

Divakaran, Vijay; Mehta, Sachin; Yao, David; Hassan, Saamir; Simpson, Steven; Wiegerinck, Erwin; Swinkels, Dorine W.; Mann, Douglas L.; Afshar-Kharghan, Vahid

2010-01-01

431

Anemia do lactente: etiologia e prevalência Anemia in infancy: etiology and prevalence  

Microsoft Academic Search

Objective: To verify the prevalence of anemia, iron deficiency anemia and iron deficiency in infants, at a Public Health Unit in the city of Goiânia - Brazil; to analyze and to correlate the hematologic and biochemical variables. Methods: A cross-sectional study was carried out. One hundred and ten full-term infants of the 120 mothers interviewed were included. The infants aged

Maria Claret; C. M. Hadler; Yara Juliano; Dirce M. Sigulem

2002-01-01

432

Congenital Fibrosarcoma as Cause for Fetal Anemia: Prenatal Diagnosis and in utero Treatment  

Microsoft Academic Search

Objectives: To discuss diagnosis and management of a case of a rare fetal tumor complicated by fetal anemia due to intratumoral hemorrhage. Case Report: We report on a 29-week-old fetus with a tumor in the posterior left shoulder region. The morphologic aspect of the tumor, lack of fetal movements and an increased middle cerebral artery (MCA) peak systolic velocity (PSV)

Matthias Scheier; Angela Ramoni; Alexander Alge; Christoph Brezinka; Gernot Reiter; Consolato Sergi; Josef Hager; Christian Marth

2008-01-01

433

STUDIES ON ANEMIA IN F1 HYBRID MICE INJECTED WITH PARENTAL STRAIN LYMPHOID CELLS  

PubMed Central

The survival of 51Cr-labeled erythrocytes has been studied in F1 hybrid mice in which wasting disease was produced by injection of parental lymphoid cells taken either from lymph nodes and thymus or from the spleen. Coincident with the development of the disease syndrome, there occurred a severe anemia accompanied by a sudden loss of circulating labeled erythrocytes, whether host or parental. This finding suggests that the anemia is not due solely to specific immunologic reaction of donor tissue against host erythrocytes.

Harriss, Eileen; Currie, Cicely; Kriss, Joseph P.; Kaplan, Henry S.

1961-01-01

434

A hemolytic peptide from the mycophilic fungus Sepedonium chrysospermum (Bull.) Fr.  

PubMed

The hemolytic activity of an extract of the mycoparasite Sepedonium chrysospermum (teleomorph Hypomyces chrysospermus) was detected and characterized. Extraction of the fungal biomass by methanol yielded a fraction in which the hemolytic activity against human red blood cells corresponded to a peptide with a molecular mass of 7,653.72 Da and an isoelectric point of approximately 5.8. The peptide was temperature resistant, and the hemolysis was only partially inhibited, even after a 30-min pre-incubation at 100°C. Its hemolytic activity was unaffected by treatment with proteolytic enzymes such as trypsin. Among the divalent cations assayed, Hg(2+) was the strongest inhibitor of hemolysis. The reducing agent, dithiothreitol, and the membrane lipid, cholesterol, demonstrated concentration-dependent inhibitory activities. Finally, hemolytic activity triggered by the peptide was analyzed by scanning electron microscopy, and a pore-forming activity was detected. PMID:22080344

Sanguineti, Elisa; Cosulich, Maria E; Salis, Annalisa; Damonte, Gianluca; Mariotti, Mauro G; Zotti, Mirca

2011-11-13

435

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

436

Purification and characterization of photosensitizing hemolytic toxin from harmful red tide phytoplankton, Heterocapsa circularisquama  

Microsoft Academic Search

We have previously found that the methanol extract prepared from Heterocapsa circularisquama, a harmful red rid dinoflagellate, showed light-dependent hemolytic activity toward rabbit erythrocytes. Interestingly, the cytotoxicity of the extract against HeLa cells was also strictly light-dependent, and no significant toxic effect was observed even at very high concentration in the dark. In this study, the hemolytic agents present in

Yousuke Miyazaki; Takuji Nakashima; Takashi Iwashita; Tsuyoshi Fujita; Kenichi Yamaguchi; Tatsuya Oda

2005-01-01

437

Hemolytic properties of synthetic nano- and porous silica particles: the effect of surface properties and the protection by the plasma corona.  

PubMed

Novel silica materials incorporating nanotechnology are promising materials for biomedical applications, but their novel properties may also bring unforeseen behavior in biological systems. Micro-size silica is well documented to induce hemolysis, but little is known about the hemolytic activities of nanostructured silica materials. In this study, the hemolytic properties of synthetic amorphous silica nanoparticles with primary sizes of 7-14 nm (hydrophilic vs. hydrophobic), 5-15 nm, 20 nm and 50 nm, and model meso/macroporous silica particles with pore diameters of 40 nm and 170 nm are investigated. A crystalline silica sample (0.5-10 ?m) is included for benchmarking purposes. Special emphasis is given to investigations of how the temperature and solution complexity (solvent, plasma), as well as the physicochemical properties (such as size, surface charge, hydrophobicity and other surface properties), link to the hemolytic activities of these particles. Results suggests the potential importance of small size and large external surface area, as well as surface charge/structure, in the hemolysis of silica particles. Furthermore, a significant correlation is observed between the hemolytic profile of red blood cells and the cytotoxicity profile of human promyelocytic leukemia cells (HL-60) induced by nano- and porous silica particles, suggesting a potential universal mechanism of action. Importantly, the results generated suggest that the protective effect of plasma towards silica nanoparticle-induced hemolysis as well as cytotoxicity is primarily due to the protein/lipid layer shielding the silica particle surface. These results will assist the rational design of hemocompatible silica particles for biomedical applications. PMID:22522009

Shi, J; Hedberg, Y; Lundin, M; Odnevall Wallinder, I; Karlsson, H L; Möller, L

2012-04-20

438

Bed bugs reproductive life cycle in the clothes of a patient suffering from Alzheimer's disease results in iron deficiency anemia.  

PubMed

We report the case of an 82-year-old patient, hospitalized for malaise. Her clothes were infested by numerous insects and the entomological analysis identified them as being Cimex lectularius (bed bugs). The history of the patient highlighted severe cognitive impairment. The biological assessment initially showed a profound microcytic, aregenerative, iron deficiency anemia. A vitamin B12 deficiency due to pernicious anemia (positive intrinsic factor antibodies) was also highlighted, but this was not enough to explain the anemia without macrocytosis. Laboratory tests, endoscopy and a CT scan eliminated a tumor etiology responsible for occult bleeding. The patient had a mild itchy rash which was linked to the massive colonization by the bed bugs. The C. lectularius bite is most often considered benign because it is not a vector of infectious agents. Far from trivial, a massive human colonization by bed bugs may cause such a hematic depletion that severe microcytic anemia may result. PMID:23673315

Sabou, Marcela; Gallo Imperiale, Delphine; Andrès, Emmanuel; Abou-Bacar, Ahmed; Foeglé, Jacinthe; Lavigne, Thierry; Kaltenbach, Georges; Candolfi, Ermanno

2013-05-15

439

Bed bugs reproductive life cycle in the clothes of a patient suffering from Alzheimer's disease results in iron deficiency anemia  

PubMed Central

We report the case of an 82-year-old patient, hospitalized for malaise. Her clothes were infested by numerous insects and the entomological analysis identified them as being Cimex lectularius (bed bugs). The history of the patient highlighted severe cognitive impairment. The biological assessment initially showed a profound microcyt