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Sample records for severe hemolytic anemia

  1. Hemolytic anemia

    Science.gov (United States)

    Anemia - hemolytic ... bones that helps form all blood cells. Hemolytic anemia occurs when the bone marrow isn't making ... destroyed. There are several possible causes of hemolytic anemia. Red blood cells may be destroyed due to: ...

  2. SEVERE IMMUNE HEMOLYTIC ANEMIA AFTER LIVER TRANSPLANTATION

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    A. I. Sushkov

    2013-01-01

    Full Text Available Clinical case of successful treatment of severe immune hemolytic anemia after liver transplantation is represen- ted in this article. The cause of complication was so-called passenger lymphocyte syndrome (a type of graft- versus-host disease. Two plasmapheresis sessions and Ig (0.5 g/kg in combination with increased maintenance immunosuppression with a short course of oral methylprednisolone in a total dose of 150 mg during 12 days were effective. The patient was discharged from hospital 34 days after transplantation in a satisfactory condition with a stable hemoglobin level. 

  3. Hemolytic Anemia

    Science.gov (United States)

    ... worsen your condition or lead to complications. Hemolytic Anemia and Children Parents of children who have hemolytic anemia usually ... members, friends, and your child's classmates about hemolytic anemia. You also may want to tell your child's teachers or other caregivers about the condition. Let ...

  4. Severe late anemia of hemolytic disease of the newborn

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    Mitchell, Simon; James, Andrew

    1999-01-01

    Late anemia is a well-recognized complication of Rhesus hemolytic disease of the newborn (HDN). The incidence of Rhesus HDN is declining, with a tendency for more severely affected pregnancies to be managed in specialist centres. Consequently, many paediatric departments may see relatively few affected infants with comparatively mild disease, and the risk of late anemia in such cases may not always be appreciated. Two cases of infants born with evidence of Rhesus isoimmunization noted at birth and encountering no immediate problems other than mild hyperbilirubinemia are described. After an uneventful early neonatal course, both infants were discharged without follow-up and presented in the second to third weeks of life with severe, life-threatening anemia, leading to neurological sequelae in one case. The importance of close surveillance, including hemoglobin measurements, in all infants with Rhesus hemolytic disease, irrespective of initial severity, is reiterated. PMID:20212966

  5. Drug-induced immune hemolytic anemia

    Science.gov (United States)

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... Drugs that can cause this type of hemolytic anemia include: Cephalosporins (a class of antibiotics), most common ...

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

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    Sudipta Sekhar Das

    2014-01-01

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

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

    Science.gov (United States)

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

    2014-07-01

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

  8. Genetic diagnosis for congenital hemolytic anemia.

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    Ohga, Shouichi

    2016-01-01

    Congenital hemolytic anemia is a group of monogenic diseases presenting with anemia due to increased destruction of circulating erythrocytes. The etiology of inherited anemia accounts for germline mutations of the responsible genes coding for the structural components of erythrocytes and extra-erythrocytes. The erythrocyte abnormalities are classified into three major disorders of red cell membrane defects, hemoglobinopathies, and red cell enzymopathies. The extra-erythrocyte abnormalities, typified by consumption coagulopathy and intravascular hemolysis, include Upshaw-Schulman syndrome and atypical hemolytic uremic syndrome. The clinical manifestations of congenital hemolytic anemia are anemia, jaundice, cholelithiasis and splenomegaly, while the onset mode and severity are both variable. Genetic overlapping of red cell membrane protein disorders, and distinct frequency and mutation spectra differing among races make it difficult to understand this disease entity. On the other hand, genetic modifiers for the phenotype of β-globin diseases provide useful information for selecting the optimal treatment and for long-term management. Recently, next generation sequencing techniques have enabled us to determine the novel causative genes in patients with undiagnosed hemolytic anemias. We herein review the concept and strategy for genetic diagnosis of inherited hemolytic anemias.

  9. Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

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    Fatih Demircioğlu

    2010-09-01

    Full Text Available A 33 weeks’ gestation, a baby with rhesus hemolytic disease (RHD, who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.

  10. Immune hemolytic anemia

    Science.gov (United States)

    ... intravenous immunoglobulin (IVIG) or removal of the spleen (splenectomy) may be considered. You may receive treatment to ... need special treatment. In most people, steroids or splenectomy can totally or partially control anemia.

  11. Parvovirus B19 infection presenting with severe erythroid aplastic crisis during pregnancy in a woman with autoimmune hemolytic anemia and alpha-thalassemia trait: a case report.

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    Chen, Chi-Ching; Chen, Chin-Shan; Wang, Wei-Yao; Ma, Jui-Shan; Shu, Hwei-Fan; Fan, Frank S

    2015-03-12

    Parvovirus B19 virus commonly causes subclinical infection, but it can prove fatal to the fetus during pregnancy and cause severe anemia in an adult with hemolytic diseases. We present the case of a woman with autoimmune hemolytic anemia who was diagnosed with parvovirus B19-induced transient aplastic crisis during her second trimester of pregnancy and faced the high risk of both fetal and maternal complications related to this specific viral infection. To the best of our knowledge, the experience of successful intravenous immunoglobulin treatment for B19 virus infection during pregnancy, as in our case, is limited. A 28-year-old and 20-week pregnant Chinese woman with genetically confirmed alpha-thalassemia trait was diagnosed with cold antibody autoimmune hemolytic anemia and suffered from transient aplastic crisis caused by B19 virus infection. She received intravenous immunoglobulin treatment to reduce the risk of hydrops fetalis. Her peripheral blood reticulocyte percentage recovered, but anemia persisted, so she underwent several courses of high dose intravenous dexamethasone for controlling her underlying hemolytic problem. Finally, her hemoglobin levels remained stable with no need of erythrocyte transfusion, and a healthy baby boy was naturally delivered. Parvovirus B19 virus infection should be considered when a sudden exacerbation of anemia occurs in a patient with hemolytic disease, and the possible fetal complications caused by maternal B19 virus infection during pregnancy should not be ignored. Close monitoring and adequate management can keep both mother and fetus safe.

  12. The challenge of microangiopathic hemolytic anemia

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    Hassanain Hani Hassan

    2017-01-01

    Full Text Available Microangiopathic hemolytic anemia (MAHA is a Coomb's-negative hemolytic anemia characterized by red cell fragmentation (schistocytes. Thrombotic microangiopathy anemia, including thrombotic thrombocytopenia and hemolytic-uremic syndrome, malignant hypertension, preeclampsia are among the most common causes. We present a case of MAHA presenting with thrombocytopenia initially diagnosed as MAHA secondary to thrombotic thrombocytopenic purpura and received five sessions plasmapheresis without improvement but with worsening of anemia and thrombocytopenia. On further inquiry, glucose-6-phosphate dehydrogenase deficiency was identified, and the patient showed dramatic recovery after the trial of B12 and folate.

  13. Hemolytic anemias during pregnancy and the reproductive years

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    Mintz, U.; Moohr, J.W.; Ultmann, J.E.

    1977-11-01

    Anemia is a common phenomenon in women during the reproductive years. In pregnancy, it is associated with an increased incidence of maternal-fetal morbidity and mortality. The approach to the investigation of anemic women suspected of having hemolytic anemia of either congenital or acquired etiology is the subject of this article. Various conditions in the pregnant women can have hematologic consequences for the newborn infant; these conditions include sensitization to fetal blood cells, infections, drug ingestion and the possession of genes for hereditary hemolytic disorders, which may be transmitted to the fetus. Because several forms of hemolytic anemias are hereditary or are caused by an altered gene, genetic consultation is important.

  14. Severe acute hepatitis and cold agglutinin-related hemolytic anemia secondary to prime infection with Epstein-Barr virus

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    Guillermo Ontanilla-Clavijo

    Full Text Available Epstein-Barr virus, a member of the Herpesviridae family, is responsible for the infectious mononucleosis clinical syndrome, which mainly includes the pharyngitis, fever, and lymphadenopathy triad after incubation for 30-50 days. The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%. From a hematological standpoint it may manifest aplastic anemia, neutropenia, and thrombocytopenia. We report a case of infectious mononucleosis that included severe acute hepatitis and was associated with severe hemolytic anemia secondary to cold agglutinins. After exclusion of other etiologies, and given the clinical suspicion of the above association, which was later confirmed by lab tests, empiric therapy was initiated with antiviral agents (aciclovir + valganciclovir and corticoids, which resulted in a progressive clinical improvement until complete remission. Therefore, we believe that this case report will reinforce the clinical evidence in support of the above combined therapy for serious infectious mononucleosis as a step prior to liver transplantation.

  15. Retinal phlebitis associated with autoimmune hemolytic anemia.

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    Chew, Fiona L M; Tajunisah, Iqbal

    2009-01-01

    To describe a case of retinal phlebitis associated with autoimmune hemolytic anemia. Observational case report. A 44-year-old Indian man diagnosed with autoimmune hemolytic anemia presented with a 1-week history of blurred vision in both eyes. Fundus biomicroscopy revealed bilateral peripheral retinal venous sheathing and cellophane maculopathy. Fundus fluorescent angiogram showed bilateral late leakage from the peripheral venous arcades and submacular fluid accumulation. The retinal phlebitis resolved following a blood transfusion and administration of systemic steroids. Retinopathy associated with autoimmune hemolytic anemia is not well known. This is thought to be the first documentation of retinal phlebitis occurring in this condition.

  16. Thrombotic Microangiopathic Hemolytic Anemia without Evidence of Hemolytic Uremic Syndrome

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    Şinasi Özsoylu

    2016-03-01

    Full Text Available In a recent issue of this journal Dr. Oymak and her colleagues presented a clinically and genetically well-studied 5-year-old boy who was seen with severe microangiopathic hemolytic anemia without laboratory findings of renal involvement despite complement factor H gene mutations [1]. Because of Yeneral’s extensive review [2] on atypical hemolytic uremic syndrome (aHUS published recently in the Turkish Journal of Hematology, I brought it to readers’ attention that more recently some authors do not use ‘aHUS’, which was historically used to distinguish heterogeneous uncharacterized syndromes from Shiga toxin-related HUS, since the term lacks both specificity and suggested causes [3]. Though in our patient with thrombotic thrombocytopenic purpura renal involvement was documented at the beginning but not in the last two recurrences, neither serum nor urinary findings indicated kidney involvement [4]. Although the discussions of Dr. Oymak et al. are well taken, the term ‘microangiopathic hemolytic anemia’ is covering the syndrome to a large extent as suggested by George and Nester

  17. Thyroid storm and warm autoimmune hemolytic anemia.

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    Moore, Joseph A; Gliga, Louise; Nagalla, Srikanth

    2017-08-01

    Graves' disease is often associated with other autoimmune disorders, including rare associations with autoimmune hemolytic anemia (AIHA). We describe a unique presentation of thyroid storm and warm AIHA diagnosed concurrently in a young female with hyperthyroidism. The patient presented with nausea, vomiting, diarrhea and altered mental status. Laboratory studies revealed hemoglobin 3.9g/dL, platelets 171×10 9 L -1 , haptoglobin storm and warm AIHA. She was started on glucocorticoids to treat both warm AIHA and thyroid storm, as well as antithyroid medications, propranolol and folic acid. Due to profound anemia and hemodynamic instability, the patient was transfused two units of uncrossmatched packed red blood cells slowly and tolerated this well. She was discharged on methimazole as well as a prolonged prednisone taper, and achieved complete resolution of the thyrotoxicosis and anemia at one month. Hyperthyroidism can affect all three blood cell lineages of the hematopoietic system. Anemia can be seen in 10-20% of patients with thyrotoxicosis. Several autoimmune processes can lead to anemia in Graves' disease, including pernicious anemia, celiac disease, and warm AIHA. This case illustrates a rarely described presentation of a patient with Graves' disease presenting with concurrent thyroid storm and warm AIHA. Copyright © 2017 Elsevier Ltd. All rights reserved.

  18. Hemolytic Anemia after Aortic Valve Replacement: a Case Report

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    Feridoun Sabzi

    2015-10-01

    Full Text Available Hemolytic anemia is exceedingly rare and an underestimated complication after aortic valve replacement (AVR.The mechanism responsible for hemolysis most commonly involves a regurgitated flow or jet that related to paravalvar leak or turbulence of subvalvar stenosis. It appears to be independent of its severity as assessed by echocardiography. We present a case of a 24-year-old man with a history of AVR in 10 year ago that developed severe hemolytic anemia due to a mild subvalvar stenosis caused by pannus formation and mild hypertrophic septum. After exclusion of other causes of hemolytic anemia and the lack of clinical and laboratory improvement, the patient underwent redo valve surgery with pannus and subvalvar hypertrophic septum resection. Anemia and heart failure symptoms gradually resolved after surgery

  19. Recommendations regarding splenectomy in hereditary hemolytic anemias

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    Iolascon, Achille; Andolfo, Immacolata; Barcellini, Wilma; Corcione, Francesco; Garçon, Loïc; De Franceschi, Lucia; Pignata, Claudio; Graziadei, Giovanna; Pospisilova, Dagmar; Rees, David C.; de Montalembert, Mariane; Rivella, Stefano; Gambale, Antonella; Russo, Roberta; Ribeiro, Leticia; Vives-Corrons, Jules; Martinez, Patricia Aguilar; Kattamis, Antonis; Gulbis, Beatrice; Cappellini, Maria Domenica; Roberts, Irene; Tamary, Hannah

    2017-01-01

    Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children. PMID:28550188

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

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    Beretta Chiara

    2009-04-01

    Full Text Available Abstract 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 of pediatric patients with refractory autoimmune hemolytic anemia. Case presentation We describe the case of an 8-month-old Caucasian girl affected by a severe form of autoimmune hemolytic anemia, which required continuous steroid treatment for 16 months. Thereafter, she received 4 weekly doses of rituximab (375 mg/m2/dose associated with steroid therapy, which was then tapered over the subsequent 2 weeks. One month after the last dose of rrituximab, she presented with recurrence of severe hemolysis and received two more doses of rrituximab. The patient remained in clinical remission for 7 months, before presenting with a further relapse. An alternative heavy immunosuppressive therapy was administered combining cyclophosphamide 10 mg/kg/day for 10 days with methylprednisolone 40 mg/kg/day for 5 days, which was then tapered down over 3 weeks. While still on steroid therapy, the patient developed an interstitial pneumonia with Acute Respiratory Distress Syndrome, which required immediate admission to the intensive care unit where extracorporeal membrane oxygenation therapy was administered continuously for 37 days. At 16-month follow-up, the patient is alive and in good clinical condition, with no organ dysfunction, free from any immunosuppressive treatment and with a normal Hb level. Conclusions This case shows that aggressive combined immunosuppressive therapy may lead to a sustained complete remission in children with refractory autoimmune hemolytic anemia. However, the severe life-threatening complication presented by our

  1. Pure red cell aplasia following autoimmune hemolytic anemia: An enigma

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    M Saha

    2013-01-01

    Full Text Available 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.

  2. Hemolytic anemia caused by kinking of dacron grafts implanted in ...

    African Journals Online (AJOL)

    Background: Hemolytic anemia caused by a kinked Dacron graft is a rare complication after repair of acute aortic dissection. We present a case of hemolytic anemia due to kinking of previously implanted Dacron graft for ascending aorta dissection treated by surgery and replaced with new Dacron. Case Details: We report a ...

  3. Autoimmune hemolytic anemia: transfusion challenges and solutions

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    Barros MM

    2017-03-01

    Full Text Available Melca M O Barros, Dante M Langhi Jr, José O Bordin Department of Clinical and Experimental Oncology, Universidade Federal de São Paulo, São Paulo, Brazil Abstract: Autoimmune hemolytic anemia (AIHA is defined as the increased destruction of red blood cells (RBCs in the presence of anti-RBC autoantibodies and/or complement. Classification of AIHA is based on the optimal auto-RBC antibody reactivity temperatures and includes warm, cold-reactive, mixed AIHA, and drug-induced AIHA subtypes. AIHA is a rare disease, and recommendations for transfusion are based mainly on results from retrospective data and relatively small cohort studies, including heterogeneous patient samples or single case reports. In this article, we will review the challenges and solutions to safely transfuse AIHA patients. We will reflect on the indication for transfusion in AIHA and the difficulty in the accomplishment of immunohematological procedures for the selection of the safest and most compatible RBC units. Keywords: hemolytic anemia, RBC autoantibodies, autoimmunity, hemolysis, direct ­antiglobulin test

  4. [Treatment and results of therapy in autoimmune hemolytic anemia].

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    Tasić, J; Macukanović, L; Pavlović, M; Koraćević, S; Govedarević, N; Kitić, Lj; Tijanić, I; Bakić, M

    1994-01-01

    Basic principles in the therapy of idiopathic autoimmune hemolytic anemia induced by warm antibody were glucocorticoides and splenectomy. Immunosupresive drugs, plasmaferesis and intravenous high doses gamma globulin therapy are also useful. In secundary autoimmune hemolytic anemia induced by warm antibody we treated basic illness. During the period of 1990-1992 we treated 21 patients with primary autoimmune hemolytic anemia and 6 patients with secondary /4 CLL and 2 Non-Hodgkin's lymphoma/. Complete remission we found as a normalisation of reticulocites and hemoglobin level respectively. Complete remission by corticoides we got in 14/21 patients, partial response in 2/21 respectively. Complete response by splenectomy we got in 2/3 splenoctomized patients (idiopathic type). For successful treatment secondary hemolytic anemias we treated primary diseases (CLL and malignant lymphoma) and we got in 4/6 patients complete remission. Our results were standard in both type of autoimmune hemolytic anaemias induced by warm antibody.

  5. Severe Aplastic Anemia (SAA)

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    ... page Print this page My Cart Severe aplastic anemia (SAA) Severe aplastic anemia (SAA) is a disease ... leukemia (ALL) Other diseases What is severe aplastic anemia (SAA)? SAA is a bone marrow disease. The ...

  6. Autoimmune hemolytic anemia: From lab to bedside

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    R K Chaudhary

    2014-01-01

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

  7. Pulmonary hypertension in chronic hemolytic anemias: Pathophysiology and treatment.

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    Haw, Alexandra; Palevsky, Harold I

    2018-04-01

    Pulmonary hypertension has emerged as a major cause of morbidity and mortality in patients with hemoglobinopathies and chronic hemolytic anemias. These hematological diseases include - but are not limited to - sickle cell disease (SCD), thalassemia, paroxysmal nocturnal hematuria, and hereditary spherocytosis. Although most studies have been based on the use of echocardiography as a screening tool for pulmonary hypertension as opposed to the gold standard of right heart catheterization for definitive diagnosis, the association between chronic hemolytic anemia and pulmonary hypertension is evident. Studies have shown that patients with SCD and a tricuspid regurgitant velocity (TRV) ≥ 2.5 m/sec are at increased risk of pulmonary hypertension and are at increased mortality risk. Additional markers of risk of pulmonary hypertension and increased mortality include a pro-BNP >160 pg/mL combined with a 6-min walk distance of pulmonary hypertension in chronic hemolytic anemias. Copyright © 2018 Elsevier Ltd. All rights reserved.

  8. Pulmonary aspergillosis and central nervous system hemorrhage as complications of autoimmune hemolytic anemia treated with corticosteroids.

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    Cleri, Dennis J; Moser, Robert L; Villota, Francisco J; Wang, Yue; Husain, Syed A; Nadeem, Shahzinah; Anjari, Tarek; Sajed, Mohammad

    2003-06-01

    Warm, active antibody adult autoimmune hemolytic anemia is the most common form of hemolytic anemia not related to drug therapy. Mortality in adult autoimmune hemolytic anemia is related to the inability to successfully treat patients' underlying disease, or the infectious complications of splenectomy and prolonged steroid therapy. Predisposing factors for invasive aspergillosis are neutropenia and steroid therapy. We present a fatal case of aspergillosis complicating a nonneutropenic case of warm active antibody adult autoimmune hemolytic anemia treated with prolonged steroid therapy.

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

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    Charalampos Grigoriadis

    2013-01-01

    Full Text Available 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.

  10. Severe hemolytic disease of the newborn from anti-e.

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    McAdams, R M; Dotzler, S A; Winter, L W; Kerecman, J D

    2008-03-01

    Maternal antibody-mediated fetal red blood cell destruction secondary to non-D Rhesus (Rh) antibodies is a significant cause of hemolytic disease of the newborn (HDN). Here, we report a rare case of severe HDN associated with maternal antibody to Rh e. In addition to severe anemia, the infant developed thrombocytopenia, conjugated hyperbilirubinemia and cholelithiasis. Resolution of the infant's cholelithiasis occurred following treatment with ursodeoxycholic acid.

  11. Alloimmunization in autoimmune hemolytic anemia patient: The differential adsorption approach

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    Ravi C Dara

    2017-01-01

    Full Text Available Patients of β-thalassemia major are dependent on regular blood transfusions for their entire lifetime. Development of antibodies against red blood cell (RBC antigen which may be alloantibody or autoantibody, several times as a result of frequent red cell component transfusions, further complicates the subsequent transfusion therapy. Among the autoantibodies, warm-reactive autoantibodies are commoner and interfere in the pretransfusion testing. These RBC autoantibodies present in patient's serum potentially react with all the cells of antibody identification panel giving “pan-reactive” picture and making alloantibody identification complex. In this report, we present our approach in a thalassemia patient who presented with warm-type autoimmune hemolytic anemia, low hemoglobin of 5.8 g/dl, and three significant alloantibodies (anti-D, anti-S, and anti-Jk b which were masked by pan-reactive warm autoantibody(s. Differential adsorption was used to unmask underlying alloantibodies. We suggest that differential adsorption procedure is an effective and efficient method for autoantibody adsorption, detection, and identification of masked alloantibody(s, especially in patients with low hemoglobin and history of recent blood transfusion.

  12. Autoimmune Hemolytic Anemia as a Complication of Nivolumab Therapy.

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    Palla, Amruth R; Kennedy, Devin; Mosharraf, Hossain; Doll, Donald

    2016-01-01

    Recently, immunotherapeutic drugs, including PD-1 inhibitors (nivolumab, pembrolizumab), PD-L1 inhibitors (atezolizumab, avelumab), and CTLA4 inhibitors (ipiliumumab), have emerged as important additions to the armamentarium against certain malignancies and have been incorporated into therapeutic protocols for first-, second-, or third-line agents for these metastatic cancers. Immune checkpoint inhibitor nivolumab is currently FDA approved for the treatment of patients with metastatic malignant melanoma [Redman et al.: BMC Med 2016;14: 20], metastatic non-small cell lung cancer [Guibert and Mazières: Expert Opin Biol Ther 2015;15: 1789-1797], metastatic renal cell cancer [Farolfi et al.: Expert Opin Drug Metab Toxicol 2016;12: 1089-1096], and relapsed or refractory classic Hodgkin's lymphoma [Villasboas and Ansell: Expert Rev Anticancer Ther 2016;16: 5-12]. Given the current and increasing indications for these drugs, it is essential for all physicians to become well versed with their common adverse effects and to be observant for other less documented clinical conditions that could be unmasked with the use of such medications. A definite association between autoimmune hemolytic anemia and the immune checkpoint inhibitor nivolumab has not been clearly documented, although a few cases have been reported recently [Kong et al.: Melanoma Res 2016;26: 202-204; Schwab et al.: Case Rep Oncol 2016;9: 373-378; Tardy et al.: Hematol Oncol 2016, DOI: 10.1002/hon.2338]. We report a case of fatal autoimmune hemolytic anemia refractory to steroids in a patient treated with nivolumab for metastatic lung cancer, and reflect on the other reported cases of autoimmune hemolytic anemia after the use of nivolumab.

  13. Autoimmune Hemolytic Anemia as a Complication of Nivolumab Therapy

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    Amruth R. Palla

    2016-11-01

    Full Text Available Recently, immunotherapeutic drugs, including PD-1 inhibitors (nivolumab, pembrolizumab, PD-L1 inhibitors (atezolizumab, avelumab, and CTLA4 inhibitors (ipiliumumab, have emerged as important additions to the armamentarium against certain malignancies and have been incorporated into therapeutic protocols for first-, second-, or third-line agents for these metastatic cancers. Immune checkpoint inhibitor nivolumab is currently FDA approved for the treatment of patients with metastatic malignant melanoma [Redman et al.: BMC Med 2016;14: 20], metastatic non-small cell lung cancer [Guibert and Mazières: Expert Opin Biol Ther 2015;15: 1789–1797], metastatic renal cell cancer [Farolfi et al.: Expert Opin Drug Metab Toxicol 2016;12: 1089–1096], and relapsed or refractory classic Hodgkin’s lymphoma [Villasboas and Ansell: Expert Rev Anticancer Ther 2016;16: 5–12]. Given the current and increasing indications for these drugs, it is essential for all physicians to become well versed with their common adverse effects and to be observant for other less documented clinical conditions that could be unmasked with the use of such medications. A definite association between autoimmune hemolytic anemia and the immune checkpoint inhibitor nivolumab has not been clearly documented, although a few cases have been reported recently [Kong et al.: Melanoma Res 2016;26: 202–204; Schwab et al.: Case Rep Oncol 2016;9: 373–378; Tardy et al.: Hematol Oncol 2016, DOI: 10.1002/hon.2338]. We report a case of fatal autoimmune hemolytic anemia refractory to steroids in a patient treated with nivolumab for metastatic lung cancer, and reflect on the other reported cases of autoimmune hemolytic anemia after the use of nivolumab.

  14. Parvovirus B19-triggered acute hemolytic anemia and thrombocytopenia in a child with Evans syndrome

    Directory of Open Access Journals (Sweden)

    ELPIS MANTADAKIS

    2018-03-01

    Full Text Available Background: Human parvovirus B19 (HPV-B19 is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. Case report. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thromocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg. Conclusion: Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present with reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

  15. Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.

    Science.gov (United States)

    Zikidou, Panagiota; Grapsa, Anastassia; Bezirgiannidou, Zoe; Chatzimichael, Athanassios; Mantadakis, Elpis

    2018-01-01

    Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with the presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg. Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

  16. Positive predictive value of diagnosis coding for hemolytic anemias in the Danish National Patient Register

    DEFF Research Database (Denmark)

    Hansen, Dennis Lund; Overgaard, Ulrik Malthe; Pedersen, Lars

    2016-01-01

    . Patients with mechanical reason for hemolysis such as an artificial heart valve, and patients with vitamin-B12 or folic acid deficiency were excluded. RESULTS: We identified 412 eligible patients: 249 with a congenital hemolytic anemia diagnosis and 163 with acquired hemolytic anemia diagnosis. In all...

  17. A thermolabile aldolase A mutant causes fever-induced recurrent rhabdomyolysis without hemolytic anemia.

    Directory of Open Access Journals (Sweden)

    Asmaa Mamoune

    2014-11-01

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

  18. Rasburicase-induced Hemolytic Anemia in an Adolescent With Unknown Glucose-6-Phosphate Dehydrogenase Deficiency.

    Science.gov (United States)

    Akande, Manzilat; Audino, Anthony N; Tobias, Joseph D

    2017-01-01

    Rasburicase, used in the prevention and treatment of tumor lysis syndrome (TLS), may cause hemolytic anemia and methemoglobinemia in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Although routine screening for G6PD deficiency has been recommended, given the turnaround time for test results and the urgency to treat TLS, such screening may not be feasible. We report a case of rasburicase-induced hemolytic anemia without methemoglobinemia in an adolescent with T-cell lymphoblastic lymphoma, TLS, and previously unrecognized G6PD deficiency. Previous reports of hemolytic anemia with rasburicase are reviewed, mechanisms discussed, and preventative strategies presented.

  19. Lung papillary adenocarcinoma complicated with paraneoplastic autoimmune hemolytic anemia: A case report

    OpenAIRE

    Xing, Limin; Wang, Huaquan; Qu, Wen; Fang, Fang; Dong, Qi-e; Shao, Zonghong

    2014-01-01

    A middle-aged woman presented at our facility and was diagnosed after surgery with lung papillary adenocarcinoma. Seven years earlier, she had suffered from autoimmune hemolytic anemia (AIHA), which was refractory. Following lung surgery, the AIHA was cured.

  20. Hypophosphatemia and hemolytic anemia associated with diabetes mellitus and hepatic lipidosis in cats.

    Science.gov (United States)

    Adams, L G; Hardy, R M; Weiss, D J; Bartges, J W

    1993-01-01

    Hypophosphatemia associated with hemolytic anemia was diagnosed in five cats with diabetes mellitus and in one cat with idiopathic hepatic lipidosis. The hematocrit began decreasing within 24 to 48 hours after documented hypophosphatemia in each case. The anemia resolved in all five surviving cats. Because of the temporal relationship and lack of other detectable causes, hemolytic anemia was presumed to be caused by hypophosphatemia. There were increased Heinz bodies in three of six hypophosphatemic cats during episodes of hemolysis. Intravenous potassium phosphate administration corrected the hypophosphatemia in four of five cats. The effective dosages of intravenous phosphate ranged from 0.011 to 0.017 mmol of phosphate/kg/h for 6 to 12 hours. Hypocalcemia (5.4 to 8.7 mg/dL) occurred in four of five cats treated with intravenous phosphate; however, only one cat developed clinical signs attributable to hypocalcemia. Based on this retrospective study, we recommend monitoring serum phosphorus concentration every 6 to 12 hours in cats likely to become hypophosphatemic. Treatment of hypophosphatemia in cats is warranted because of the apparent increased susceptibility of cats to hypophosphatemia-induced hemolysis. Cats with severe hypophosphatemia (< or = 1.5 mg/dL) should be given oral or parenteral phosphate if contraindications do not exist.

  1. Severe anemia in Malawian children

    NARCIS (Netherlands)

    Calis, Job C. J.; Phiri, Kamija S.; Faragher, E. Brian; Brabin, Bernard J.; Bates, Imelda; Cuevas, Luis E.; de Haan, Rob J.; Phiri, Ajib I.; Malange, Pelani; Khoka, Mirriam; Hulshof, Paul J. M.; van Lieshout, Lisette; Beld, Marcel G. H. M.; teo, Yik Y.; Rockett, Kirk A.; Richardson, Anna; Kwiatkowski, Dominic P.; Molyneux, Malcolm E.; Boele van Hensbroek, Michael

    2008-01-01

    Background Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. Methods We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and

  2. Severe anemia in Malawian children

    NARCIS (Netherlands)

    Calis, Job Cj; Phiri, Kamija S.; Faragher, E. Brian; Brabin, Bernard J.; Bates, Imelda; Cuevas, Luis E.; de Haan, Rob J.; Phiri, Ajib I.; Malange, Pelani; Khoka, Mirriam; Hulshof, Paul Jm; van Lieshout, Lisette; Beld, Marcel Ghm; teo, Yik Y.; Rockett, Kirk A.; Richardson, Anna; Kwiatkowski, Dominic P.; Molyneux, Malcolm E.; van Hensbroek, Michaël Boele

    2016-01-01

    Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool

  3. Severe Anemia in Malawian Children

    NARCIS (Netherlands)

    Calis, J.C.J.; Kamija, S.P.; Faragher, E.B.; Brabin, B.J.; Bates, I.; Cuevas, L.E.; Haan, de R.J.; Phiri, A.I.; Malange, P.; Khoka, M.; Hulshof, P.J.M.; Lieshout, L.; Beld, M.G.H.M.; Teo, Y.Y.; Rockett, K.A.; Richardson, A.; Kwiatkowski, D.P.; Molyneux, M.E.; Hensbroek, van M.B.

    2008-01-01

    Background Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. Methods We conducted a case¿control study of 381 preschool children with severe anemia (hemoglobin concentration,

  4. Hemolytic anemia in two patients with glioblastoma multiforme: A possible interaction between vorinostat and dapsone.

    Science.gov (United States)

    Lewis, Jennifer A; Petty, William J; Harmon, Michele; Peacock, James E; Valente, Kari; Owen, John; Pirmohamed, Munir; Lesser, Glenn J

    2015-06-01

    Patients undergoing treatment for glioblastoma multiforme are routinely placed on prophylactic treatment for Pneumocystis jirovecii pneumonia because of significant therapy-induced lymphopenia. In patients with sulfa allergies, dapsone prophylaxis is often used due to its efficacy, long half-life, cost effectiveness, and general safety at low doses. However, dapsone may uncommonly induce a hemolytic anemia, particularly in patients deficient of glucose-6-phosphate dehydrogenase. This hemolysis is thought to be a result of oxidative stress on red blood cells induced by dapsone metabolites which produce reactive oxygen species that disrupt the red blood cell membrane and promote splenic sequestration. A single case report of dapsone-induced hemolytic anemia in a patient with glioblastoma multiforme has been reported. We present two patients with glioblastoma multiforme who developed severe hemolytic anemia shortly after initiating therapy with vorinostat, a pan-active histone deacetylase inhibitor, while on prophylactic dapsone. There are several potential mechanisms by which histone deacetylase inhibition may alter dapsone metabolism including changes in hepatic acetylation or N-glucuronidation leading to an increase in the bioavailability of dapsone's hematotoxic metabolites. In addition, vorinostat may lead to increased hemolysis through inhibition of heat shock protein-90, a chaperone protein that maintains the integrity of the red blood cell membrane cytoskeleton. The potential interaction between dapsone and vorinostat may have important clinical implications as more than 10 clinical trials evaluating drug combinations with vorinostat in patients with malignant glioma are either ongoing or planned in North America. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  5. Abordagem ambulatorial do nutricionista em anemia hemolítica Nutritional ambulatory approach in hemolytic anemia

    Directory of Open Access Journals (Sweden)

    Maria Aparecida Vieira

    1999-04-01

    Full Text Available Descreve a atuação do nutricionista em ambulatório de Hematologia Pediátrica em um hospital escola e relata as condutas dietéticas necessárias na abordagem de crianças com anemia hemolítica com e sem sobrecarga de ferro, e também as atitudes mais freqüentes dos familiares em relação à alimentação desses pacientes.The Authors describe the performance of the Dietitian in a Pediatric Hematology Ambulatory. They emphasize the necessary dietetic procedures for adequate management of children with hemolytic anemia, with and without iron overload. Furthermore, they approach the family's attitude towards the patient's nutrition.

  6. A Rare Association of Autoimmune Hemolytic Anemia with Gastric Adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Kavita Agrawal

    2017-01-01

    Full Text Available An 80-year-old male presented with dyspnea on exertion for at least two months. He also complained of progressive dysphagia and weight loss of 35 pounds over the last eight months. Initial blood tests showed hemoglobin of 6.1 g/dl, reticulocytes count of 19.7%, total bilirubin of 3.2 mg/dl, lactate dehydrogenase of 600 U/L, and haptoglobin of less than 8 mg/dl, and direct Coombs test was positive for warm immunoglobulin G. The impression was autoimmune hemolytic anemia (AIHA. The evaluation of dysphagia with esophagogastroduodenoscopy revealed a single irregular 4 cm malignant appearing ulcerated mass at the incisura angularis of the stomach. The mass was confirmed as adenocarcinoma on biopsy. Diagnostic laparoscopy was positive for malignant cells and he was diagnosed with stage IV adenocarcinoma of the stomach. Other extensive workup to determine the etiology of AIHA was negative (described in detail below. Surgery was deferred primarily due to metastasis of cancer. Initially, hemoglobin was stabilized by intravenous methylprednisolone, high dose immunoglobulins, and packed red blood cell transfusions. After a few weeks, hemoglobin started trending down again. The patient was weaned off steroids and paradoxically IgG-mediated autohemolysis was controlled with the initiation of palliative chemotherapy. Our case highlights a rare occurrence of AIHA in association with gastric adenocarcinoma.

  7. Hemolytic anemia following high dose intravenous immunoglobulin in patients with chronic neurological disorders

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Christiansen, I; Harbo, Thomas

    2014-01-01

    High dose intravenous immunoglobulin (IVIG) is an established treatment for various neuromuscular disorders. Recently, cases of hemolytic anemia following IVIG have been observed. The objective of this study was to determine the extent of anemia and hemolysis after IVIG and its relationship...

  8. Autoimmune hemolytic anemia in a patient with Malaria

    Directory of Open Access Journals (Sweden)

    Rajesh Sonani

    2013-01-01

    Full Text Available 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.

  9. Post Blood Transfusion Hypertensive Encephalopathy in a Child with Congenital Hemolytic Anemia: A Case Report

    Directory of Open Access Journals (Sweden)

    Dhiman Arshpreet

    2017-11-01

    Full Text Available Introduction: Children having hemolytic anemias who have received multiple blood transfusions exhibit a rare complication of development of hypertension and seizures following transfusion, which may or may not be associated with intracranial hemorrhage. Case description: A 9-year-old boy presented with history of progressive paleness of body and weakness for the 30 days. There was a history of blood transfusion one week ago and multiple transfusions for one year of age. Examination revealed tachycardia, tachypnea, severe pallor and splenohepatomegaly. Blood work revealed a hemoglobin level of 4.0 grams with peripheral smear findings suggestive of hemolytic anemia. After blood transfusion, child complained of difficulty in breathing, vomiting and visual loss, followed by convulsions. Blood pressure was 180/110 mmHg. Seizure was controlled with intravenous midazolam and hypertension with furosemide and labetalol. CT brain was normal. As hypertension got under control, child gradually gained consciousness. Conclusion: A less intensive transfusion regimen among such patients along with prompt management of hypertension can prevent this potentially fatal syndrome.

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

    Directory of Open Access Journals (Sweden)

    Şinasi Özsoylu

    2013-06-01

    Full Text Available A female in the Netherlands with severe autoimmune hemolytic anemia (AIHA was treated with conventional corticosteroid (2 mg/kg/d in divided doses and blood transfusions for 18 months without improvement. The presented patient responded to megadose methylprednisolone (MDMP 30 mg/kg/d for 3 d, followed by 20 mg/kg for 4 d, and subsequently 10, 5, 2, and 1 mg/kg/d each for 1 week.

  11. Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis.

    Science.gov (United States)

    Salagre, Kaustubh D; Sahay, Ravindra Nath; Patil, Anuja; Pati, Anuja; Joshi, Amita; Shukla, Akash

    2013-10-01

    A 48 year old lady presented with jaundice and exertional breathlesness. Her laboratory reports showed anaemia, reticulocytosis, leucocytosis, elevated Lactate Dehydrogenase (LDH), alkaline phosphatase levels, hyperbillirubinemia and positive direct Coomb's test. After ruling out all the other causes of autoimmunity and hemolytic anemia, she was diagnosed as leukemoid reaction due to autoimmune hemolytic anemia with primary sclerosing cholangitis. Patient showed immediate improvement after corticosteroids.

  12. Autoimmune hemolytic anemia, as part of Evans' syndrome, caused by cold reactive IgG autoantibodies

    NARCIS (Netherlands)

    Jaarsma, AS; Muis, N; DeGraaf, SSN

    1996-01-01

    We describe a boy with Evans' syndrome, consisting of immune thrombocytopenic purpura at age 2 and autoimmune hemolytic anemia (AIHA) at age 4. AIHA was caused by cold Ige autoantibodies. This is unusual because AIHA is generally associated with either warm IgG antibodies or cold IgM antibodies.

  13. Severe Hemolytic Jaundice in a Neonate with a Novel COL4A1 Mutation.

    Science.gov (United States)

    Tomotaki, Seiichi; Mizumoto, Hiroshi; Hamabata, Takayuki; Kumakura, Akira; Shiota, Mitsutaka; Arai, Hiroshi; Haginoya, Kazuhiro; Hata, Daisuke

    2016-12-01

    We report our experience with a preterm infant with severe hemolytic jaundice who required exchange transfusion just after birth. The patient was negative for alloimmune hemolysis as a result of maternal-fetal blood type incompatibility, and tests for inherited defects in erythrocyte metabolism, membrane function, and hemoglobin synthesis were normal. We also performed a bone marrow examination, but could not identify the cause of hemolysis. The patient had several other complications, including porencephaly, epilepsy, elevated serum levels of creatine kinase, and persistent microscopic hematuria. Later, we detected a genetic mutation in COL4A1, which was recently found to be associated with hemolytic anemia. We therefore believe that all of the patient's clinical features, including hemolytic anemia, were due to the mutation in COL4A1. Genetic testing for COL4A1 mutations is recommended in neonates who exhibit hemolytic disease of unknown etiology, especially when other complications compatible with COL4A1-related disorders are present. Copyright © 2014. Published by Elsevier B.V.

  14. [The immunometaboic effects of benfotiamine and riboxine on hemolytic anemia].

    Science.gov (United States)

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

    2002-01-01

    Single (80 mg/kg) or multiply repeated (30 mg/kg) intramuscular injections of phenylhydrazine decrease the functional activity of mononuclear blood cells and the reduces immunological reactivity of the organism. Benfotiamine and riboxin decrease the extent of changes in the immunological response to a single administration of phenylhydrazine but do not significantly influence the immunological functions impaired by repeated injections of the hemolytic toxin.

  15. Severe anemia in Malawian children.

    Science.gov (United States)

    Calis, Job Cj; Phiri, Kamija S; Faragher, E Brian; Brabin, Bernard J; Bates, Imelda; Cuevas, Luis E; de Haan, Rob J; Phiri, Ajib I; Malange, Pelani; Khoka, Mirriam; Hulshof, Paul Jm; van Lieshout, Lisette; Beld, Marcel Ghm; Teo, Yik Y; Rockett, Kirk A; Richardson, Anna; Kwiatkowski, Dominic P; Molyneux, Malcolm E; van Hensbroek, Michaël Boele

    2016-09-01

    Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool children without severe anemia in urban and rural settings in Malawi. Causal factors previously associated with severe anemia were studied. The data were examined by multivariate analysis and structural equation modeling. Bacteremia (adjusted odds ratio, 5.3; 95% confidence interval [CI], 2.6 to 10.9), malaria (adjusted odds ratio, 2.3; 95% CI, 1.6 to 3.3), hookworm (adjusted odds ratio, 4.8; 95% CI, 2.0 to 11.8), human immunodeficiency virus infection (adjusted odds ratio, 2.0; 95% CI, 1.0 to 3.8), the G6PD -202/-376 genetic disorder (adjusted odds ratio, 2.4; 95% CI, 1.3 to 4.4), vitamin A deficiency (adjusted odds ratio, 2.8; 95% CI, 1.3 to 5.8), and vitamin B 12 deficiency (adjusted odds ratio, 2.2; 95% CI, 1.4 to 3.6) were associated with severe anemia. Folate deficiency, sickle cell disease, and laboratory signs of an abnormal inflammatory response were uncommon. Iron deficiency was not prevalent in case patients (adjusted odds ratio, 0.37; 95% CI, 0.22 to 0.60) and was negatively associated with bacteremia. Malaria was associated with severe anemia in the urban site (with seasonal transmission) but not in the rural site (where malaria was holoendemic). Seventy-six percent of hookworm infections were found in children under 2 years of age. There are multiple causes of severe anemia in Malawian preschool children, but folate and iron deficiencies are not prominent among them. Even in the presence of malaria parasites, additional or alternative causes of severe anemia should be considered.

  16. Report of a case with Hodgkin's lymphoma, tuberculosis and autoimmune hemolytic anemia

    OpenAIRE

    TUĞCU, Deniz; KEBUDİ, Rejin; ZÜLFİKAR, Bülent; AYAN, İnci; GÖRGÜN, Ömer; AĞAN, Mehmet; SOMER, Alper; AKINCI, Ferhan

    2007-01-01

    Tuberculosis has been described in association with malignancies including Hodgkin's disease (HD). In this article, a patient with diagnoses of H D, tuberculosis and hemolytic anemia is reported. Both tuberculosis and HD may present with similar symptoms and signs, and one of the diagnoses may be overlooked. The physicians should be aware of the simultaneous occurrence of both of these diseases when they are faced with initial therapeutic failure, during care of H D and tuberculosis patients.

  17. Diagnostic and therapeutic considerations in idiopathic hypereosinophilia with warm autoimmune hemolytic anemia

    Directory of Open Access Journals (Sweden)

    Sweidan AJ

    2015-09-01

    Full Text Available Alexander J Sweidan,1,2 Adam K Brys,3 David D Sohn,1,2 Milan R Sheth4 1University of California Los Angeles, Los Angeles, CA, USA; 2Department of Internal Medicine, St Mary Medical Center, Long Beach, CA, USA; 3School of Medicine, Duke University, Durham, NC, USA; 4Long Beach Memorial Hospital, Long Beach, CA, USA Abstract: Hypereosinophilic syndrome (HES encompasses numerous diverse conditions resulting in peripheral hypereosinophilia that cannot be explained by hypersensitivity, infection, or atopy and that is not associated with known systemic diseases with specific organ involvement. HES is often attributed to neoplastic or reactive causes, such as chronic eosinophilic leukemia, although a majority of cases remains unexplained and are considered idiopathic. Here, we review the current diagnosis and management of HES and present a unique case of profound hypereosinophilia associated with warm autoimmune hemolytic anemia requiring intensive management. This case clearly illustrates the limitations of current knowledge with respect to hypereosinophilia syndrome as well as the challenges associated with its classification and management. Keywords: hypereosinophilia, eosinophils, myeloproliferative disorder, autoimmune hemolytic anemia, idiopathic autoimmune hemolytic anemia, leukemia

  18. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl

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    Tahar Gargah

    2012-01-01

    Full Text Available The hemolytic uremic syndrome (HUS, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, represents one of the major causes of acute renal failure in infancy and childhood. The typical form occurring after an episode of diarrhea caused by Escherichia coli is the most frequent in children. Other microorganisms also may be responsible for HUS, such as Streptococcus pneumoniae, which causes more severe forms of the disease. We report an 8-month-old girl who presented with pneumonia and subsequently developed HUS. Renal biopsy showed characteristic lesion of thrombotic microangiopathy and extensive cortical necrosis. She was managed with peritoneal dialysis but did not improve and developed severe sepsis due to staphylococcal peritonitis, resulting in the death of the patient. Streptococcus pneumoniae-induced HUS is uncommon, but results in severe disease in the young. There is a high risk of these patients developing end-stage kidney disease in the long term.

  19. Hemolytic anemia after ingestion of the natural hair dye Lawsonia inermis (henna) in a dog.

    Science.gov (United States)

    Jardes, Daniel J; Ross, Linda A; Markovich, Jessica E

    2013-01-01

    To describe the clinical presentation and case management of a dog that developed hemolytic anemia and evidence of renal tubular dysfunction after ingestion of a natural hair dye containing Lawsonia inermis (henna). To review cases of henna toxicity reported in the human literature. An 8-year-old female spayed Border Collie was presented 5 days after ingestion of a box of natural hair dye. The dog was showing signs of lethargy, vomiting, diarrhea, and weakness. A serum biochemistry profile, complete blood count, and urinalysis demonstrated evidence of renal tubular dysfunction and a regenerative anemia without spherocytosis. The dog was treated with a transfusion of packed RBCs and IV fluids, resulting in significant clinical improvement. Repeat diagnostics showed resolution of the anemia and no lasting evidence of tubular dysfunction. To the authors' knowledge, this is the first reported case in the veterinary literature of toxicity following ingestion of Lawsonia inermis (henna). Henna ingestion was associated with the development of hemolytic anemia and acute kidney injury. © Veterinary Emergency and Critical Care Society 2013.

  20. Use of recombinant erythropoietin for the management of severe hemolytic disease of the newborn of a K0 phenotype mother.

    Science.gov (United States)

    Manoura, Antonia; Korakaki, Eftychia; Hatzidaki, Eleftheria; Saitakis, Emmanuel; Maraka, Sofia; Papamastoraki, Isabella; Matalliotakis, Emmanuel; Foundouli, Kaliopi; Giannakopoulou, Christine

    2007-01-01

    Very few people do not express any Kell antigens on their red blood cells (K0 phenotype). They can be immunized by transfusion or pregnancy and develop antibodies against Kell system antigens. These maternal antibodies can cause severe hemolytic disease of the fetus/newborn, as a result of the suppression of erythropoiesis and hemolysis. Multiple intrauterine transfusions in the management of severe hemolytic disease have been shown to cause erythropoietic suppression as well. Recombinant erythropoietin has been successfully used in the management of late anemia of infants with Rh hemolytic disease and in 1 case of KEL1 (Kell)-associated hemolytic disease. The authors present the case of severe hemolytic disease of a newborn due to KEL5 (Ku) isoimmunization of his K0 phenotype mother. Regular intrauterine transfusions were performed to manage the severe fetal anemia (Hb 3 g/dL). A male infant was born at the 36th week of gestation having normal hemoglobin (15.8 g/dL) and developed only mild hyperbilirubinemia. On the 15th day of life, the infant's hematocrit had fallen to 27.3%, with low reticulocyte count and low erythropoietin level. The infant was managed successfully with recombinant erythropoietin.

  1. Zinc-induced hemolytic anemia caused by ingestion of pennies by a pup

    International Nuclear Information System (INIS)

    Latimer, K.S.; Jain, A.V.; Inglesby, H.B.; Clarkson, W.D.; Johnson, G.B.

    1989-01-01

    A 4-month-old Pomeranian pup was examined because of anorexia, salivation, and persistent vomiting. Initial laboratory testing revealed marked hemolytic anemia with spherocytosis. Survey abdominal radiography revealed 4 metal objects which, when removed by gastrotomy, were identified as pennies. Of 4 pennies, 3 were minted since 1983 and were heavily pitted over the surface and rim. Partially digested pennies were composed of a copper-plated high zinc concentration alloy. Further laboratory testing indicated a marked increase in serum zinc concentration in the pup (28.8 mg/L), confirming metal toxicosis. Serum zinc concentrations decreased during recovery

  2. Hemolytic disease of the fetus and newborn with late-onset anemia due to anti-M: a case report and review of the Japanese literature.

    Science.gov (United States)

    Yasuda, Hiroyasu; Ohto, Hitoshi; Nollet, Kenneth E; Kawabata, Kinuyo; Saito, Shunnichi; Yagi, Yoshihito; Negishi, Yutaka; Ishida, Atsushi

    2014-01-01

    Hemolytic disease of the fetus and newborn (HDFN) attributed to M/N-incompatibility varies from asymptomatic to lethally hydropic. Case reports are rare, and the clinical significance of anti-M is not completely understood. A challenging case of HDFN due to anti-M prompted an investigation of the Japanese literature, in order to characterize the clinical spectrum of M/N-incompatibility pregnancies in Japan and report results to English-language readers. Japanese reports of HDFN attributed to M/N incompatibility were compiled. Abstracted data include maternal antibody titers at delivery, fetal direct antiglobulin test, hemoglobin, total bilirubin, reticulocyte count at birth, and therapeutic interventions. We investigated characteristics of HDFN due to M/N-incompatible pregnancies in Japan after encountering a case of severe HDFN along with late-onset anemia in an infant born to a woman carrying IgG anti-M with a titer of 1. In total, thirty-three babies with HDFN due to anti-M and one due to anti-N have been reported in Japan since 1975. The median maternal antibody titer was 64 at delivery and was 16 or less in 10 of 34 women (29%). Five of 34 babies (15%) were stillborn or died as neonates. Twenty-one of 29 survivors (72%) had severe hemolytic anemia and/or hydrops fetalis. The reticulocyte count of neonates with anemia stayed below the reference interval. Sixteen (55%) developed late-onset anemia and 14 (48%) were transfused with M-negative RBCs. Significant positive correlation (P hemolytic anemia and/or hydrops fetalis. Low reticulocyte count in neonates with late-onset anemia is consistent with suppressed erythropoiesis due to anti-M. © 2013.

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Satyam Arora

    2015-01-01

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

  5. Mucoepidermoid carcinoma of the lung with initial presentation of microangiopathic hemolytic anemia and thrombocytopenia

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    Yuan-Chun Huang

    2017-12-01

    Full Text Available Mucoepidermoid carcinoma is a rare entity of lung malignancy that is subclassified into high-grade or low-grade types according to its histological features. High-grade mucoepidermoid carcinoma is a more aggressive form of malignancy, with a tendency towards lymph node involvement and distant metastasis. Cancer-related microangiopathic hemolytic anemia as a less common situation of paraneoplastic syndrome may be encountered with metastatic malignancy, but has not been reported previously in mucoepidermoid carcinoma of the lung. Herein, we report a 78-year-old male patient who presented with hemoptysis for one day. Laboratory tests showed microangiopathic hemolytic anemia and thrombocytopenia. A chest X-ray demonstrated consolidation in the left lung field. Chest computed tomography revealed a mass in the left upper lobe, and a subsequent bronchoscopic biopsy was performed. The histopathological results indicated a high-grade mucoepidermoid carcinoma. Magnetic resonance imaging of the brain demonstrated leptomeningeal carcinomatosis. The patient refused systemic chemotherapy, and palliative radiation therapy only was conducted for local disease control. The patient has performed well for 12 months to date since diagnosis of the tumor.

  6. Clinical Outcomes of Splenectomy in Children: Report of the Splenectomy in Congenital Hemolytic Anemia (SICHA) Registry

    Science.gov (United States)

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

    2014-01-01

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

  7. [A case of severe hemolytic disease of the newborn due to anti-Dia antibody].

    Science.gov (United States)

    Lee, Sun Min; Im, Sun Ju; Park, Su Eun; Lee, Eun Yup; Kim, Hyung Hoi

    2007-10-01

    Here we report a severe case of hemolytic anemia of the newborn with kernicterus caused by anti-Di(a) antibody. A full term male infant was transferred due to hyperbilirubinemia on the third day of life. Despite single phototherapy, the baby's total bilirubin had elevated to 30.1 mg/dL. After exchange transfusion, total bilirubin decreased to 11.45 mg/dL. The direct antiglobulin test on the infant's red cells was positive. The maternal and infant's sera showed a negative reaction in routine antibody detection tests, but were positive in Di(a) panel cells. The frequency of the Di(a) antigen among the Korean population is estimated to be 6.4-14.5%. Anti-Di(a) antibody could cause a hemolytic reaction against transfusion or hemolytic disease of the newborn. We suggest the need for reagent red blood cell panels to include Di(a) antigen positive cells in antibody identification test for Korean.

  8. Observação de anemia hemolítica auto-imune em artrite reumatóide Observation of autoimmune hemolytic anemia in rheumatoid arthritis

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    Ricardo A. S. Souza

    2003-01-01

    Full Text Available Artrite reumatóide é uma doença difusa do tecido conjuntivo que se caracteriza pelo acometimento articular e sistêmico. Disfunções hematológicas como anemia ocorrem em até 65% dos pacientes, sendo a anemia das doenças crônicas a forma mais comum. A anemia hemolítica auto-imune pode estar associada à difusa do tecido conjuntivo, sendo classicamente associada ao lúpus eritematoso sistêmico e fazendo parte dos seus critérios de classificação. A presença de anemia hemolítica auto-imune em artrite reumatóide é relatada raramente na literatura e os mecanismos etiopatogênicos para o seu desenvolvimento ainda não estão esclarecidos. Descrevemos um caso de artrite reumatóide no adulto e outro de artrite reumatóide juvenil que desenvolveram anemia hemolítica auto-imune e discutimos os prováveis mecanismos etiopatogênicos envolvidos.Rheumatoid arthritis is a connective tissue disease characterized by articular and systemic involvement. Hematological abnormalities such as anemia may occur in up to 65% of the patients, with chronic disease anemia being the commonest form. Autoimmune hemolytic anemia can be associated with different connective tissue diseases, particularly systemic lupus erythematosus and it is part of its classification criteria. On the other hand, the presence of autoimmune hemolytic anemia in rheumatoid arthritis has rarely been described in the literature and the pathogenic mechanisms for its development remain unclear. We describe here a case of rheumatoid arthritis and another of juvenile rheumatoid arthritis that developed to autoimmune hemolytic anemia and present the probable etiopathogenic mechanisms.

  9. Microangiopathic Hemolytic Anemia Following Three Different Species of Hump-Nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka.

    Science.gov (United States)

    Namal Rathnayaka, Rathnayaka Mudiyanselage M K; Ranathunga, Anusha Nishanthi; Kularatne, Senanayake A M; Rajapakse, Jayanthe; Ranasinghe, Shirani; Jayathunga, Radha

    2018-03-01

    There are 3 species of hump-nosed pit vipers in Sri Lanka: Hypnale hypnale, Hypnale zara, and Hypnale nepa. The latter 2 are endemic to the country. Microangiopathic hemolytic anemia (MAHA) is a known complication of hump-nosed pit viper bites. It was previously documented as a complication of general viper bites and not species specific. We report a series of 3 patients who developed MAHA after being bitten by each species of hump-nosed pit viper. The first patient was bitten by H hypnale and developed a severe form of MAHA associated with acute kidney injury and thrombocytopenia falling into the category of thrombotic microangiopathy. The other 2 developed MAHA that resolved without any complications. Copyright © 2017 Wilderness Medical Society. Published by Elsevier Inc. All rights reserved.

  10. Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

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    Kim Ma

    2016-01-01

    Full Text Available Warm autoimmune hemolytic anemia (wAIHA is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA.

  11. Ceftriaxone-induced immune hemolytic anemia as a life-threatening complication of antibiotic treatment of 'chronic Lyme disease'.

    Science.gov (United States)

    De Wilde, Maarten; Speeckaert, Marijn; Callens, Rutger; Van Biesen, Wim

    2017-04-01

    'Chronic Lyme disease' is a controversial condition. As any hard evidence is lacking that unresolved systemic symptoms, following an appropriately diagnosed and treated Lyme disease, are related to a chronic infection with the tick-borne spirochaetes of the Borrelia genus, the term 'chronic Lyme disease' should be avoided and replaced by the term 'post-treatment Lyme disease syndrome.' The improper prescription of prolonged antibiotic treatments for these patients can have an impact on the community antimicrobial resistance and on the consumption of health care resources. Moreover, these treatments can be accompanied by severe complications. In this case report, we describe a life-threatening ceftriaxone-induced immune hemolytic anemia with an acute kidney injury (RIFLE-stadium F) due to a pigment-induced nephropathy in a 76-year-old woman, who was diagnosed with a so-called 'chronic Lyme disease.'

  12. 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.

    Science.gov (United States)

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

    2011-12-01

    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.

  13. A Newborn Case of “c” Subgroup Mismatch Presenting with Severe Hemolysis and Anemia

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    Ezgi Yangın Ergon

    2017-12-01

    Full Text Available Hemolysis and jaundice related to Rh incompatibility in the neonatal period has decreased substantially due to the widespread use of anti-D gammaglobulin in recent years. Nevertheless, the rate of subgroup mismatch in the etiology of hemolytic diseases of the newborn has increased significantly. In this article an 8-day-old newborn infant with “c” subgroup incompatibility and presenting with severe anemia, in whom hemolysis could be controlled with intravenous immunoglobulin infusion and subgroup appropriate blood transfusion, has been presented. Scientific studies have demonstrated that the hemolytic disease of patients who don’t have major blood group incompatibility but carry anti-C antibodies can be rather serious. Therefore, subgroup mismatch should always be kept in mind for newborns presenting with severe hemolytic anemia, and transfusion or if necessary exchange transfusion should be provided with subgroup matched blood products.

  14. Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

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    Bures Vladimir

    2005-04-01

    Full Text Available Abstract We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. Conclusion Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

  15. Anti-M Antibody Induced Prolonged Anemia Following Hemolytic Disease of the Newborn Due to Erythropoietic Suppression in 2 Siblings.

    Science.gov (United States)

    Ishida, Atsushi; Ohto, Hitoshi; Yasuda, Hiroyasu; Negishi, Yutaka; Tsuiki, Hideki; Arakawa, Takeshi; Yagi, Yoshihito; Uchimura, Daisuke; Miyazaki, Toru; Ohashi, Wataru; Takamoto, Shigeru

    2015-08-01

    Hemolytic disease of the newborn (HDN) arising from MNSs incompatibility is rare, with few reports of prolonged anemia and reticulocytopenia following HDN. We report the younger of 2 male siblings, both of whom had anti-M-induced HDN and anemia persisting for over a month. Peripheral reticulocytes remained inappropriately low for the degree of anemia, and they needed multiple red cell transfusions. Viral infections were ruled out. Corticosteroids were given for suspected pure red cell aplasia. Anemia and reticulocytopenia subsequently improved. Colony-forming unit erythroid assay revealed erythropoietic suppression of M antigen-positive erythroid precursor cells cultured with maternal or infant sera containing anti-M. In conclusion, maternal anti-M caused HDN and prolonged anemia by erythropoietic suppression in 2 siblings.

  16. Two new glucose 6-phosphate dehydrogenase variants associated with congenital nonspherocytic hemolytic anemia found in Japan: GD(-) Tokushima and GD(-) Tokyo.

    Science.gov (United States)

    Miwa, S; Ono, J; Nakashima, K; Abe, S; Kageoka, T

    1976-01-01

    Two new variants of glucose 6-phosphate dehydrogenase (G6PD) deficiency associated with chronic nonspherocytic hemolytic anemia were discovered in Japan. Gd(-) Tokushima was found in a 17-years-old male whose erythrocytes contained 4.4% of normal enzyme activity. Partially purified enzyme revealed a main band of normal electrophoretic mobility with additional two minor bands of different mobility; normal Km G6P, and Km NADP five-to sixfold higher than normal; normal utilization of 2-deoxy-G6P, galactose-6P, and deamino-NADP; marked thermal instability; a normal pH curve; and normal Ki NADPH. The hemolytic anemia was moderate to severe. Gd(-) Tokyo was characterized from a 15-year-old male who had chronic nonspherocytic hemolytic anemia of mild degree. The erythrocytes contained 3% of normal enzyme activity, and partially purified enzyme revealed slow electrophoretic mobility (90% of normal for both a tris-hydrochloride buffer system and a tris-EDTA-borate buffer system, and 70% of normal for a phosphate buffer system); normal Km G6P and Km NADP; normal utilization of 2-deoxy-G6P, galactose-6P, and deamino-NADP; greatly increased thermal instability; a normal pH curve; and normal Ki NADPH. These two variants are clearly different from hitherto described G6PD variants, including the Japanese variants Gd(-) Heian and Gd(-) Kyoto. The mothers of both Gd(-) Tokushima and Gd(-) Tokoyo were found to be heterozygote by an ascorbate-cyanide test.

  17. Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

    Directory of Open Access Journals (Sweden)

    matteo molica

    2016-04-01

    Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

  18. Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature

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    Yu Tian

    2009-08-01

    Full Text Available The association between primary biliary cirrhosis (PBC and autoimmune hemolytic anemia (AIHA is uncommon; only fourteen such case reports have been described. In this report, three patients who developed AIHA on the basis of PBC underwent successful therapy with corticosteroids and ursodeoxycholic acid (UDCA. Patient 3 was more complicated, suffering from PBC, Evans syndrome, Sjögren syndrome and Klinefelter syndrome simultaneously. This has not previously been reported in the world literature. Review of all fifteen cases showed that there is a prominent occurrence sequence that AIHA might take place on the basis of PBC. With sufficient doses of corticosteroids or immunosuppressant therapy, besides hemolysis under effective control, liver function also improved. According to the criteria of secondary AIHA, we may call them PBC-related AIHA. Thus, patients with PBC with serum bilirubin levels rising suddenly should undergo screening for associated hemolysis. Recommended treatment for PBC-related AIHA includes sufficient doses of corticosteroids to control the hemolysis in the acute phase, and immunosuppressant or adequate dose of UDCA to maintain therapy. These case reports have been increasing in recent years, so further reserch is needed to illustrate the incidence and natural courses of these two organ-specific autoimmune diseases.

  19. Characterization of autoantibodies in autoimmune hemolytic anemia following treatment with interferon alfa

    International Nuclear Information System (INIS)

    Bencomo Hernandez, Antonio; Gutierrez Diaz, Adys; Avila Cabrera, Onel; Rodriguez, Luis Ramon

    2012-01-01

    We studied 13 patients with chronic myeloid leukemia and autoimmune hemolytic anemia induced by interferon alfa. They underwent tests for immune protein detection and characterization of IgG subclasses in RBCs by direct antiglobulin test (PAD) and the microplate technique. Also they were applied ELISA test for quantifying immunoglobulins in the red blood cells. It was detected the presence of IgG and C3 in 53.84 % of cases, IgG alone in 23.07 % and in 15.38 % were identified IgG and IgA autoantibodies. In 11 patients the presence of IgG1 was showed and also in one case the subclass IgG3 autoantibodies was identified. The ELISA detected antibodies at concentrations of 183 IgG molecules per erythrocyte in a patient with negative PAD. In high-grade hemolysis patients, it was found a concentration of autoantibodies between 1 500 and 3 180 molecules of IgG per erythrocyte, while in low-grade hemolysis patients it behaved between 183 and 1 000 molecules. There was a negative correlation between Hb and plasma haptoglobin values with the number of IgG molecules per erythrocyte and a positive correlation between the latter with the reticulocyte count

  20. [Recombinant erythropoietin as treatment for hyporegenerative anemia following hemolytic disease of the newborn].

    Science.gov (United States)

    Donato, Hugo; Bacciedoni, Viviana; García, Cecilia; Schvartzman, Gabriel; Vain, Néstor

    2009-04-01

    The aim of the study is to report results of erythropoietin treatment for late hyporegenerative anemia in the hemolytic disease of the newborn (HDN). Reports previously published concern only a few cases, with controversial results. Case series report concerning 50 neonates with HDN due to Rh, ABO or KpA antigens, aged more than 7 days. Erythropoietin treatment started when hematocrit dropped to levels requiring transfusion, with an inappropriate reticulocyte response (Reticulocyte Production Index <1). At start of treatment mean age was 24.3 +/- 12.0 days (range 8-65 days), hematocrit 24.1 +/- 2.8% (range 18-30%), and Reticulocyte Production Index 0.34 +/- 0.25 (range 0.05-0.98). Hematocrit and Reticulocyte Production Index showed significant increases after 7 and 14 days of treatment (p <0.001). No difference was observed either between infants with Rh-HDN and ABO-HDN or between Rh-HDN patients with or without intrauterine transfusions. Seven infants (14%) required one packed RBC transfusion during erythropoietin therapy, 2 of them within 72 hours from starting treatment. The percentage of transfused infants showed no difference either between ABO-HDN and Rh-HDN or between Rh-HDN with and without intrauterine transfusions. Moderate, short-lasting neutropenia, not associated to infections, was observed in 11 patients. No other adverse effect was observed. The administration of erythropoietin appears to be a safe and useful therapy. Its efficacy should be confirmed by randomized studies.

  1. Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.

    Science.gov (United States)

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

    2016-01-01

    The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. A Newborn Case of “c” Subgroup Mismatch Presenting with Severe Hemolysis and Anemia

    OpenAIRE

    Ezgi Yangın Ergon; Senem Alkan Özdemir; Rüya Çolak; Kıymet Çelik; Özgür Olukman; Şebnem Çalkavur

    2017-01-01

    Hemolysis and jaundice related to Rh incompatibility in the neonatal period has decreased substantially due to the widespread use of anti-D gammaglobulin in recent years. Nevertheless, the rate of subgroup mismatch in the etiology of hemolytic diseases of the newborn has increased significantly. In this article an 8-day-old newborn infant with “c” subgroup incompatibility and presenting with severe anemia, in whom hemolysis could be controlled with intravenous immunoglobulin infusion and subg...

  3. Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

    Directory of Open Access Journals (Sweden)

    Nazan Sarper

    2011-09-01

    Full Text Available Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA. Materials and Methods: The medical records of all patients (n=19 diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed.Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years. In all, 13 patients had primary (idiopathic AIHA, whereas 2 had primary Evans Syndrome (ES, 2 had autoimmune lymphoproliferative syndrome (ALPS+ES, and 1 had Wiskott-Aldrich syndrome (WAS+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia.Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS.

  4. Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.

    Science.gov (United States)

    Fabron, A; Moreira, G; Bordin, J O

    1999-01-07

    Patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA. A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N+s+, Le(a+b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.

  5. Case report: Severe hemolytic disease of the fetus and newborn due to anti-C+G.

    Science.gov (United States)

    Jernman, Riina; Stefanovic, Vedran; Korhonen, Anu; Haimila, Katri; Sareneva, Inna; Sulin, Kati; Kuosmanen, Malla; Sainio, Susanna

    2015-01-01

    Anti-G is commonly present with anti-D and/or anti-C and can confuse serological investigations. in general, anti-G is not considered a likely cause of severe hemolytic disease of the fetus and newborn (HDFN), but it is important to differentiate it from anti-D in women who should be administered anti-D immunoglobulin prophylaxis. We report one woman with three pregnancies severely affected by anti-C+G requiring intrauterine treatment and a review of the literature. In our case, the identification of the correct antibody was delayed because the differentiation of anti-C+G and anti-D+C was not considered important during pregnancy since the father was D-. In addition, anti-C+G and anti-G titer levels were not found to be reliable as is generally considered in Rh immunization. Severe HDFN occurred at a maternal anti-C+G antibody titer of S and anti-G titer of 1 in comparison with the critical titer level of 16 or more in our laboratory. close collaboration between the immunohematology laboratory and the obstetric unit is essential. In previously affected families, early assessment for fetal anemia is required even when titers are low.

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

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    Annelieke A.A. van der Linde

    2011-12-01

    Full Text Available 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.

  7. Hemolysis of the red cell : Towards improved understanding of hereditary hemolytic anemia and new diagnostics

    NARCIS (Netherlands)

    Huisjes, Henk Rick

    2018-01-01

    The condition in which in the oxygen-carrying capacity of RBCs or their number is insufficient to meet physiological needs is characterized as anemia. Anemia is an underestimated burden of disease and despite that the vast majority of anemia is caused by iron deficiency, a substantial number of

  8. Severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia.

    Science.gov (United States)

    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy R; Xu, Min; Burroughs, Lauri M; Woolfrey, Ann E; Fleming, Mark D; Shimamura, Akiko

    2015-11-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. © 2015 Wiley Periodicals, Inc.

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

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    Sarfraz Saleemi

    2014-01-01

    Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established.

  10. A case of coombs-positive severe late anemia without hyperbilirubinemia, refractory to blood transfusion, improved with immunoglobulin

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    Supriya Kushwah

    2017-01-01

    Full Text Available Rhesus hemolytic disease of newborn is a well-known disease with early and late complications mainly manifesting as severe hyperbilirubinemia requiring prompt treatment such as exchange transfusion and immunoglobulins. We report a case of Coombs-positive severe late anemia without hyperbilirubinemia which presented with features such as sepsis and failure to gain weight. Baby was refractory to blood transfusion initially, but later on successfully improved with immunoglobulins.

  11. Late complications following total-body irradiation and bone marrow rescue in mice: predominance of glomerular nephropathy and hemolytic anemia

    International Nuclear Information System (INIS)

    Down, J.D.; Berman, A.J.; Mauch, P.; Warhol, M.

    1990-01-01

    Late mortality and pathology were assessed in various mouse strains following total-body irradiation (TBI) and bone marrow transplantation. Long-term survival data revealed both radiation dose- and strain-dependent onset of mortality between 1 and 2 years post-treatment. Renal damage appeared to have contributed to the late mortality in most treatment groups as shown by glomerular lesions, elevated blood urea nitrogen and an accompanying fall in hematocrit. Hemolysis was deduced to be the major cause of anemia, as concluded from results of 51 Cr-labeled erythrocyte survival. No decrease in erythropoiesis was evident as seen from spleen and bone marrow 59 Fe uptake. These findings are together consistent with the manifestation of a hemolytic uremic syndrome (HUS) with kidney glomeruli representing the principal sites of injury responsible for both renal dysfunction and microangiopathic hemolysis. (author)

  12. IgG4-Related Disease Combined with Autoimmune Hemolytic Anemia and Steroid-Responsive Transient Hypercalcemia

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    Sho Hasegawa

    2015-01-01

    Full Text Available A 67-year-old man with elevated serum immunoglobulin G4 (IgG4 levels, systemic lymphadenopathy infiltrated by IgG4-positive plasma cells, and Coombs-positive autoimmune hemolytic anemia (AIHA showed marked hypercalcemia. Although the intact parathyroid hormone (PTH level was elevated, 99mTc-MIBI scintigraphy and thyroid ultrasonography revealed no evidence of primary hyperparathyroidism. Liver biopsy showed marked infiltration of IgG4-positive plasma cells, which confirmed the diagnosis of IgG4-related disease (IgG4-RD. Corticosteroid therapy was initiated, and subsequently, intact PTH and serum calcium levels gradually normalized. Transient hypercalcemia in a patient with AIHA may therefore be associated with IgG4-RD.

  13. Late complications following total-body irradiation and bone marrow rescue in mice: predominance of glomerular nephropathy and hemolytic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Down, J.D.; Berman, A.J.; Mauch, P. (Harvard Medical School, Boston, MA (USA)); Warhol, M. (Pennsylvania Hospital, Philadelphia, PA (USA). Dept. of Pathology); Yeap, B. (Dana Farber Cancer Inst., Boston, MA (USA))

    1990-03-01

    Late mortality and pathology were assessed in various mouse strains following total-body irradiation (TBI) and bone marrow transplantation. Long-term survival data revealed both radiation dose- and strain-dependent onset of mortality between 1 and 2 years post-treatment. Renal damage appeared to have contributed to the late mortality in most treatment groups as shown by glomerular lesions, elevated blood urea nitrogen and an accompanying fall in hematocrit. Hemolysis was deduced to be the major cause of anemia, as concluded from results of {sup 51}Cr-labeled erythrocyte survival. No decrease in erythropoiesis was evident as seen from spleen and bone marrow {sup 59}Fe uptake. These findings are together consistent with the manifestation of a hemolytic uremic syndrome (HUS) with kidney glomeruli representing the principal sites of injury responsible for both renal dysfunction and microangiopathic hemolysis. (author).

  14. Anemia hemolítica causada por Indigofera suffruticosa (Leg. Papilionoideae em bovinos Hemolytic anemia caused by Indigofera suffruticosa (Leg. Papilionoideae in cattle

    Directory of Open Access Journals (Sweden)

    José Diomedes Barbosa Neto

    2001-03-01

    animals had hemoglobinuria, which was transitory, inspite continuation of the administration of the plant. Two animals had no further manifestations, a third animal showed only slight other manifestations, and the other three had additional symptoms of moderate intensity. These were apathy, whitish visible mucous membranes, rough hair coat, anorexia, descrease in frequency and intensity of the ruminal movements, tachycardia, positive venous pulse and dispnoea. Before the occurrence of the hemolytic crisis the urine had a bluish-green colour. None of the experimental animals died, but one was euthanized whilst showing hemoglobinuria. Post-mortem findings were anemia, the bladder containing wine-red urine, swollen dark-brown kidneys, liver on the outside and on the cut-surface bluish and with perceptible lobular design. The main histological changes were found in liver and kidney. In the liver there was coagulative necrosis and cloudy swelling and/or cytoplasmatic microvacuolization of the hepatocytes; in the kidney there was severe nephrosis, associated with large amounts of filtrate and/or hemoglobine in the Bowman spaces, in the tubules and also in the cytoplasm of the epithelial cells.

  15. [Neonatal ABO incompatibility underlies a potentially severe hemolytic disease of the newborn and requires adequate care].

    Science.gov (United States)

    Senterre, T; Minon, J-M; Rigo, J

    2011-03-01

    ABO allo-immunization is the most frequent hemolytic disease of the newborn and ABO incompatibility is present in 15-25 % of pregnancies. True ABO alloimmunization occurs in approximately one out of 150 births. Intensity is generally lower than in RhD allo-immunization. We report on three cases showing that ABO allo-immunization can lead to severe hemolytic disease of the newborn with potentially threatening hyperbilirubinemia and complications. Early diagnosis and adequate care are necessary to prevent complications in ABO incompatibility. A direct antiglobulin test is the cornerstone of diagnosis and should be performed at birth on cord blood sampling in all group infants born to O mothers, especially if of African origin. Risk factor analysis and attentive clinical monitoring during the first days of life are essential. Vigilance is even more important for infants discharged before the age of 72 h. Every newborn should be assessed for the risk of developing severe hyperbilirubinemia and should be examined by a qualified healthcare professional in the first days of life. Treatment depends on the total serum bilirubin level, which may increase very rapidly in the first 48 h of life in cases of hemolytic disease of the newborn. Phototherapy and, in severe cases, exchange transfusion are used to prevent hyperbilirubinemia encephalopathy. Intravenous immunoglobulins are used to reduce exchange transfusion. Treatments of severe hemolytic disease of the newborn should be provided and performed by trained personnel in neonatal intensive care units. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  16. [Severe hemolytic disease of the newborn as a result of late and undiagnosed alloimmunization--case report].

    Science.gov (United States)

    Drozdowska-Szymczak, Agnieszka; Czaplińska, Natalia; Borek-Dziecioł, Beata; Kociszewska-Najman, Bozena; Bartkowiak, Robert; Wielgoś, Mirosław

    2014-03-01

    We report a case of a hemolytic disease in a newborn from the first pregnancy due to anti-D antibodies. The maternal blood group was A Rhesus negative. She had an antibody screening test twice during the pregnancy (in the second trimester) and it was negative. The pregnancy was uneventful, without any invasive procedures and bleeding. The infant was born at 39 weeks of gestation in good overall condition. After the delivery the blood group of the neonate was indicated - A Rhesus positive, BOC positive. Anti-D antibodies were detected in maternal blood. Neonatal blood tests revealed severe anemia (hemoglobin level: 6.0g/dl, hematocrit: 22.2%, erythrocytes: 2.01T/L). During the first day of neonatal life, the newborn received two transfusions of red blood cells. Bilirubin level and rate of rise were not recommendation enough for exchange transfusion. The newborn was treated with continuous phototherapy since the delivery The perinatal period was complicated with intrauterine infection and respiratory failure. Hematopoietic vitamins and iron supplementation was initiated in the second week of neonatal life due to persistent anemia. The child remained under medical care of a hematologic clinic and received human recombinant erythropoietin treatment.

  17. Gastric antral vascular ectasia causing severe anemia.

    Science.gov (United States)

    Toyota, M; Hinoda, Y; Nakagawa, N; Arimura, Y; Tokuchi, S; Takaoka, A; Kitagawa, S; Usuki, T; Yabana, T; Yachi, A; Imai, K

    1996-10-01

    Gastric antral vascular ectasia (GAVE) that caused continuous gastrointestinal bleeding is reported in a 76-year-old woman who had been treated with repeated blood transfusions because of severe anemia. Endoscopic examination was performed and diffuse speckled telangiectasia of the entire antrum was observed. Laboratory data showed SGOT > SGPT, decreased chE level and the increased levels of serum gastrin and ICG at 15 min. Anti-HCV antibody was positive. Image examination revealed splenomegaly. There was no family history of telangiectasia, and no telangiectasia was found in other organs. The diagnosis was established as GAVE with liver cirrhosis. Surgical resection of the distal stomach resulted in termination of the bleeding, and the cirrhotic changes of the surface of the liver were revealed at that time, providing further evidence of liver cirrhosis. Although the pathogenesis of GAVE is unknown, liver cirrhosis and hypergastrinemia are thought to be associated with the condition. Importantly, this condition is a cause of severe gastrointestinal bleeding in elderly patients.

  18. A teenager presents with fulminant hepatic failure and acute hemolytic anemia.

    Science.gov (United States)

    Bose, Somnath; Sonny, Abraham; Rahman, Nadeem

    2015-03-01

    A teenager was admitted to an outside hospital ED following an episode of melena. He had been complaining of intermittent abdominal pain, nausea, malaise, and easy fatigability for 2 months, with significant worsening of symptoms 2 weeks prior to this episode. He had no significant medical, surgical, or family history. On presentation at the outside ED, he was found to be profoundly icteric and encephalopathic. Initial laboratories suggested anemia, acute kidney injury, and acute liver failure, leading to a presumptive diagnosis of acute fulminant liver failure necessitating transfer to our institution.

  19. Effects of therapeutic plasma exchange on serum immunoglobulin concentrations in a dog with refractory immune-mediated hemolytic anemia.

    Science.gov (United States)

    Scagnelli, Alyssa M; Walton, Stuart A; Liu, Chin-Chi; Acierno, Mark J

    2018-05-01

    CASE DESCRIPTION A 9-year-old 8.3-kg (18.3-lb) neutered male Miniature Schnauzer was referred for diagnosis and treatment of a sudden onset of lethargy, anorexia, vomiting, and pallor. CLINICAL FINDINGS On physical examination, the dog was lethargic with pale mucous membranes and a capillary refill time ≥ 2 seconds. Skin and sclera were mildly icteric. Signs of pain were elicited during abdominal palpation, and an enlarged spleen was noted. Results of agglutination testing and cytologic findings were consistent with immune-mediated hemolytic anemia (IMHA). No contributing factors for development of IMHA were identified. TREATMENT AND OUTCOME Initial treatment included management with immunosuppressant medications. Three packed RBC transfusions were administered, but clinical signs continued to progress. Therefore, therapeutic plasma exchange (TPE) was performed 5 and 9 days after admission. Following each TPE procedure, the dog had an appreciable clinical improvement and decrease in RBC autoagglutination, and the Hct stabilized. Serum IgG and IgM concentrations were measured during and after both TPE procedures. Despite anticoagulative treatment, the dog developed a thrombus in the splenic vein, necessitating a splenectomy. CLINICAL RELEVANCE The decrease and rebound in serum IgG and IgM concentrations following TPE provided evidence that TPE may have the same immunomodulatory effects in dogs as have been proposed to occur in people. Further, findings suggested that TPE may be a useful alternative in dogs with refractory IMHA when traditional treatments fail.

  20. A case of red-cell adenosine deaminase overproduction associated with hereditary hemolytic anemia found in Japan.

    Science.gov (United States)

    Miwa, S; Fujii, H; Matsumoto, N; Nakatsuji, T; Oda, S; Asano, H; Asano, S

    1978-01-01

    A case of red cell adenosine deaminase (ADA) overproduction associated with hereditary hemolytic anemia is reported here. This appears to be the second report. Proband is a 38-year-old Japanese male who had hemoglobin, 15.8 g/100 ml; reticulocyte count, 4.5%; serum indirect bilirubin, 4.9 mg/100 ml; 51Cr-labeled red cell half-life, 12 days; red cells showed moderate stomatocytosis. His red cell ADA activity showed 40-fold increase while that of the mother showed 4-fold increase. The mother was hematologically normal. The father had a normal enzyme activity. The proband and the mother showed slightly high serum uric acid levels. The proband's red cell showed: ATP, 628 nmoles/ml (normal, 1,010--1,550); adenine nucleotide pool, 46% of the normal mean; 2,3-diphosphoglycerate content, 3,782 nmoles/ml (normal 4,170--5,300); increased oxygen affinity of hemoglobin, P50 of intact erythrocytes being 21.8 mmHg (normal, 24.1--26.1). Red cell glycolytic intermediates in the proband were low in general, and the rate of lactate production was low. Kinetic studies using crude hemolysate revealed a normal Km for adenosine, normal electrophoretic mobility but slightly abnormal pH curve and slightly low utilization of 2-deoxyadenosine. The ADA activity of lymphocytes was nearly normal.

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

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    Joseph Philip

    2014-01-01

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

  2. Hemolytic anemia repressed hepcidin level without hepatocyte iron overload: lesson from Günther disease model.

    Science.gov (United States)

    Millot, Sarah; Delaby, Constance; Moulouel, Boualem; Lefebvre, Thibaud; Pilard, Nathalie; Ducrot, Nicolas; Ged, Cécile; Lettéron, Philippe; de Franceschi, Lucia; Deybach, Jean Charles; Beaumont, Carole; Gouya, Laurent; De Verneuil, Hubert; Lyoumi, Saïd; Puy, Hervé; Karim, Zoubida

    2017-02-01

    Hemolysis occurring in hematologic diseases is often associated with an iron loading anemia. This iron overload is the result of a massive outflow of hemoglobin into the bloodstream, but the mechanism of hemoglobin handling has not been fully elucidated. Here, in a congenital erythropoietic porphyria mouse model, we evaluate the impact of hemolysis and regenerative anemia on hepcidin synthesis and iron metabolism. Hemolysis was confirmed by a complete drop in haptoglobin, hemopexin and increased plasma lactate dehydrogenase, an increased red blood cell distribution width and osmotic fragility, a reduced half-life of red blood cells, and increased expression of heme oxygenase 1. The erythropoiesis-induced Fam132b was increased, hepcidin mRNA repressed, and transepithelial iron transport in isolated duodenal loops increased. Iron was mostly accumulated in liver and spleen macrophages but transferrin saturation remained within the normal range. The expression levels of hemoglobin-haptoglobin receptor CD163 and hemopexin receptor CD91 were drastically reduced in both liver and spleen, resulting in heme- and hemoglobin-derived iron elimination in urine. In the kidney, the megalin/cubilin endocytic complex, heme oxygenase 1 and the iron exporter ferroportin were induced, which is reminiscent of significant renal handling of hemoglobin-derived iron. Our results highlight ironbound hemoglobin urinary clearance mechanism and strongly suggest that, in addition to the sequestration of iron in macrophages, kidney may play a major role in protecting hepatocytes from iron overload in chronic hemolysis. Copyright© Ferrata Storti Foundation.

  3. Investigating the Antioxidant Properties of Royal Jelly and Vitamin C on Enzymes, Histomorphometric and Liver Cells Apoptosis in Mice Suffering Hemolytic Anemia

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    Hojat Anbara

    2016-09-01

    Full Text Available Background & Objective: Hemolytic anemia induced by phenylhydrazine (PHZ as a hemolytic composition can change the function and structure of liver. Therefore, the present study attempts to evaluate the protective effects of vitamin C and royal jelly co-administration against the oxidative damages and liver apoptosis induced by hemolytic anemia in adult mice. Materials & Methods: 32 adult male mice were divided equally and randomly into four groups. The first group received normal saline with a dose of 0.1 ml, IP. The second group received a dose of vitamin C (250 kg/mg, IP along with 100 kg/mg dose of royal jelly administered orally. The third group was administered with 6 mg/100 gr, IP phenylhydrazine in 48 hour intervals. Finally, the fourth group received vitamin C and royal jelly in the doses similar to the first three groups along with phenylhydrazine with the same doses of previous groups. After 35 days, the serum and testis samples were taken and were used for serum analysis and histochemical and histomorphometric studies. Results: Phenylhydrazine increased the level of serum concentration of aspartate transaminase, alkaline phosphatase, alanine transaminase, malondialdehyde and lactate dehydrogenase and decreased the superoxide dismutase along with the total antioxidant capacity and serum albumin. Moreover, phenylhydrazine increased the apoptosis, the number of kupffer cells and the diameter of hepatocytes. Prescribing the royal jelly with vitamin C improved the changes of abovementioned parameters significantly. Conclusion: Royal jelly with vitamin C is an antioxidant with the potential properties in preventing the oxidative damages and apoptosis induced by phenylhydrazine-induced hemolytic anemia in mouse liver.

  4. Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength.

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    Kizzy-Clara Cita

    Full Text Available To investigate the association between priapism in men with sickle cell anemia (SCA and hemorheological and hemolytical parameters.Fifty-eight men with SCA (median age: 38 years were included; 28 who had experienced priapism at least once during their life (priapism group and 30 who never experienced this complication (control group. Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion. Hematological and biochemical parameters were obtained through routine procedures. The Laser-assisted Optical Rotational Cell Analyzer was used to measure red blood cell (RBC deformability at 30 Pa (ektacytometry and RBC aggregation properties (laser backscatter versus time. Blood viscosity was measured at a shear rate of 225 s-1 using a cone/plate viscometer. A principal component analysis was performed on 4 hemolytic markers (i.e., lactate dehydrogenase (LDH, aspartate aminotransferase (ASAT, total bilirubin (BIL levels and reticulocyte (RET percentage to calculate a hemolytic index.Compared to the control group, patients with priapism exhibited higher ASAT (p = 0.01, LDH (p = 0.03, RET (p = 0.03 levels and hemolytic indices (p = 0.02. Higher RBC aggregates strength (p = 0.01 and lower RBC deformability (p = 0.005 were observed in patients with priapism compared to controls. After removing the hydroxycarbamide-treated patients, RBC deformability (p = 0.01 and RBC aggregate strength (p = 0.03 were still different between the two groups, and patients with priapism exhibited significantly higher hemolytic indices (p = 0.01 than controls.Our results confirm that priapism in SCA is associated with higher hemolytic rates and show for the first time that this complication is also associated with higher RBC aggregate strength and lower RBC deformability.

  5. Twin pregnancy complicated by severe hemolytic disease of the fetus and newborn due to anti-g and anti-C.

    Science.gov (United States)

    Trevett, Thomas N; Moise, Kenneth J

    2005-11-01

    Hemolytic disease of the fetus and newborn caused by anti-G antibodies is rare, and in most previously reported cases, leads to a mild anemia. The RhG antigen is usually found in association with both RhD and RhC. We report a case of a twin pregnancy affected by both anti-G and anti-C alloantibodies leading to severe hemolytic disease of the fetus and newborn requiring multiple intrauterine transfusions and prolonged postnatal therapy. A patient with a prolonged history of previously affected pregnancies due to anti-D and anti-C was subsequently found to be affected with anti-G instead. She required aggressive therapy during her pregnancy, initially with intravenous immune globulin and plasmapheresis until umbilical blood sampling and intrauterine transfusions were feasible. Although hemolytic disease of the fetus and newborn due to anti-G antibodies is rare and usually mild, these pregnancies should be followed up closely and in utero therapy should be offered if necessary.

  6. Anemia Due to Excessive Bleeding

    Science.gov (United States)

    ... Hemolytic Anemia Hemoglobin C, S-C, and E Diseases Iron Deficiency Anemia Sickle Cell Disease Thalassemias Vitamin Deficiency Anemia (See ... Hemolytic Anemia Hemoglobin C, S-C, and E Diseases Iron Deficiency Anemia Sickle Cell Disease Thalassemias Vitamin Deficiency Anemia NOTE: ...

  7. Efficacy of D- red blood cell transfusion and rituximab therapy in autoimmune hemolytic anemia with anti-D and panreactive autoantibodies arising after hematopoietic stem cell transplant.

    Science.gov (United States)

    Minakawa, Keiji; Ohto, Hitoshi; Yasuda, Hiroyasu; Saito, Shunichi; Kawabata, Kinuyo; Ogawa, Kazuei; Nollet, Kenneth E; Ikeda, Kazuhiko

    2018-04-17

    Autoimmune hemolytic anemia (AIHA) is caused by autoantibodies to red blood cells (RBCs), which can be panreactive and/or specific to Rh/other blood group antigens. We report a severe case of AIHA after bone marrow transplantation (BMT) due to autoanti-D triggered by reactivation of Epstein-Barr virus (EBV) infection. A combined strategy of D- RBC transfusion and administration of anti-CD20 monoclonal antibody (MoAb) resolved the hemolysis. A 33-year-old male underwent allogeneic BMT from an ABO-identical and HLA-matched unrelated male donor. Five months later, while having mild chronic graft-versus-host disease, he manifested AIHA, with a hemoglobin (Hb) level of 5.1 g/dL on AIHA Day 2 (Posttransplant Day 156) and was refractory to D+ RBCs, with a Hb level of 2.4 g/dL on AIHA Day 6. Anti-D-like autoantibodies (titer 1280, subclass immunoglobulin G 1 , monocyte monolayer assay 28.7%) and panreactive (titer 40) were identified. Changing the RBC transfusion strategy to D- increased his Hb level to 6.7 g/dL on Day 10. Administration of anti-CD20 MoAb mitigated EBV-related B-cell proliferation and reduced anti-D autoantibody titer to 320 by Day 16 with normalized Hb concentration after 6 months. In severe AIHA, when standard treatment and regular RBC transfusions are ineffective, transfusion of RBCs lacking the target antigen(s) of autoantibodies and administration of anti-CD20 MoAb should be considered. © 2018 AABB.

  8. Severe anemia in 3 toddlers with gastric lactobezoar.

    Science.gov (United States)

    Klein-Franke, A; Kropshofer, G; Gassner, I; Meister, B; Salvador, C; Scholl-Bürgi, S; Mueller, T; Heinz-Erian, P

    2013-05-01

    Anemia in toddlers may result from many disorders including excessive feeding with cow's milk. Another sequel of age-inadequate cow's milk nutrition may be gastric lactobezoar (GLB), a dense lump of coagulated milk and mucus in the stomach. 3 toddlers presented with a history of excessive intake of full cream cow's milk, abdominal distension, vomiting, dehydration, fatigue, marked pallor and tachycardia. Diagnostic imaging revea-led large GLBs as the likely origin of the abdominal symptoms. Laboratory evaluation showed severe anemia with depleted iron stores and signs of protein catabolism. Non-cow's milk-induced causes of anemia including defects of erythropoiesis, hemoglobin structure, RBC-enzymes and blood coagulation, hemolysis, immune disorders, infection, inflammation, extraintestinal hemorrhage, nephropathy were - according to the available data - unlikely to cause the anemia in our patients. Thus their anemia is thought to be due to age-inadequate cow's milk nutrition leading to 1) low intake, decreased absorption/bioavailability and increased intestinal loss of iron, and 2) GLB which induced blood loss following mechanical irritation of the gastric mucosa and vomiting causing high gastric pH and decrease in duodenal iron absorption. The anemia in our patients is due to both exaggerated feeding with cow's milk and adverse effects of GLBs. This hypothesis is supported by the finding that, after erythrocyte transfusion, iron substitution, age-adapted nutrition and GLB-dissolution, the anemia did not recur. We propose to include GLB in the differential diagnosis of anemia in cow's milk fed small children. © Georg Thieme Verlag KG Stuttgart · New York.

  9. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

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    An-Shine Chao

    2009-01-01

    Full Text Available We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebral artery was 0.8 m/s, indicating severe fetal anemia. Multiple intrauterine transfusions for the anemic fetus were administered. However, persistent severe fetal anemia and placentomegaly caused poor neonatal death and mirror syndrome in the mother. Uncommon red blood cell alloimmunization has to be watched for early in any population, especially in a woman with a history of unexplained perinatal loss.

  10. Ultra-performance liquid chromatography-tandem mass spectrometry-based multiplex enzyme assay for six enzymes associated with hereditary hemolytic anemia.

    Science.gov (United States)

    Park, Chul Min; Lee, Kyunghoon; Jun, Sun-Hee; Song, Sang Hoon; Song, Junghan

    2017-08-15

    Deficiencies in erythrocyte metabolic enzymes are associated with hereditary hemolytic anemia. Here, we report the development of a novel multiplex enzyme assay for six major enzymes, namely glucose-6-phosphate dehydrogenase, pyruvate kinase, pyrimidine 5'-nucleotidase, hexokinase, triosephosphate isomerase, and adenosine deaminase, deficiencies in which are implicated in erythrocyte enzymopathies. To overcome the drawbacks of traditional spectrophotometric enzyme assays, the present assay was based on ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS). The products of the six enzymes were directly measured by using ion pairing UPLC-MS/MS, and the precision, linearity, ion suppression, optimal sample amounts, and incubation times were evaluated. Eighty-three normal individuals and 13 patients with suspected enzymopathy were analyzed. The UPLC running time was within 5min. No ion suppression was observed at the retention time for the products or internal standards. We selected an optimal dilution factor and incubation time for each enzyme system. The intra- and inter-assay imprecision values (CVs) were 2.5-12.1% and 2.9-14.3%, respectively. The linearity of each system was good, with R 2 values >0.97. Patient samples showed consistently lower enzyme activities than those from normal individuals. The present ion paring UPLC-MS/MS assay enables facile and reproducible multiplex evaluation of the activity of enzymes implicated in enzymopathy-associated hemolytic anemia. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. A Fatal Case of Severe Hemolytic Disease of Newborn Associated with Anti-Jkb

    Science.gov (United States)

    Kim, Won Duck

    2006-01-01

    The Kidd blood group is clinically significant since the Jk antibodies can cause acute and delayed transfusion reactions as well as hemolytic disease of newborn (HDN). In general, HDN due to anti-Jkb incompatibility is rare and it usually displays mild clinical symptoms with a favorable prognosis. Yet, we apparently experienced the second case of HDN due to anti-Jkb with severe clinical symptoms and a fatal outcome. A female patient having the AB, Rh(D)-positive boodtype was admitted for jaundice on the fourth day after birth. At the time of admission, the patient was lethargic and exhibited high pitched crying. The laboratory data indicated a hemoglobin value of 11.4 mg/dL, a reticulocyte count of 14.9% and a total bilirubin of 46.1 mg/dL, a direct bilirubin of 1.1 mg/dL and a strong positive result (+++) on the direct Coomb's test. As a result of the identification of irregular antibody from the maternal serum, anti-Jkb was detected, which was also found in the eluate made from infant's blood. Despite the aggressive treatment with exchange transfusion and intensive phototherapy, the patient died of intractable seizure and acute renal failure on the fourth day of admission. Therefore, pediatricians should be aware of the clinical courses of hemolytic jaundice due to anti-Jkb, and they should be ready to treat this disease with active therapeutic interventions. PMID:16479082

  12. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... may require intravenous (IV) iron therapy or a blood transfusion . Iron supplements Your doctor may recommend that you ... Anemia Aplastic Anemia Arrhythmia Blood Donation Blood Tests Blood Transfusion Heart-Healthy Lifestyle Changes Heart Failure Hemolytic Anemia ...

  13. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Blood Transfusion Heart-Healthy Lifestyle Changes Heart Failure Hemolytic Anemia Hemophilia Pernicious Anemia Restless Legs Syndrome Von Willebrand Disease Other Resources NHLBI resources Your Guide to Anemia [ ...

  14. Magnitude and correlates of moderate to severe anemia among ...

    African Journals Online (AJOL)

    Introduction: Moderate to severe anemia is an important clinical problem in HIV patients on Highly Active Antiretroviral Therapy. The rate of progression and mortality in this sub group of patients is high compared to non anemic patients. In sub Saharan Africa with scale up of Anti retroviral therapy, the magnitude of this ...

  15. Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report [v1; ref status: indexed, http://f1000r.es/24q

    Directory of Open Access Journals (Sweden)

    Dino Mijatovic

    2014-03-01

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

  16. Severe hemolytic disease of the newborn in a group B African-American infant delivered by a group O mother.

    Science.gov (United States)

    Drabik-Clary, Kathryn; Reddy, Vishnu V B; Benjamin, William H; Boctor, Fouad N

    2006-01-01

    Maternal-fetal ABO incompatibility is a common hematological problem affecting the newborn. In general, hemolysis is minimal and the clinical course is relatively benign, rarely causing the escalating levels of hyperbilirubinemia and significant anemia commonly associated with Rh hemolytic disease of the newborn (HDN). The incidence of HDN ranges from one in 150 births to 1:3000 births, depending on the degree of anemia and level of serum bilirubin. The etiology of ABO hemolytic disease of the newborn (ABO-HDN) is complex because anti-A and anti-B antibodies are composed mainly of IgM. Since only IgG antibodies cross the placenta, those pregnant women with high levels of IgG anti-A,B, anti-A, or anti-B with an ABO incompatible fetus will be the ones to give birth to an infant with ABO-HDN. We describe a case of a B/Rh positive term newborn born to an O/Rh negative African-American mother demonstrating aggressive hemolysis and a robust response of the bone marrow. This case was successfully managed with phototherapy and simple RBC transfusion without the need for exchange transfusion.

  17. ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association

    Directory of Open Access Journals (Sweden)

    Naglaa A Khalifa

    2018-01-01

    Full Text Available Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS. We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case–control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014. The study included 15 consecutive children with typical HUS as well as 15 healthy children as a control group. Routine laboratory investigations were performed. Assessment of serum ADAMTS-13 level was performed using the Quantikine human ADAMTS-13 ELISA kit. Data were analyzed using Statistical Package for Social Sciences version 16. Nonparametric values were expressed as median and range, and the median of two groups was tested by Mann–Whitney test. The serum ADAMTS-13 level was significantly lower in HUS patients when compared to the control group (P < 0.05. There were significant negative correlations between ADAMTS-13 level and duration on dialysis, as well as serum urea and creatinine. Furthermore, there were significant positive correlations between serum ADAMTS-13 level and both hemoglobin level and platelet count. Our study suggests that the ADAMTS-13 level was decreased in children with severe typical HUS and its deficiency correlated with disease severity.

  18. Microangiopathic Hemolytic Anemia in 57-year-old woman with Borderline Serous Tumor of the Ovary:Real-Time Management of Common Pathways of Hemostatic Failure

    Directory of Open Access Journals (Sweden)

    Gloria Joan Morris

    2012-05-01

    Full Text Available We present a case of a 57-year-old woman who underwent surgery for the removal of an ovarian mass but subsequently experienced microangioathic hemolytic anemia post-operatively, associated with fevers, renal insufficiency, hypertension, and hemolysis. While her clinical situations was initially suspicious for thrombotic thrombocytopenic purpura (TTP, further sorting of clinical information led to other explanations of these findings, including a systemic inflammatory response. Multiple triggers of the coagulation system which can lead to a common pathway of hemostatic failure were considered, and specific criteria seen in disseminated intravascular coagulation (DIC, TTP, heparin-induced thrombocytopenia (HIT, catastrophic antiphospholipid anitbody syndrom (APS, all of which can seem to overlap when a physician is faced with distinguishing the diagnosis clinically. We propose a chronologic and strategic approach for the clinician to consider when approaching this diagnostic dilemma.

  19. A Coincidental Discovery of a New Stable Variant (Hb Hachioji or HBB: c.187C>T) in a Patient with Chronic Hemolytic Anemia of Unexplained Origin.

    Science.gov (United States)

    Mella, Ferania; Yamashiro, Yasuhiro; Adhiyanto, Chris; Tanaka, Tatehiko; Nitta, Takenori; Amao, Yuki; Kimoto, Masafumi

    2018-01-01

    We report a new hemoglobin (Hb) variant, Hb Hachioji (HBB: c.187C>T), which was detected in a 32-year-old male with hemolytic anemia. The proband had undergone splenectomy in his childhood after being diagnosed with hereditary spherocytosis (HS) with no clinical improvement. A recent study showed that Heinz bodies were frequently observed in his red cells, however, no abnormal band was separated by isoelectric focusing (IEF), and the isopropanol (instability) test was negative. Direct sequencing revealed that the proband was a heterozygous carrier of a novel mutation (GCT>GTT) at codon 62 of the β-globin gene, leading to an alanine to valine substitution. This variant was named Hb Hachioji. Characterization at the mRNA level by cDNA sequencing detected β Hachioji mRNA, as well as β A mRNA. Subsequently, study of the proband's family indicated that his father was a carrier of this Hb variant, although unexpectedly, the father was asymptomatic and clinically healthy. Oxygen affinity measurement of total Hb showed no alteration in the P 50 and oxygen equilibrium curve. The presence of Hb Hachioji was confirmed by mass spectrometry (MS). Hb Hachioji comprised approximately 50.0% of the total Hb and was a stable variant. The phenotypic discrepancy between these two carriers suggests that Hb Hachioji may not be associated with the hemolytic involvement in the proband. P4.2Nippon, which is the primary cause of most cases of Japanese HS, was absent in the proband's parents. The coexistence of glucose-6-phosphate dehydrogenase (G6PD) deficiency was ruled out. Thus, the cause of hemolytic involvement in this patient remains unclear.

  20. The Prevalence of Anemia and Moderate-Severe Anemia in the US Population (NHANES 2003-2012)

    Science.gov (United States)

    2016-01-01

    Since anemia is associated with poor health outcomes, the prevalence of anemia is a significant public health indicator. Even though anemia is primarily caused by iron deficiency, low oxygen-carrying capacity may result from other conditions such as chronic diseases, which remain a relevant health concern in the United States. However, studies examining current rates of anemia in the total US population and in more specific subgroups are limited. Data from five National Health and Nutrition Examination Surveys (NHANES) from 2003 to 2012 were analyzed to assess two outcomes: anemia and moderate-severe anemia, which were based upon serum hemoglobin levels (Hb) as per World Health Organization (WHO) definitions. Statistical analysis using SAS examined temporal trends and the prevalence of anemia among sexes, age groups, and races/ethnicities. The study estimated that an average of 5.6% of the U.S. population met the criteria for anemia and 1.5% for moderate-severe anemia during this 10-year period. High-risk groups such as pregnant women, elderly persons, women of reproductive age, non-Hispanic blacks, and Hispanics were identified, and relationships between multiple risk factors were examined. Rates of anemia in men increased monotonically with age, while that of women increased bimodally with peaks in age group 40–49 years and 80–85 years. The effect of risk factors was observed to compound. For instance, the prevalence of anemia in black women aged 80–85 years was 35.6%, 6.4 times higher than the population average. Moreover, anemia is a growing problem because of the increased prevalence of anemia (4.0% to 7.1%) and moderate-severe anemia (1.0% to 1.9%), which nearly doubled from 2003–2004 to 2011–2012. Thus, these results augment the current knowledge on anemia prevalence, severity, and distribution among subgroups in the US and raised anemia as an issue that requires urgent public health intervention. PMID:27846276

  1. MRI evaluation of cranial bone marrow signal intensity and thickness in chronic anemia

    International Nuclear Information System (INIS)

    Yildirim, Tulin; Agildere, A. Muhtesem; Oguzkurt, Levent; Barutcu, Ozlem; Kizilkilic, Osman; Kocak, Rikkat; Alp Niron, Emin

    2005-01-01

    Background and purpose: The aim is to assess the magnetic resonance imaging (MRI) findings for cranial bone marrow (CBM) signal intensity and thickness in patients with chronic anemia and compared these with findings in healthy subjects. We also investigated the relationships between CBM changes and age, type of anemia (hemolytic versus non-hemolytic), and severity of anemia. Methods: We quantitatively evaluated CBM signal intensity and thickness on images from 40 patients with chronic anemia (20 with congenital hemolytic anemia (HA) and 20 with acquired anemia) and compared these to findings in 28 healthy subjects. The intensity of CBM relative to scalp, white matter (WM), gray matter (GM), and muscle intensity was also investigated in patients and subjects in the control group. The sensitivity and specificity of CBM hypointense to GM and CBM hypointense to WM as markers of anemia were evaluated. Relationships between age and CBM thickness/intensity, and between anemia severity (hemoglobin (Hb) level) and CBM thickness/intensity were evaluated. Results: Cranial bone marrow signal intensity was lower in the chronic anemia patients than in the controls (P 0.05 for both). There were no correlations between age and CBM intensity or thickness, or between anemia severity and CBM intensity or thickness. Conclusion: Patients with chronic anemia exhibit lower CBM signal intensity on MRI than healthy subjects. Patients with hemolytic anemia have thicker CBM than patients with non-hemolytic anemia or healthy individuals. Decreased CBM intensity may indicate that the patient has anemia, and increased CBM thickness may specifically point to hemolytic anemia. These MRI findings may signal the need for further evaluation for the clinician

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

    Directory of Open Access Journals (Sweden)

    Peyman Naji

    2013-01-01

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

  3. Púrpura trombocitopênica e anemia hemolítica auto-imune em pacientes internados com lúpus eritematoso sistêmico juvenil Trombocytopenic purpura and autoimmune hemolytic anemia in hospitalized patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Jochebed Kyoung Kim

    2007-02-01

    esplenectomia. CONCLUSÕES: o CHE isolado foi uma manifestação grave em pacientes internados com LESJ, habitualmente associado a uma doença ativa e sistêmica.OBJECTIVE: to evaluate the hematological involvement (HI in hospitalized patients with juvenile systemic lupus erythematosus (JSLE. METHODS: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança - University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl, reticulocytosis, increase in lactate dehydrogenase (LDH and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. RESULTS: HI occurred in 14 patients (18.9%, with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis. All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. CONCLUSIONS: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally

  4. Anemia hemolítica auto-imune e outras manifestações imunes da leucemia linfocítica crônica Autoimmune hemolytic anemia and other autoimmune diseases related to chronic lymphocytic leukemia

    Directory of Open Access Journals (Sweden)

    José O. Bordin

    2005-12-01

    Full Text Available A leucemia linfocítica crônica (LLC é freqüentemente associada a manifestações auto-imunes principalmente relacionadas às células do sistema hematopoético causando anemia hemolítica auto-imune (AHAI, púrpura trombocitopênica imune (PTI, aplasia pura de série vermelha (APSV, e neutropenia imune. A LLC é diagnosticada em até 15% dos pacientes com AHAI, e em cerca de 50% dos pacientes com AHAI secundária a doença maligna. A PTI ocorre em 2%, e a APSV em 1% dos pacientes com LLC. Prednisona é o tratamento inicial de escolha para a citopenia imune associada à LLC. Para cerca de 60% dos pacientes que apresentam recidiva da manifestação auto-imune tem sido utilizada esplenectomia, imunoglobulina endovenosa, ou ciclosporina. Embora as evidências sobre fisiopatologia sejam limitadas, os mecanismos fisiopatológicos da auto-imunidade na LLC estão relacionados à atividade dos linfócitos B leucêmicos que atuam como células apresentadoras de antígeno aberrantes, e são eficientes em processar e apresentar proteínas da membrana de hemácias e de plaquetas às células TH auto-reativas. Linfócitos TH específicos para certos auto-antígenos podem escapar de mecanismos de controle de auto-tolerância, e, se ativados, podem causar doença auto-imune. O diagnóstico de AHAI contra-indica o uso de fludarabina em pacientes com LLC, pois esse análogo da purina tem sido associado ao desenvolvimento de AHAI grave e fatal, com risco consideravelmente mais alto para pacientes mais imunossuprimidos devido a vários tratamentos anteriores.Chronic lymphocytic leukemia (CLL is frequently associated with autoimmune diseases directed against hematopoietic cells, including autoimmune hemolytic anemia (AIHA, immune thrombocytopenic purpura (ITP, pure red cell aplasia (PRCA, and immune neutropenia. CLL represents the diagnosis in up to 15% of the patients with AIHA, and in 50% of the patients with AIHA secondary to malignancy. ITP occurs in 2% and

  5. Severe iron-deficiency anemia still an issue in toddlers.

    Science.gov (United States)

    Paoletti, Gabrielle; Bogen, Debra L; Ritchey, A Kim

    2014-12-01

    Chronic, severe iron-deficiency anemia (IDA) in the first years of life increases the risk of irreversibly compromised cognitive, affective, and motor development. While IDA in infants has decreased because of dietary changes (iron-fortified formula and delaying cow's milk), toddlers (13-36 months) are equally vulnerable to the adverse effects of IDA. We aimed to show that despite public health efforts, severe IDA remains a problem in toddlers and is associated with excess milk consumption. Retrospective chart review of children 6 to 36 months admitted to or evaluated by hematology at a children's hospital from January 1, 2005 to December 31, 2010 with a severe microcytic anemia (hemoglobin [Hb] appetite, and pica were the most common symptoms, found in 43%, 29%, and 22% of patients, respectively. Only 41% of parents reported pale skin while 77% of physicians recorded it on physical exam. Daily cow's milk consumption surpassed 24 ounces for 47 of 48 children with reported intake; 11 consumed more than 64 ounces per day. Despite current screening recommendations, severe IDA continues to be a problem in toddlers and strongly correlates with excess cow's milk consumption. This reiterates the importance of screening for IDA into routine toddler care. © The Author(s) 2014.

  6. [Hereditary hemorrhagic telangiectasia presenting with hematuria and severe anemia].

    Science.gov (United States)

    Paz, A; Goren, E; Segal, M

    1995-07-01

    A patient with hereditary hemorrhagic telangiectasia was admitted with hematuria and severe anemia after mild recurrent episodes of epistaxis. Telangiectasias were found in the skin and buccal and nasal mucosa. No defect in the coagulation mechanism was found; thrombocyte count and function were normal. On cystoscopy, tortuous engorged vessels, some actively bleeding, were seen in the trigonal mucosa. Biopsy showed enlarged vessels in the lamina propria. Electrocoagulation of the bleeding vessels stopped hematuria, but 6 months later it recurred. This time Nd-YAG laser was used to stop the bleeding after electrocoagulation was ineffective.

  7. Severe hemolytic disease of the newborn due to anti-Di b treated with phototherapy and intravenous immunoglobulin.

    Science.gov (United States)

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

    2008-01-01

    The Di(b) antigen usually occurs with high incidence, except in certain Asian and South American Indian populations. In general, hemolysis caused by anti-Di(b) is not severe and its clinical course is benign. We report a Korean neonate with severe hemolytic disease of the newborn caused by anti-Di(b). The phenotype and genotype of the Diego blood group system of the patient and his mother were Di(a+b+) and Di(a+b-), respectively. The mother's serum and eluate from the neonate's erythrocytes contained anti-Di(b). This case was successfully managed with phototherapy and high dose iv immunoglobulin. Since most commercial antibody detection panels do not contain Di(b-) red cells, it is important to consider anti-Di(b) in cases of hemolytic disease of the newborn caused by an antibody against a high frequency antigen.

  8. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... heavy menstrual periods. Individuals with a gene for hemophilia, including symptomatic female carriers who have heavy menstrual ... Heart-Healthy Lifestyle Changes Heart Failure Hemolytic Anemia Hemophilia Pernicious Anemia Restless Legs Syndrome Von Willebrand Disease ...

  9. Anemia in the Newborn

    Science.gov (United States)

    ... Overview of Horseshoe Kidney Additional Content Medical News Anemia in the Newborn By Andrew W. Walter, MS ... for the Professional Version Blood Problems in Newborns Anemia in the Newborn Hemolytic Disease of the Newborn ...

  10. Prevalence, severity, and related factors of anemia in HIV/AIDS patients

    Directory of Open Access Journals (Sweden)

    Mohsen Meidani

    2012-01-01

    Full Text Available Objective: The prevalence of anemia in HIV infected patients has not been well characterized in Iran. This study aimed to describe the prevalence of anemia and related factors in HIV positive patients. Materials and Methods: In a cross-sectional study, anemia prevalence and risk factors of 212 HIV positive patients were assessed, at the behavioral disease consulting center in Isfahan. The relationship between anemia, demographic variables, and clinical histories were analyzed. Mild to moderate anemia was defined as hemoglobin 8-13 g/dL for men and 8-12 g/dL for women. Severe anemia was defined as hemoglobin, 8 g/dL. Results: A total of 212 HIV positive patients with a mean±SD age of 36.1 ± 9.1 years were assessed. We found that hemoglobin levels were between 4.7 and 16.5 gr/dL. In this study, the overall prevalence of anemia was 71%, with the majority of patients having mild to moderate anemia. Mild to moderate anemia and severe anemia occurred in 67% and 4% of patients, respectively. The mean absolute CD4 count was 348 ± 267.8 cells/cubic mm. Sixty one of 212 patients were at late stage of HIV infection (males=51 and female=10. Of the 212 HIV positive patients enrolled, 17 (8% had a positive history of tuberculosis. We found a strong association between anemia and death. Conclusion: Normocytic anemia with decreased reticulocyte count was the most common type of anemia in overall. Prevalence of anemia in this study is relatively higher than other similar studies. Such a high prevalence of anemia needs close monitoring of patients on a zidovudine-based regimen. Better screening for anemia and infectious diseases, and modified harm reduction strategy (HRS for injection drug users are primary needs in HIV seropositive patients.

  11. Microangiopathic Hemolytic Anemia in 57-year-old woman with Borderline Serous Tumor of the Ovary:Real-Time Management of Common Pathways of Hemostatic Failure

    Directory of Open Access Journals (Sweden)

    Gloria Joan Morris

    2012-01-01

    Full Text Available

    We present a case of a 57-year-old woman who underwent surgery for the removal of an ovarian mass but subsequently experienced microangioathic hemolytic anemia post-operatively, associated with fevers, renal insufficiency, hypertension, and hemolysis. While her clinical situations was initially suspicious for thrombotic thrombocytopenic purpura (TTP, further sorting of clinical information led to other explanations of these findings, including a systemic inflammatory response. Multiple triggers of the coagulation system which can lead to a common pathway of hemostatic failure were considered, and specific criteria seen in disseminated intravascular coagulation (DIC, TTP, heparin-induced thrombocytopenia (HIT, catastrophic antiphospholipid anitbody syndrom (APS, all of which can seem to overlap when a physician is faced with distinguishing the diagnosis clinically. We propose a chronologic and strategic approach for the clinician to consider when approaching this diagnostic dilemma.

  12. Hemolytic crisis

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/003270.htm Hemolytic crisis To use the sharing features on this page, please enable JavaScript. Hemolytic crisis occurs when large numbers of red blood cells ...

  13. Prevalence and severity of anemia among pregnant women in primary health centers/Erbil City

    Directory of Open Access Journals (Sweden)

    Rushna Ghazi Abdulwahid

    2017-07-01

    Full Text Available Anemia in pregnancy is a major public health problem, especially in developing countries. Nutritional anemia is the most common type of anemia worldwide and mainly includes iron, folic acid, vitamin B12 and vitamin C deficiencies. Iron deficiency contributes to half of the burden of anemia globally. The objectives of this study were to find out the prevalence and severity of anemia among pregnant women. A descriptive study was conducted on 600 pregnant women, during the period October 2015 to November 2016 at four primary health care centers in Erbil city. Data gathered through interview with study sample and filling questionnaire format and reviewing their antenatal records. Blood hemoglobin level was measured to assess the severity of anemia. Chi-square test and regression were used to analyze the data. Prevalence of anemia was 46.2%, and 67.1% of the anemic women had mild anemia. There was a significant association of severity of anemia with wife education and occupation, smoking, the number of abortions and cesarean section, ante partum hemorrhage, taking tablets of iron and folic acid per month. The study concluded that prevalence of anemia considered high in Erbil city, the highest percentage had mild anemia.

  14. Anemia

    Science.gov (United States)

    ... child might have anemia. They will do a physical exam and review your health history and symptoms. To diagnose anemia, your doctor ... and Wellness Staying Healthy Healthy Living Travel Occupational Health First Aid and ... Pets and Animals myhealthfinder Food and Nutrition Healthy Food ...

  15. Identification of Stages of Erythroid Differentiation in Bone Marrow and Erythrocyte Subpopulations in Blood Circulation that Are Preferentially Lost in Autoimmune Hemolytic Anemia in Mouse.

    Directory of Open Access Journals (Sweden)

    Sreoshi Chatterjee

    Full Text Available Repeated weekly injections of rat erythrocytes produced autoimmune hemolytic anemia (AIHA in C57BL/6 mice after 5-6 weeks. Using the double in vivo biotinylation (DIB technique, recently developed in our laboratory, turnover of erythrocyte cohorts of different age groups during AIHA was monitored. Results indicate a significant decline in the proportion of reticulocytes, young and intermediate age groups of erythrocytes, but a significant increase in the proportion of old erythrocytes in blood circulation. Binding of the autoantibody was relatively higher to the young erythrocytes and higher levels of intracellular reactive oxygen species (ROS were also seen in these cells. Erythropoietic activity in the bone marrows and the spleen of AIHA induced mice was examined by monitoring the relative proportion of erythroid cells at various stages of differentiation in these organs. Cells at different stages of differentiation were enumerated flow cytometrically by double staining with anti-Ter119 and anti-transferrin receptor (CD71 monoclonal antibodies. Erythroid cells in bone marrow declined significantly in AIHA induced mice, erythroblast C being most affected (50% decline. Erythroblast C also recorded high intracellular ROS level along with increased levels of membrane-bound autoantibody. No such decline was observed in spleen. A model of AIHA has been proposed indicating that binding of autoantibodies may not be a sufficient condition for destruction of erythroid cells in bone marrow and in blood circulation. Last stage of erythropoietic differentiation in bone marrow and early stages of erythrocytes in blood circulation are specifically susceptible to removal in AIHA.

  16. A case of fetal intestinal volvulus without malrotation causing severe anemia.

    Science.gov (United States)

    Nakagawa, Tomoko; Tachibana, Daisuke; Kitada, Kohei; Kurihara, Yasushi; Terada, Hiroyuki; Koyama, Masayasu; Sakae, Yukari; Morotomi, Yoshiki; Nomura, Shiho; Saito, Mika

    2015-01-01

    Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

  17. A Case of Fetal Intestinal Volvulus without Malrotation Causing Severe Anemia

    Directory of Open Access Journals (Sweden)

    Tomoko Nakagawa

    2015-01-01

    Full Text Available Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

  18. Evaluation of severity in aplastic anemia by MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jeong Mi; Lim, Gye Yeon; Kim, Euy Neyng; Lee, Jae Mun; Kim, Dong Wook; Han, Chi Wha; Kim, Chun Choo [The Catholic University College of Medicine, Seoul (Korea, Republic of)

    1999-02-01

    To evaluate the role of bone marrow (BM) magnetic resonance (MR) imaging for assessment of the severity of aplastic anemia (AA). Eighty patients with AA, ranging in age from 16 to 44 years underwent MR imaging. Fifty four patients had clinically severe AA(SAA), while in 26 the condition was moderate(MAA). Sagittal T1-weighted images (T1WI) and short tau inversion recovery (STIR) images of lumbar vertebral BM were analysed. Bulk T1, T2 and rho values (msec) were also measured, with mixed sequences. Signal intensity (SI) on both T1WI and STIR was classified into four patterns according to the amount fatty marrow : pattern I, homogeneous fatty marrow ; 2, fatty marrow with focal cellular nodules ; 3, mixed fatty and cellular marrow ; 4, cellular marrow with focal fatty nodules. These SI patterns and bulk T1, T2 and rho values of the lumbar BM were compared with the clinical severity of AA. On both T1WI and STIR sequences, MR imaging of lumbar vertebral BM in patients with AA showed various SI patterns. Pattern 1, 2 and 3 were much frequently seen in the SAA group (48 of 54 patients on T1WI and 43 of 54 on STIR) and pattern 4 was common in the MAA group (16 of 26 patients on T1WI and 18 of 26 on STIR). The SI patterns of AA seen on both T1WI and STIR sequences closely correlated with clinical severity (x 2 test, p=0.0001). Bulk T1 value was significantly different between SAA and MAA (SAA : 382.82msec {+-} 113.91 ; MAA : 517.99msec {+-} 151.92 ; t test, p=0.0001). The SI pattern seen on MR imaging, and T1 relaxation time of lumbar spinal BM can be useful for assessing the severity of AA.

  19. Evaluation of severity in aplastic anemia by MR imaging

    International Nuclear Information System (INIS)

    Park, Jeong Mi; Lim, Gye Yeon; Kim, Euy Neyng; Lee, Jae Mun; Kim, Dong Wook; Han, Chi Wha; Kim, Chun Choo

    1999-01-01

    To evaluate the role of bone marrow (BM) magnetic resonance (MR) imaging for assessment of the severity of aplastic anemia (AA). Eighty patients with AA, ranging in age from 16 to 44 years underwent MR imaging. Fifty four patients had clinically severe AA(SAA), while in 26 the condition was moderate(MAA). Sagittal T1-weighted images (T1WI) and short tau inversion recovery (STIR) images of lumbar vertebral BM were analysed. Bulk T1, T2 and rho values (msec) were also measured, with mixed sequences. Signal intensity (SI) on both T1WI and STIR was classified into four patterns according to the amount fatty marrow : pattern I, homogeneous fatty marrow ; 2, fatty marrow with focal cellular nodules ; 3, mixed fatty and cellular marrow ; 4, cellular marrow with focal fatty nodules. These SI patterns and bulk T1, T2 and rho values of the lumbar BM were compared with the clinical severity of AA. On both T1WI and STIR sequences, MR imaging of lumbar vertebral BM in patients with AA showed various SI patterns. Pattern 1, 2 and 3 were much frequently seen in the SAA group (48 of 54 patients on T1WI and 43 of 54 on STIR) and pattern 4 was common in the MAA group (16 of 26 patients on T1WI and 18 of 26 on STIR). The SI patterns of AA seen on both T1WI and STIR sequences closely correlated with clinical severity (x 2 test, p=0.0001). Bulk T1 value was significantly different between SAA and MAA (SAA : 382.82msec ± 113.91 ; MAA : 517.99msec ± 151.92 ; t test, p=0.0001). The SI pattern seen on MR imaging, and T1 relaxation time of lumbar spinal BM can be useful for assessing the severity of AA

  20. Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report.

    Science.gov (United States)

    Balaban, Vasile Daniel; Popp, Alina; Grasu, Mugur; Vasilescu, Florina; Jinga, Mariana

    2015-09-01

    Primary intestinal lymphangiectasia (Waldmann's disease) is a rare disease characterized by dilated lymphatics in the small bowel leading to an exudative enteropathy with lymphopenia, hypoalbuminemia and hypogammaglobulinemia. We report the case of a 23 year-old male who presented with chronic anemia and in whom primary intestinal lymphangiectasia was diagnosed. A low-fat diet along with nutritional therapy with medium-chain triglyceride supplementation improved the protein-losing enteropathy, but did not solve the anemia. Octreotide was also unsuccessful, and after attempting angiographic embolization therapy, limited small bowel resection together with antiplasmin therapy managed to correct the anemia and control the exudative enteropathy. Although primary intestinal lymphangiectasia is usually adequately managed by nutritional therapy, complications such as anemia can occur and can prove to be a therapeutic challenge.

  1. Evolving Hematopoietic Stem Cell Transplantation Strategies in Severe Aplastic Anemia

    Science.gov (United States)

    Dietz, Andrew C.; Lucchini, Giovanna; Samarasinghe, Sujith; Pulsipher, Michael A.

    2016-01-01

    Purpose of Review Significant improvements in unrelated donor hematopoietic stem cell transplantation (HSCT) in recent years has solidified its therapeutic role in severe aplastic anemia (SAA) and led to evolution of treatment algorithms, particularly for children. Recent Findings Advances in understanding genetics of inherited bone marrow failure syndromes (IBMFS) have allowed more confidence in accurately diagnosing SAA and avoiding treatments that could be dangerous and ineffective in individuals with IBMFS, which can be diagnosed in 10–20% of children presenting with a picture of SAA. Additionally long-term survival after matched sibling donor (MSD) and matched unrelated donor (MUD) HSCT now exceed 90% in children. Late effects after HSCT for SAA are minimal with current strategies and compare favorably to late effects after up-front immunosuppressive therapy (IST), except for patients with chronic graft versus host disease (GVHD). Summary 1) Careful assessment for signs or symptoms of IBMFS along with genetic screening for these disorders is of major importance. 2) MSD HSCT is already considered standard of care for up-front therapy and some groups are evaluating MUD HSCT as primary therapy. 3) Ongoing studies will continue to challenge treatment algorithms and may lead to an even more expanded role for HSCT in SAA. PMID:26626557

  2. The Fanconi anemia pathway limits the severity of mutagenesis.

    Science.gov (United States)

    Hinz, John M; Nham, Peter B; Salazar, Edmund P; Thompson, Larry H

    2006-08-13

    Fanconi anemia (FA) is a developmental and cancer predisposition disorder in which key, yet unknown, physiological events promoting chromosome stability are compromised. FA cells exhibit excess metaphase chromatid breaks and are universally hypersensitive to DNA interstrand crosslinking agents. Published mutagenesis data from single-gene mutation assays show both increased and decreased mutation frequencies in FA cells. In this review we discuss the data from the literature and from our isogenic fancg knockout hamster CHO cells, and interpret these data within the framework of a molecular model that accommodates these seemingly divergent observations. In FA cells, reduced rates of recovery of viable X-linked hypoxanthine phosphoribosyltransferase (hprt) mutants are characteristically observed for diverse mutagenic agents, but also in untreated cultures, indicating the relevance of the FA pathway for processing assorted DNA lesions. We ascribe these reductions to: (1) impaired mutagenic translesion synthesis within hprt during DNA replication and (2) lethality of mutant cells following replication fork breakage on the X chromosome, caused by unrepaired double-strand breaks or large deletions/translocations encompassing essential genes flanking hprt. These findings, along with studies showing increased spontaneous mutability of FA cells at two autosomal loci, support a model in which FA proteins promote both translesion synthesis at replication-blocking lesions and repair of broken replication forks by homologous recombination and DNA end joining. The essence of this model is that the FANC protein pathway serves to restrict the severity of mutational outcome by favoring base substitutions and small deletions over larger deletions and chromosomal rearrangements.

  3. Anticardiolipin antibodies in D+ hemolytic uremic syndrome.

    NARCIS (Netherlands)

    Loo, D.M.W.M. te; Alfen-van der Velden, J. van; Onland, W.; Heuvel, L.P.W.J. van den; Monnens, L.A.H.

    2002-01-01

    The diarrhea-associated form of the hemolytic uremic syndrome (D+ HUS) is characterized by a triad of symptoms, namely thrombocytopenia, hemolytic anemia, and acute renal failure. Histopathological studies of patients with D+ HUS show microthrombi in arterioles and glomeruli of the kidney. Recently,

  4. Diagnosis and treatment of severe hemolytic disease of the fetus and newborn: a 10-year nationwide retrospective study.

    Science.gov (United States)

    Sainio, Susanna; Nupponen, Irmeli; Kuosmanen, Malla; Aitokallio-Tallberg, Ansa; Ekholm, Eeva; Halmesmäki, Erja; Orden, Maija-Riitta; Palo, Pertti; Raudaskoski, Tytti; Tekay, Aydin; Tuimala, Jarno; Uotila, Jukka; Stefanovic, Vedran

    2015-04-01

    Outcome after intrauterine transfusions due to severe hemolytic disease of the fetus and newborn. Nationwide population-based retrospective cohort study. All women treated with intrauterine transfusions for hemolytic disease of the fetus and newborn in Finland in 2003-2012. 339 intrauterine transfusions, performed in 104 pregnancies of 84 women. Information on antenatal screening of red cell antibodies and red cell units issued for intrauterine transfusion was obtained from the Finnish Red Cross Blood Service database, and obstetric and neonatal data from hospital records. Procedure-related complications, perinatal mortality, neonatal morbidity. Overall survival was 94.2% (95% confidence interval 89.7-98.7). There were four fetal and two neonatal deaths. Procedure-related fetal loss rate was 1.2% (95% confidence interval 0.04-2.4) per procedure and 3.8% (95% confidence interval 0.1-7.5) per pregnancy. Of the four procedure-related losses, three were due to technically difficult intrauterine transfusions causing infection and preterm birth. Of the live born infants, 19% (95% confidence interval 11.3-26.7) were born before 32 weeks' gestation. The incidence of severe neonatal morbidity (respiratory distress syndrome, severe cerebral injury, sepsis) was 22.2% (95% confidence interval 13.4-30.2). Poor outcome (death, severe neonatal morbidity) was negatively associated with gestational age at first transfusion (p = 0.001) and at birth (p = 0.00006). Follow-up of the infants was too incomplete to assess the neurodevelopmental outcome. Although overall survival is comparable with previous studies, our concern is procedure-related infections and preterm births. Close collaboration between the university hospitals is needed to ensure timely treatment, operator skills and systematic follow-up of the children. © 2015 Nordic Federation of Societies of Obstetrics and Gynecology.

  5. Transforming growth factor 15 increased in severe aplastic anemia patients.

    Science.gov (United States)

    Shao, Yuanyuan; Wang, Honglei; Liu, Chunyan; Cao, Qiuying; Fu, Rong; Wang, Huaquan; Wang, Ting; Qi, Weiwei; Shao, Zonghong

    2017-10-01

    The patients with severe aplastic anemia (SAA) usually rely on red cell transfusion which lead to secondary iron overload. Transforming growth differentiation factor-15 (GDF-15) plays an important role in erythropoiesis and iron regulation. In this study, we investigated the level of GDF-15 and other indexes of iron metabolism in SAA patients to explore the correlation with GDF-15 and iron overload in SAA. The levels of serum GDF-15, hepcidin (Hepc), and erythropoietin (EPO) were determined by ELISA. The levels of serum iron (SI), ferritin, TIBC, and transferrin saturation (TS) were measured by an auto analyzer. Iron staining of bone marrow cells was used for testing extracellular and intracellular iron. The GDF-15 level in the experimental group was higher than that of the case-control group and normal control group (all p < 0.05). The Hepc level in the experimental group and case-control group were both higher than that of healthy controls (all p < 0.05). The Hepc level was significantly lower in the experimental group patients who had excessive GDF-15 (r = -0.766, p = 0.000). There was a positive correlation between the level of GDF15 and EPO in the experimental group (r = 0.68, p < 0.000). The level of GDF15 in SAA patients was positively correlated with SI levels (r = 0.537, p = 0.008), TS levels (r = 0.466, p = 0.025), and sideroblasts (%) (r = 0.463, p = 0.026). Moreover, there was a positive correlation between GDF-15 level and blood transfusion-dependent time (r = 0.739, p = 0.000). Our data indicated that GDF-15 plays an important role in iron metabolism in SAA. GDF-15 might be a novel target for SAA therapy.

  6. Multidisciplinary approach to anemia

    Directory of Open Access Journals (Sweden)

    Anca Ghiațău

    2015-08-01

    Full Text Available Introduction: We present the case of a 65 years- old woman who was admitted with a severe macrocytic anemia Hb= 5.7g/dl and diffuse bone pain. Biologically she has moderate thrombocytopenia 35 000/µl, a hepatic cytolysis and cholestatic syndrome. Material and method: The patient was extensively evaluated before presentation for a mild iron - deficiency anemia for which she underwent endoscopic examination of the upper and lower gastrointestinal tract- normal. The bone marrow aspiration on admission revealed a marked hyperplasia of the erythroblastic line with ~50% basophilic erythroblasts suggesting a regenerative erythroid hyperplasia. These changes along with the marked reticulocytosis on the peripheral blood smear oriented us towards a hemolytic anemia; Folic acid, vitamin B12, autoimmune tests and hemolytic tests were all normal. We continued the investigations with a thoraco-abdominopelvic computed tomography which identified diffuse demineralization, vertebral compactation and pelvic stress fractures. The breast examination revealed a right breast nodule, but the breast ultrasonography pleaded for benignity. Lacking a clear definitive diagnosis we decided to perform a bone marrow biopsy. Results: The osteo- medullary biopsy pointed towards a medullar invasion from a lobular mammary carcinoma; In these circumstances we performed an ultrasound guided biopsy of the right mammary lump thus histologically confirming a tumoral invasion of the bone marrow with subsequent anemia. The patient started chemotherapy in the Oncology ward. Conclusion: The particularity of this case consists in the pattern of anemia, which initially seemed iron deficient and afterwards macrocytic – apparently hemolytic and was actually due to the tumoral medullar invasion and also the nonspecific ultrasonographic appearance of the breast tumor.

  7. Severe Hemorrhage from the Umbilical Cord at Birth: A Preventable Cause of Neonatal Shock

    OpenAIRE

    Singh, Neetu; Suresh, Gautham

    2013-01-01

    Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe...

  8. Serial blood donations for intrauterine transfusions of severe hemolytic disease of the newborn with the use of recombinant erythropoietin in a pregnant woman alloimmunized with anti-Ku.

    Science.gov (United States)

    Lydaki, Evaggelia; Nikoloudi, Irene; Kaminopetros, Petros; Bolonaki, Irene; Sifakis, Stavros; Kikidi, Katerina; Koumantakis, Evgenios; Foundouli, Kaliopi

    2005-11-01

    The management of a pregnant woman with the rare Ko phenotype and anti-Ku is a special challenge, because matched blood is extremely rare and the possibility of severe hemolytic disease of the newborn is high. A 30-year-old woman with rare Ko (Knull) phenotype presented at 18 weeks of gestation with positive indirect agglutination test results. She had anti-Ku due to previous blood transfusion, one pregnancy, and two abortions. During this pregnancy, anti-Ku titers ranged from 1024 to 4096. At the 26th week of gestation ultrasound showed a hydropic fetus and urgent intrauterine exchange transfusion was performed with the maternal red blood cells (RBCs). Recombinant human erythropoietin (rHu-EPO) and intravenous (IV) iron were administered to the mother to ensure an adequate supply of matched RBCs for intrauterine transfusions and possible perinatal hemorrhage. Intrauterine transfusions were repeated every 1 to 3 weeks. By 35 weeks 2 days of gestation, the mother had donated 4 units of blood, and four intrauterine transfusions had been performed. Cesarean section was then decided and a healthy male newborn was born. He was treated with phototherapy but without exchange transfusions. By the 15th day of life rHu-EPO was administrated to the newborn because of anemia. The maternal RBCs completely disappeared from the child's blood by Day 100. As shown in this case, treatment with rHu-EPO and IV Fe has effectively increased the mother's capacity to donate RBCs for autologous use and intrauterine transfusions, with no adverse effects to the mother or the child.

  9. Anemia

    Science.gov (United States)

    ... a hemoglobin value of less than 13.5 gm/dl in a man or less than 12.0 gm/dl in a woman. Normal values for children ... types of anemia cannot be prevented, eating healthy foods can help you avoid both iron-and vitamin- ...

  10. Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia

    NARCIS (Netherlands)

    Kekre, Natasha; Zhang, Ying; Zhang, Mei Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem M.; Joachim Deeg, H.; Kapoor, Neena; Lee, Jong Wook; Nakamura, Ryotaro; Pulsipher, Michael A.; Eapen, Mary; Antin, Joseph H

    2017-01-01

    For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin

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

    International Nuclear Information System (INIS)

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

    1988-01-01

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

  12. Syngeneic peripheral blood stem cell transplantation with immunosuppression for hepatitis-associated severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    Aleksandar Savic

    2010-12-01

    Full Text Available Hepatitis-associated aplastic anemia occurs in up to 10% of all aplastic anemia cases. Syngeneic bone marrow transplantation is rare in patients with severe aplastic anemia and usually requires pre-transplant conditioning to provide engraftment. We report on a 29-year-old male patient with hepatitis-associated severe aplastic anemia who had a series of severe infectious conditions before transplantation, including tracheal inflammation. Life-threatening bleeding, which developed after bronchoscopy, was successfully treated with activated recombinant factor VII and platelet transfusions. Syngeneic peripheral blood stem cell transplantation using immunosuppressive treatment with antithymocyte globulin and cyclosporin A without high-dose pre-transplant conditioning was performed, followed by complete hematologic and hepatic recovery.

  13. What Is Aplastic Anemia?

    Science.gov (United States)

    ... Home / Anemia Aplastic Anemia Also known as What Is Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is ... heart, heart failure , infections, and bleeding. Severe aplastic anemia can even cause death. Overview Aplastic anemia is ...

  14. MCPIP1 deficiency in mice results in severe anemia related to autoimmune mechanisms.

    Directory of Open Access Journals (Sweden)

    Zhou Zhou

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

  15. [Chronic and severe anemia caused by Ancylostoma duodenale in Ecuador. Diagnosis by duodenoscopy].

    Science.gov (United States)

    Calvopiña, Manuel; Flores, Jessica; Guaman, Isabel; Lara, Gabriela; Abarca, Jeyson

    2017-10-01

    For 11 years, a 38-year-old male residing in a subtropical region of Ecuador, was repeatedly diagnosed with chronic anemia, and treated with blood transfusions in a hospital of province of Cotopaxi, Ecuador. He was transferred to Quito for severe anemia, having hemoglobin of 4 g/dL. Duodenoscopy was performed and adult nematodes, identified later as Ancylostoma duodenale, were observed. The patient was successfully treated with albendazole for five consecutive days and given blood transfusions. In the control visit at eight months, without anemia and no hookworm ova in the stool examined were found.

  16. Unusual Manifestation of Severe Conjugated Hyperbilirubinemia in an Infant with Streptococcus pneumoniae-associated Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    Jung-Pin Chen

    2007-01-01

    Full Text Available Streptococcus pneumoniae is an uncommon etiologic organism in children with hemolytic uremic syndrome (HUS. Historically, severe S. pneumoniae-associated HUS usually has a poor clinical outcome. The clinical manifestations of marked jaundice and hepatic dysfunction in this form of HUS are extremely rare. We report a 10-month-old female infant with S. pneumoniae-associated HUS who had the unusual manifestation of severely elevated conjugated bilirubin and hepatic transaminases. Screening for viral hepatitis was negative, and evidence of biliary obstruction and hepatotoxic drug exposure was also absent. The patient was treated with antihypertensive agents for 2.5 months and required peritoneal dialysis for a period of 26 days. Hepatic function returned to normal on the 8th day of hospitalization. Renal function was mildly impaired at 1-year follow-up. Our report suggests that severe conjugated hyperbilirubinemia is a rare manifestation of S. pneumoniae-associated HUS in children. It is important for pediatricians that pneumococcal infection with severe hematologic and renal disorders should be investigated for evidence of S. pneumoniae-associated HUS. [J Formos Med Assoc 2007;106(2 Suppl:S17-S22

  17. Incidence and predictors of severe anemia in Asian HIV-infected children using first-line antiretroviral therapy

    NARCIS (Netherlands)

    Bunupuradah, Torsak; Kariminia, Azar; Chan, Kwai-Cheng; Ramautarsing, Reshmie; Huy, Bui Vu; Han, Ning; Nallusamy, Revathy; Hansudewechakul, Rawiwan; Saphonn, Vonthanak; Sirisanthana, Virat; Chokephaibulkit, Kulkanya; Kurniati, Nia; Kumarasamy, Nagalingeswaran; Yusoff, Nik Khairulddin Nik; Razali, Kamarul; Fong, Siew Moy; Sohn, Annette H.; Lumbiganon, Pagakrong

    2013-01-01

    There are limited data on treatment-related anemia in Asian HIV-infected children. Data from Asian HIV-infected children aged <18 years on first-line highly active antiretroviral therapy (HAART) were used. Children who had pre-existing severe anemia at baseline were excluded. Anemia was graded using

  18. Parvovirose e anemia acentuada em paciente imunocompetente Parvovirus and severe anemia in an immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Juliana Annete Damasceno

    2012-04-01

    Full Text Available Paciente de 16 anos, sexo masculino, com vírus da imunodeficiência humana (HIV negativo e sem doença hematológica prévia, desenvolveu anemia acentuada devido à infecção por parvovírus B19. A doença apresentou evolução bifásica, com acalmia clínica e retorno dos sintomas após 15 dias. Ao exame físico, apresentava-se descorado e febril, sem adeno e organomegalias, com sinais de insuficiência cardíaca. O aspirado de medula óssea mostrava megaloblastos com nucléolos aberrantes e, na histologia, foram observadas células gigantes com nucleolação aberrante e presença do corpúsculo de inclusão nuclear típico da parvovirose. O exame de imuno-histoquímica mostrou positividade para anticorpo específico para parvovírus. A sorologia comprovou a infecção.A 16-year-old human immunodeficiency virus (HIV negative male patient without hematological disease developed acute anemia due to parvovirus B19 infection. The disease showed a biphasic evolution: clinical remission and return of symptoms after 15 days. Physical examination revealed paleness and fever, neither adeno nor organomegalies, and signs of heart failure. The bone marrow aspiration showed megaloblasts with aberrant nucleoli. As far as histology is concerned, giant cells with aberrant nucleoli and the presence of intranuclear inclusions typical of Parvoviruses were observed. Immunohistochemistry revealed positivity for specific Parvovirus antibody. Serology confirmed parvovirus B19 infection.

  19. Interleukin-6 and Interleukin-8 Levels Correlate With the Severity of Aplastic Anemia in Children.

    Science.gov (United States)

    Gupta, Vineeta; Kumar, Sushil; Sonowal, Rimjhim; Singh, Surya K

    2017-04-01

    The aim of this study was to evaluate the levels of interleukin (IL)-6 and IL-8 in patients with aplastic anemia and its correlation with severity of the disease. IL-6 and IL-8 levels were measured in 40 patients with aplastic anemia in the age group of 4 to 14 years. A total of 40 healthy children served as controls. Quantitative estimation of IL-6 and IL-8 was performed using a solid-phase sandwich ELISA kit. Results were presented as IL-6 and IL-8 concentrations in pg/mL. Patients received immunosuppressive therapy per the British Committee for Standards in Haematology Guidelines 2009. Mean age of the patients was 9.78±2.74 years. IL-6 level of patients was elevated compared with controls (193.48±352.3 vs. 4.58±3.39; Paplastic anemia (724.33±519.42), followed by severe aplastic anemia (80.51±66.28 pg/mL), and non-severe aplastic anemia (6.01±1.89). Differences were statistically significant. A similar trend was also observed for IL-8 levels, where the levels were 41.02±24.23, 11.34±8.0, and 1.67±0.71 for very severe aplastic anemia, severe aplastic anemia, and non-severe aplastic anemia, respectively. The differences were again statistically significant. IL levels were also correlated with the treatment outcome. Responders had lower levels compared with nonresponders, but the difference was not statistically significant (186.36±322.45 vs. 198.74±368.10). Levels of ILs decreased in responders, but were not comparable with that of controls 6 months after therapy. High levels of IL-6 and IL-8 were observed in children with aplastic anemia. Increased levels showed correlation with disease severity and therefore appear to play an important role in aplastic anemia. However, levels had no significant correlation with the treatment outcome.

  20. Determinants of severe anemia among laboring mothers in Mekelle city public hospitals, Tigray region, Ethiopia.

    Directory of Open Access Journals (Sweden)

    Yirga Ebuy

    Full Text Available Anemia is a global public health problem that has affected a significant number of pregnant mothers worldwide. The World Health Organization has estimated the prevalence of anemia in pregnant women at 18% and 56% in developed and developing countries, respectively. This study aimed to identify factors associated with severe anemia among laboring women in Mekelle city public hospitals, Tigray, Ethiopia.This unmatched case-control study involved 264 (88 = cases and 176 = controls pregnant women who were recruited when they came for delivery service in Mekelle city public hospitals. The data was collected from July to August, 2016. In this study, a systematic sampling technique was used inselecting controls, but the cases were enrolled until the required sample size was reached. Bivariate and multivariate analyses were conducted to find predictors of severe anemia. Statistically significant predictors of severe anemia were identified at P-value <0.05 and 95% confidence interval.A total of 264 pregnant women who came for delivery services were enrolled in this study. The major predicting variables for the occurrence of severe anemia among laboring women were residency (AOR = 3.28, 95% CI: 1.26-8.48, number of pregnancies (AOR = 2.46, 95% CI: 1.14-5.29, iron folate supplementation (AOR = 3.29, 95% CI: 1.27-8.49, dietary diversification score (AOR = 3.23, 95% CI: 1.19-8.71 and duration of menstrual cycle (AOR = 2.37, 95% CI: 1.10-5.10. The variable 'blood loss during pregnancy' (AOR = 6.63 95% CI: 2.96-14.86 was identified as a strong predictor of the outcome variable, severe anemia.This study identified determinants of severe anemia among laboring women in Mekelle city public hospitals, Northern Ethiopia. To reduce anemia, strengthening health education provision related to the importance of birth spacing and consuming diversified and iron-enriched food should be considered. Moreover, screening of pregnant women for state of anemia during their

  1. Severe anemia and hydrops in a neonate with parvovirus B19 infection: a case report

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    Negar Sajjadian

    2013-12-01

    Full Text Available Background: Anemia at the time of birth may cause some problem like asphyxia, heart failure shock or even death in a neonate. Different etiologies can be considered for this problem. Parvovirus B19, as a viral organism, can cause hydrops fetalis and neonatal anemia and consequent complications. We present here a case of newborn infant with severe anemia who had human parvovirus B19 infection.Case Presentation: A male newborn with gestational age of 36 week was born from a mother with poor prenatal care and history of contact with domestic animal. The neonate was very pale with Apgar score 2 at 1 min and received resuscitation, mechanical ventilation and repeated blood transfusion The hemoglobin level was significantly low. Analysis was made based on the clinical presentations. According to the case history, physical and laboratory findings, neonatal severe anemia induced by parvovirus B19 infection was suggested and Laboratory work up documented his infection with parovirus B19.Conclusion: Parvovirus B19 (B19 virus is the smallest single strand linear DNA virus in animal viruses, which is the only strain of parvovirus that is pathogenic in humans. Human parvovirus B19 may cross the placenta and result in fetal infection, morbidity and death. Parvovirus is an uncommon cause of neonatal anemia and hydrops fetalis so this etiology must be considered in differential diagnosis of anemia at birth.

  2. Severe hemolytic disease of fetus and newborn caused by red blood cell antibodies undetected at first-trimester screening (CME).

    Science.gov (United States)

    Dajak, Slavica; Stefanović, Vedran; Capkun, Vesna

    2011-07-01

    The objective was to determine clinical consequences of anti-D and non-D antibodies undetected at first-trimester screening for infant or fetus. This retrospective cohort study included all pregnant women with red blood cell (RBC) antibodies who were tested between 1993 and 2008. Data were obtained from the forms for tracking immunization at the transfusion department. Each form was analyzed for three data sets: the order of screening at which the antibodies were detected (initial or repeated screening), the order of pregnancy (first pregnancy or higher), and whether the antibodies caused severe hemolytic disease of fetus and newborn (HDFN). In D- women, anti-D was detected in 1.3% of cases. The anti-D was undetected in 72 (37%) cases on the first-trimester screening, of which eight cases were complicated by severe HDFN. In this group, three patients were primigravidae. An overall non-D incidence of 0.2% was observed. In 16 cases, non-D were undetected on the first-trimester screening (10 anti-c, two anti-E, two anti-C, one anti-S, and one case of anti-Rh17). Non-D antibodies undetected on initial screening caused 11 cases of severe HDFN (27% of all severe non-D HDFN). Ten of them were in multiparous women. Seven of 11 cases with severe HDFN that were missed were caused by anti-c. The third-trimester screening may detect RBC antibodies that were not present or detected on the first-trimester screening. Such screening may be especially relevant in D+ multiparous women due to the risk of HDFN. © 2010 American Association of Blood Banks.

  3. Sequences responsible for the distinctive hemolytic potentials of Friend and Moloney murine leukemia viruses are dispersed but confined to the psi-gag-PR region.

    OpenAIRE

    Richardson, J; Corbin, A; Pozo, F; Orsoni, S; Sitbon, M

    1993-01-01

    Friend and Moloney murine leukemia viruses (F- and M-MuLV) induce distinct diseases in hematopoietic tissues following inoculation of newborn mice of susceptible strains. F-MuLV induces erythroleukemia preceded by severe early hemolytic anemia; M-MuLV induces thymomas and only very mild hemolysis. The major viral determinant of severe early hemolytic anemia residues in the env gene, but sequences located outside this gene can modulate this effect. By means of genetic chimeras of F- and M-MuLV...

  4. Prevalence and severity of anemia in pediatric hemodialysis patients, a single center study

    Directory of Open Access Journals (Sweden)

    Afshin Azhir

    2006-12-01

    Full Text Available BACKGROUNDS: This study was conducted to determine the prevalence and severity of anemia in children and adolescents on chronic hemodialysis, and to identify independent predictors of anemia in children on hemodialysis. METHODS: This cross-sectional study was performed between September 2005 and January 2006. The study population consisted of 25 patients aged 7−20 years on chronic hemodialysis from pediatric hemodialysis centers in Isfahan. RESULTS: A total of 22 (88% patients had hemoglobin levels of <11 g/dL (anemic and 12 patients (48% had hemoglobin levels of <8 g/dL (severe anemia. The mean age of these patients was 15.5 ± 3.7 years. Mean time on chronic dialysis was 20.44 ± 15.25 months. Anemia was more common and more severe among children who were on dialysis for less than 6 months. There was an inverse relationship between the severity of anemia and duration of hemodialysis (P = 0.019, r = – 0.465. Nearly all patients were treated with erythropoietin, Children with more severe anemia received slightly higher dose of erythropoietin (P = 0.09, r = 0.202. There was a significant difference between serum albumin values in anemic patients and patients without anemia (P = 0.023. There was a correlation between serum albumin and hemoglobin level (r = 0.511, P = 0.01. Intact PTH levels were >200 pg/ml in 16 patients (66% and >400 pg/ml in 9 patients (37. 5%. There was a reverse correlation between intact PTH level >200 pg/ml and hemoglobin level (r = -0.505, P = 0.046. CONCLUSIONS: The prevalence of anemia in hemodialysis children in Isfahan appears to be higher than that reported in the other studies in spite of extensive use of rHuEPO and iron supplementation. We found this to be especially true for patients new on hemodialysis (less than 6 months and in those with low albumin and severe hyperparathyroidism. KEY WORDS: Hemodialysis, anemia, children.

  5. Celiac Disease in Children with Moderate-to-Severe Iron-deficiency Anemia.

    Science.gov (United States)

    Narang, Manish; Natarajan, Ravikumar; Shah, Dheeraj; Puri, Amarender Singh; Manchanda, Vikas; Kotru, Mrinalini

    2018-01-15

    To evaluate the proportion of children with moderate to severe iron-deficiency anemia who have associated celiac disease. This cross-sectional analytical study was conducted among children aged 1 to 12 years of age with moderate-to-severe iron deficiency anemia and control children without anemia. Serum IgA-tissue trans-glutaminase levels were assessed in both cases and controls. All children with positive celiac serology underwent upper gastrointestinal endoscopy and duodenal biopsy; biopsy finding of Marsh grade 3 was considered positive for celiac disease. There were 152 anemic children and 152 controls with mean (SD) hemoglobinof 7.7 (1.8) and 12.2 (0.74) g/dL, respectively. 16 (10.5%) cases and 3 (2%) control patients had positive serology for celiac disease [OR (95% CI) 5.33 (1.52-18.67), P=0.007]. Six (3.9%) children with iron-deficiency anemia and none of the controls had biopsy features diagnostic of celiac disease. In the Northern Indian tertiary-care hospital outpatient setting, Celiac disease was associated with 4% of children presenting with moderate-to-severe anemia.

  6. Severe iron deficiency anemia and marked eosinophilia in adolescent girls with the diagnosis of human fascioliasis.

    Science.gov (United States)

    Tavil, Betül; Ok-Bozkaya, İkbal; Tezer, Hasan; Tunç, Bahattin

    2014-01-01

    Human fascioliasis (HF), caused by the common liver fluke Fasciola hepatica, is an endemic infection in many parts of tropical countries. HF can also be seen in some of the non-tropical countries. This report describes two girls with severe iron deficiency anemia and eosinophilia, who were diagnosed as HF. The infection was successfully eliminated with the administration of triclabendazole. No side effects or recurrence was observed after the treatment. It should be kept in mind that marked eosinophilia with severe iron deficiency anemia should alert pediatricians to the possibility of F. hepatica infection.

  7. Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia.

    Science.gov (United States)

    Kekre, Natasha; Zhang, Ying; Zhang, Mei-Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem; Deeg, H Joachim; Kapoor, Neena; Lee, Jong-Wook; Nakamura, Ryotaro; Pulsipher, Michael A; Eapen, Mary; Antin, Joseph H

    2017-07-01

    For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using human leukocyte antigen (HLA)-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft. There were no differences in hematopoietic recovery by type of antithymocyte globulin. Among recipients of HLA-matched sibling transplants, day 100 incidence of acute (17% versus 6%, P aplastic anemia. Copyright© 2017 Ferrata Storti Foundation.

  8. Relationship between severity of depression symptoms and iron deficiency anemia in women with major depressive disorder

    Directory of Open Access Journals (Sweden)

    Seyed gholamreza Noorazar

    2015-11-01

    Full Text Available Introduction: Iron deficiency (ID is a common nutritional problem lead to many unintended consequences such as decrease energy, immune system problems, and neurological dysfunction. The most common psychological disorder is depression. A patient with ID anemia (IDA show signs and symptoms of behavioral and mood disorders like depression. Methods: In this study, 100 female patients with diagnosed major depression in years 2010 and 2011 were studied. In all patients standard Hamilton depression rating scale (HDRS was used to evaluate depression severity. Blood samples were taken for complete blood count difference analysis and evaluating anemia and in those with hemoglobin (Hb < 12 mg/dl, ferritin, and total iron binding capacity were checked to evaluate IDA. Results: Patients mean age was 36.34 ± 10.43 years old. Mean HDRS score was 32.20 ± 4.07. 19 had anemia, and among them 8% had IDA. Mean HDRS score in patients with IDA (33.37 ± 1.90 was higher than those without (32.09 ± 4.19, but the difference was not significant (P = 0.39. There was no difference between patients with and without anemia in HDRS score. The negative relation was observed between Hb levels, and HDRS score (Pearson correlation = -0.21, P = 0.03. Conclusion: We observed that the negative correlation between Hb levels and HDRS score. It demonstrates the effect of Hb decrease and anemia occurrence on depression severity; however, it needs more studies.

  9. Influence of immune-mediated hemolytic anemia on flow velocities in the portal vein and caudal vena cava measured by use of pulsed-wave Doppler ultrasonography in dogs.

    Science.gov (United States)

    Smith, Rachel Policelli; Koenigshof, Amy M; Smith, Daniel J; Strom, Phillip R; Nelson, Nathan C

    2018-05-01

    OBJECTIVE To compare blood flow velocities of the portal vein (PV) and caudal vena cava (CVC) measured by use of pulsed-wave Doppler ultrasonography in clinically normal dogs and dogs with primary immune-mediated hemolytic anemia (IMHA). ANIMALS 11 client-owned dogs admitted to a veterinary teaching hospital for management of primary IMHA and 21 staff- or student-owned clinically normal dogs. PROCEDURES Flow velocities in the PV and CVC at the porta hepatis were evaluated in conscious unsedated dogs with concurrent ECG monitoring; evaluations were performed before dogs with IMHA received heparin or blood transfusions. Three measurements of peak velocity at end expiration were obtained for each vessel, and the mean was calculated. Results were compared between IMHA and control groups. RESULTS Mean ± SD blood flow velocity in the CVC differed between control (63.0 ± 18.6 cm/s) and IMHA (104 ± 36.9 cm/s) groups. Variance in dogs with IMHA was significantly greater than that for the clinically normal dogs. No significant difference in blood flow velocity in the PV was detected between IMHA and control dogs. CONCLUSIONS AND CLINICAL RELEVANCE Higher blood flow velocities were detected by use of pulsed-wave Doppler ultrasonography in the CVC of dogs with naturally occurring IMHA and may be used to predict anemia in patients suspected of having IMHA.

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

    Science.gov (United States)

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

    2014-01-01

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

  11. Reação transfusional hiper-hemolítica em pacientes portadores de anemia falciforme: relato de dois casos Hyper-hemolytic transfusional reaction in sickle cell patients: two case reports

    Directory of Open Access Journals (Sweden)

    Cláudia C.S. Naufel

    2002-12-01

    increased frequency of transfusions to which these patients are submitted, knowledge of the main risks and an adequate diagnosis of the complications caused by transfusional therapy are of fundamental importance. An atypical form of transfusional reaction, denominated hyperhemolytic transfusional reaction was recently described in sickle cell anemia patients after the transfusion of apparently compatible hemacias. In this case, previous conditions can exacerbate the hemolytic condition and put the life of the patient at risk. The pathophysiological conditions of this disease are not yet understood well and the treatment consists of suspending transfusions, corticoid therapy and / or administration of immunoglobulin. The aim of this work is o present two case reports of hyperhemolytic transfusional reaction in sickle cell anemia patients.

  12. Graft failure following bone marrow transplantation for severe aplastic anemia risk factors and treatment results

    NARCIS (Netherlands)

    Champlin, R.E.; Horowitz, M.M.; Bekkum, D.W. van; Camitta, B.M. Elfenbein, G.E.; Gale, R.P.; Gluckman, E.; Good, R.A.; Rimm, A.A. Rozman, C.; Speck, B. Bortin, M.M

    1989-01-01

    Graft failure was analyzed in 625 patients receiving allogeneic bone marrow transplants from HLA-identical sibling donors as treatment for severe aplastic anemia. Sixty-eight (11%) had no or only transient engraftment. Second bone marrow transplants were successful in achieving extended survival in

  13. Erythropoiesis in HIV-infected and uninfected Malawian children with severe anemia

    NARCIS (Netherlands)

    Calis, Job C. J.; Phiri, Kamija S.; Vet, Raymond J. W. M.; de Haan, Rob J.; Munthali, Francis; Kraaijenhagen, Robert J.; Hulshof, Paul J. M.; Molyneux, Malcolm E.; Brabin, Bernard J.; Boele van Hensbroek, Michaël; Bates, Imelda

    2010-01-01

    Anemia is common in HIV infection, but the pathophysiology is poorly understood. Bone marrow analysis in 329 severely anemic (hemoglobin <5 g/dl) Malawian children with (n = 40) and without (n = 289) HIV infection showed that HIV-infected children had fewer CD34(+) hematopoietic progenitors (median

  14. Elliptocytes and tailed poikilocytes correlate with severity of iron-deficiency anemia.

    Science.gov (United States)

    Rodgers, M S; Chang, C C; Kass, L

    1999-05-01

    This study examines the relationships between abnormal RBC morphology, RBC indices measured with an automated hematology analyzer, serum iron studies, and severity of anemia in patients with findings indicative of iron-deficiency anemia. Counts and morphologic classification of 1,000 RBCs from each of 22 patients were performed, and correlations were determined between parameters. The Student t test was used to determine the level of significance for correlations between parameters. Several significant relationships were found. As the percentage of elliptocytes increased, hemoglobin concentration, hematocrit, RBC concentration, and mean corpuscular hemoglobin level decreased (r = .48, .44, .40, and .49, respectively; P < .05). As the percentage of tailed poikilocytes increased, hemoglobin concentration, hematocrit, and RBC concentration decreased (r = .70, .77, and .71, respectively; P < .01) and RBC distribution width increased (r = .73; P < .01). Of significance, serum ferritin levels, long considered the best single indicator of iron deficiency, showed no correlation with the morphologic abnormalities assessed, severity of anemia, or any of the analyzer-generated indices. Our results indicate that microscopic evaluation of RBC morphology remains an important tool for the pathologist to evaluate the severity of anemia in patients with iron deficiency.

  15. Inborn anemias in mice

    International Nuclear Information System (INIS)

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

    1981-06-01

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

  16. Bone marrow transplantation in severe aplastic anemia and acute or chronic leukemia

    Energy Technology Data Exchange (ETDEWEB)

    Schaefer, U W; Mahmoud, H K; Beelen, D W; Hoffmann, W; Becher, R; Schmidt, C G; Bamberg, M; Quast, U; Haralambie, E; Linzenmeier, G

    1986-04-01

    In Essen 121 bone marrow transplantations were carried out. The indications were severe aplastic anemia, acute leukemia in relapse, acute leukemia in remission or chronic myeloid leukemia. The conditioning regimen consisted of cyclophosphamide or the combination of cyclophosphamide and total body irradiation. All patients were treated under strict gnotobiotic care. To mitigate the risk of CMV infections intravenous CMV-hyperimmunoglobulin and CMV-negative blood products have been applied routinely since two years. MTX was used as prophylaxis against GVH-disease. In case of severe aplastic anemia 13 patients (72%) are still alive with a median observation time of 24 months. In the prognostically unfavourable group of acute leukemia in relapse only one patient showed long term survival. In this patient leukemic relapse occurred six years after transplantation. The survival rate of AML patients grafted during the first remission is 55% with a median observation time of 40 months. For patients grafted in the first consecutive remission of ALL the survival rate is 42% with a maximal observation time of 29 months. Out of 37 patients grafted because of CML, eight were in an advanced stage of the disease. 13 patients are still alive, the maximal observation time is 37 months. The overall incidence of GVHD in patients at risk was 28% in aplastic anemia, 26% in AML, 9% in ALL and 63% in CML. In aplastic anemia no patient developed an interstitial pneumonia. In leukemia the risk of fatal interstitial pneumonia was 34%.

  17. Bone marrow transplantation in severe aplastic anemia and acute or chronic leukemia

    International Nuclear Information System (INIS)

    Schaefer, U.W.; Mahmoud, H.K.; Beelen, D.W.; Hoffmann, W.; Becher, R.; Schmidt, C.G.; Bamberg, M.; Quast, U.; Haralambie, E.; Linzenmeier, G.; Stollmann, B.; Grosse-Wilde, H.; Richter, H.J.; Hantschke, D.; Henneberg, K.; Luboldt, W.

    1986-01-01

    In Essen 121 bone marrow transplantations were carried out. The indications were severe aplastic anemia, acute leukemia in relapse, acute leukemia in remission or chronic myeloid leukemia. The conditioning regimen consisted of cyclophosphamide or the combination of cyclophosphamide and total body irradiation. All patients were treated under strict gnotobiotic care. To mitigate the risk of CMV infections intravenous CMV-hyperimmunoglobulin and CMV-negative blood products have been applied routinely since two years. MTX was used as prophylaxis against GVH-disease. In case of severe aplastic anemia 13 patients (72%) are still alive with a median observation time of 24 months. In the prognostically unfavourable group of acute leukemia in relapse only one patient showed long term survival. In this patient leukemic relapse occurred six years after transplantation. The survival rate of AML patients grafted during the first remission is 55% with a median observation time of 40 months. For patients grafted in the first consecutive remission of ALL the survival rate is 42% with a maximal observation time of 29 months. Out of 37 patients grafted because of CML, eight were in an advanced stage of the disease. 13 patients are still alive, the maximal observation time is 37 months. The overall incidence of GVHD in patients at risk was 28% in aplastic anemia, 26% in AML, 9% in ALL and 63% in CML. In aplastic anemia no patient developed an interstitial pneumonia. In leukemia the risk of fatal interstitial pneumonia was 34%. (orig.) [de

  18. Genetics Home Reference: atypical hemolytic-uremic syndrome

    Science.gov (United States)

    ... Kidney Diseases: Kidney Failure: Choosing a Treatment That's Right for You Educational Resources (6 links) Disease InfoSearch: Hemolytic uremic syndrome, atypical MalaCards: genetic atypical hemolytic-uremic syndrome Merck Manual Consumer Version: Overview of Anemia Merck Manual Consumer Version: ...

  19. A successful desensitization protocol for horse-derived antithymocyte globulin in severe aplastic anemia.

    Science.gov (United States)

    Demir, Esen; Cigerci Günaydın, Nurşen; Karadaş, Nihal; Gülen, Figen; Tanac, Remziye; Yılmaz, Deniz

    2015-03-01

    Horse antithymocyte globulin (h-ATG) (ATGAM(®) ) is the first choice of treatment in very severe patients with aplastic anemia who do not have any HLA matched sibling donor. h-ATG is a heterologous serum that may cause anaphylaxis. Alternative treatment strategies must be planned in case of hypersensitivity. Desensitization must be considered in patients without an alternative treatment of choice. We aimed to present the h-ATG desensitization protocol and consider its effectiveness in patients with aplastic anemia who are hypersensitized with h-ATG and do not have an alternative treatment of choice. Skin prick tests were performed with non-diluted solution in eight very severe patients with aplastic anemia who are followed up in Ege University Children's Hospital. Although skin prick test was found negative in these eight patients, different dilution h-ATG intradermal tests were performed and found positive in all patients. h-ATG desensitization program was started to these hypersensitized patients. Desensitization program was started to six male and two female very severe patients with aplastic anemia whose ages were between seven and 19 yr (median: 12.9 yr). All of the patients completed the desensitization program. While local reaction was seen in two patients, systemic reaction was seen in one patient and late reaction was seen in one patient during and after desensitization program. A successful desensitization program with h-ATG in children with aplastic anemia is presented. Even though there is not an exposure before to such high allergy potential heterologous serum, skin tests should be performed and desensitization must be started to patients who are hypersensitized to h-ATG. As the expected effectiveness of the treatment is so much, the desensitization protocol can be carried out safely and effectively with trained stuff although allergic reactions can be seen. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Reduced Hsp70 and Glutamine in Pediatric Severe Malaria Anemia

    DEFF Research Database (Denmark)

    Kempaiah, Prakasha; Dokladny, Karol; Karim, Zachary

    2016-01-01

    by decreased HSPA1A, a heat shock protein (Hsp) 70 coding gene. Hsp70 is a ubiquitous chaperone that regulates Nuclear Factor-kappa B (NF-κB) signaling and production of pro-inflammatory cytokines known to be important in malaria pathogenesis (e.g., IL-1β, IL-6 and TNF-α). Since the role of host Hsp70...... in malaria pathogenesis is unexplored, we investigated Hsp70 and molecular pathways in children with SMA. Validation experiments revealed that leukocytic HSP70 transcripts were reduced in SMA relative to non-severe malaria, and that intraleukocytic hemozoin (PfHz) was associated with lower HSP70. HSP70...... was correlated with reticulocyte production and Hb. Since glutamine (Gln) up-regulates Hsp70, modulates NF-κB activation, and attenuates over-expression of pro-inflammatory cytokines, circulating Gln was measured in children with malaria. Reduced Gln was associated with increased risk of developing SMA...

  1. Development of Hemolytic Anemia in a Nivolumab-Treated Patient with Refractory Metastatic Squamous Cell Skin Cancer and Chronic Lymphatic Leukemia

    Directory of Open Access Journals (Sweden)

    K.S. Schwab

    2016-06-01

    Full Text Available Management of patients with metastatic squamous cell skin cancer, refractory to initial therapy with standard chemotherapy and radiation protocols, remains difficult with poor overall prognosis and limited therapeutic options. Recently, promising response rates with nivolumab, a programmed death receptor-1-blocking antibody, in squamous cancer of the head and neck have been demonstrated. Considering the similar histological patterns of squamous cell cancer of the skin and squamous cell cancer of the head and neck, we assumed that nivolumab could also be effective in our patients with refractory metastatic squamous cell cancer of the skin. So far, there have been no clinical data on the therapeutic efficacy of nivolumab in squamous cell skin cancer. We here present a case of a patient with metastatic squamous cell skin cancer refractory to previous therapies, who showed a good response to nivolumab over a period of 5 months, but developed a serious hemolytic crisis under nivolumab treatment after eight applications.

  2. Circulating thrombopoietin levels in normal healthy blood donors and in aplastic anemia patients in relation to disease severity

    Directory of Open Access Journals (Sweden)

    Abhay Singh

    2015-01-01

    Full Text Available Background: Thrombopoietin (TPO is the key hematopoietic growth factor regulating the production of platelets from bone marrow megakaryocytes and maintaining platelet hemostasis. This study was done to find any relationship between the levels of thrombopoietin and the severity of disease in patients with aplastic anemia. Materials and Methods: Serum samples were collected from 52 patients with a confirmed diagnosis of aplastic anemia and 45 normal healthy blood donors of both sexes over a period of 2 years, and TPO was estimated by using commercially available TPO-specific-enzyme-linked immunosorbent assay. Results: The median TPO level of 1190 pg/ml (range 625-7651 pg/ml in aplastic anemia patients was significantly higher than the median TPO level of 121.1 pg/ml (81.25-237.7 pg/ml in normal healthy blood donors (P = 0.000. No significant difference was observed in TPO levels of male and female patients (P = 0.453. The median TPO concentrations observed in very severe aplastic anemia, severe aplastic anemia, and nonsevere aplastic anemia were 2765 pg/ml (range 625-6451 pg/ml, 1190 pg/ml (range 672.1-7651 pg/ml, and 1111.5 pg/ml (range 761.1-2289.2 pg/ml, respectively. TPO in patients of very severe aplastic anemia was significantly higher than patients of nonsevere aplastic anemia (P = 0.043, with no significant relation among rest of the groups. Discussion: TPO levels in aplastic anemia patients were significantly higher than in healthy blood donors; however, in aplastic anemia patients TPO levels were significantly higher only in patients with very severe disease.

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

    Directory of Open Access Journals (Sweden)

    Adiyyatu Sa′idu Usman

    2013-01-01

    Full Text Available 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.

  4. Extraskeletal myxoid chondrosarcoma in the lung: asymptomatic lung mass with severe anemia

    Directory of Open Access Journals (Sweden)

    Zhou Qianjun

    2012-08-01

    Full Text Available Abstract Extraskeletal myxoid chondrosarcoma (EMC is a rare soft-tissue sarcoma, which primarily occurs deep in the extremities, especially in skeletal muscle, or tendon. EMC of the pleura has been described, however, no case of primary EMC arising from lung has been previously reported. We describe herein, a 51-year-old Asian female initially manifested with signs of severe anemia who presented with a lung mass unrelated to pleura that was morphologically typical EMC, with strong immunoreactivity for vimentin and NSE. Two weeks after resection, the anemia was cured. The patient continued with follow-up, without sign of abnormality 32 months after operation. Virtual slides The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2882199847396682

  5. Recovery from anemia in patients with severe aortic stenosis undergoing transcatheter aortic valve implantation--prevalence, predictors and clinical outcome

    DEFF Research Database (Denmark)

    De Backer, Ole; Arnous, Samer; Lønborg, Jacob

    2014-01-01

    INTRODUCTION: Preoperative anemia is common in patients with severe aortic stenosis undergoing transcatheter aortic valve implantation (TAVI) and has been linked to a poorer outcome--including a higher 1-year mortality. The aim of this study was to investigate the impact of successful TAVI...... on baseline anemia. METHODS: A total of 253 patients who survived at least 1 year following TAVI were included in this study. The prevalence, predictors and clinical outcome of hemoglobin (Hb)-recovery were assessed. RESULTS: The prevalence of baseline anemia was 49% (n = 124)--recovery from anemia occurred......-recovery, while blood transfusion (OR 0.31, P = 0.038) and chronic kidney disease (CKD, OR 0.33, P = 0.043) were identified as negative predictors at, respectively, one and two years after TAVI. When compared to patients without baseline anemia, those anemic patients with Hb-recovery had a similar functional...

  6. Protective Effects of Royal Jelly and Vitamin C against Experimental Hemolytic Anemia on Sex Hormones and Histochemical Testicle Tissue Histochemistry of Adult Mice

    Directory of Open Access Journals (Sweden)

    H Anbara

    2016-03-01

    Full Text Available Introduction: Phenylhydrazine (PHZ is a well-known hemolytic compound inducing intoxication in erythrocytes. Therefore, the present study aimed to evaluate the protective effects of royal jelly and vitamin C against phenylhydrazine-induced damages in mouse testicles. Methods: In this study, 64 adult male mice were randomly and equally assigned to eight groups. The first group received normal saline (0.1ml intraperitoneally. The second group received PHZ (6 mg/100 gr intraperitoneally in 48-hour intervals. The third group received vitamin C (250 mg/kg/day intraperitoneally perday a long with PHZ. The fourth group received royal jelly (100 mg/kg/day through gavage. The fifth group received PHZ along with vitamin C and royal jelly in similar doses to the previous groups. The sixth group received only vitamin C, the seventh group recieved only royal jelly, and finally the eighth group received similar doses of vitamin C and royal jelly. After 35 days, serum and tissue samples were taken and used for histochemical (Mallory-Azan, Alkaline phosphatase, Oil red-O and PAS, and serum analyses (Testosterone, LH, FSH. Results: The study results revealed the histochemical changes in testicular tissue of the phenylhydrazine group, in which vitamin C and royal jelly partly improved the changes. Furthermore, serum analyses demonstrated a significant decrease in testosterone, FSH and LH levels, which this decrease was diminished by royal jelly and vitamin C. Conclusions: Royal jelly and vitamin C seem to have the potential to decrease serum and tissue damages induced by phenylhydrazine via restraining free radicals.

  7. Thrombolytic therapy for critical limb ischemia in a Jehovah's Witness with severe anemia

    Directory of Open Access Journals (Sweden)

    Jeremy D. Kauffman, MD

    2017-09-01

    Full Text Available A patient's refusal to receive blood products can pose both clinical and ethical challenges to the surgeon. In this report, we review the case of a Jehovah's Witness presenting with critical lower limb ischemia and severe anemia for whom the decision of whether to perform thrombolytic therapy was complicated by his refusal to accept blood products. The case demonstrates that thrombolytic therapy can produce favorable results in severely anemic patients even when transfusion is not an option. We conclude that offering thrombolytic therapy in this context is a reasonable therapeutic option from both a clinical and ethical perspective.

  8. A child with severe iron-deficiency anemia and a complex TMPRSS6 genotype.

    Science.gov (United States)

    Capra, Anna Paola; Ferro, Elisa; Cannavò, Laura; La Rosa, Maria Angela; Zirilli, Giuseppina

    2017-10-01

    We report a case of a 7-year-old girl with severe hypochromic microcytic anemia, who was unresponsive to classical iron supplements. We suspected IRIDA, iron-refractory iron-deficiency anemia, a genetic iron metabolism disorder, caused by TMPRSS6 variations. TMPRSS6 encodes matriptase-2, a negative regulator of hepcidin, and its pathological variants are related to normal to high levels of hepcidin. We analyzed the TMPRSS6 gene and we improved clinical management of the patient, selecting the appropriate supplementation therapy. Intervention & Technique: The parenteral iron therapy was started, but the patient was only partially responsive and the anemia persisted. To confirm the diagnosis, the TMPRSS6 gene sequence was analyzed by DNA sequencing and other relevant biochemical parameters were evaluated. The TMPRSS6 sequence analysis showed a complex genotype with a rare heterozygous missense variant, in addition to other common polymorphisms. The serum hepcidin value was normal. We unexpectedly observed a normalization of patient's hemoglobin (Hb) levels only after liposomal iron treatment. The proband was symptomatic for IRIDA during a critical phase of growth and development, but we did not find a clearly causative genotype. A long-term result, improving stably patient's Hb levels, was obtained only after liposomal iron supplementation. Children may be at greater risk for iron deficiency and the degree of anemia as well as the response to the iron supplements varies markedly patient to patient. Here, we show the importance of comprehensive study of these patients in order to collect useful information about genotype-phenotype association of genes involved in iron metabolism.

  9. Hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Faruk Öktem

    2011-12-01

    Full Text Available Haemolytic uremic syndrome (HUS is a severe disease with microangiopathic anemia, thrombocytopenia and leading cause of acute renal failure in children. Several etiological factors causing to HUS have been identified, like infections, genetic mutations, drugs, systemic diseases. In this review, we present the new classification of the disease, detailed information about pathogenesis, diagnostic methods and therapeutic approaches.

  10. Neonatal management and outcome in alloimmune hemolytic disease.

    Science.gov (United States)

    Ree, Isabelle M C; Smits-Wintjens, Vivianne E H J; van der Bom, Johanna G; van Klink, Jeanine M M; Oepkes, Dick; Lopriore, Enrico

    2017-07-01

    Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management. Areas covered: This review focusses on the current neonatal management and outcome of hemolytic disease and discusses postnatal treatment options as well as literature on long-term neurodevelopmental outcome. Expert commentary: Despite major advances in neonatal management, multiple issues have to be addressed to optimize postnatal management and completely eradicate kernicterus. Except for strict adherence to guidelines, improvement could be achieved by clarifying the epidemiology and pathophysiology of HDFN. Several pharmacotherapeutic agents should be further researched as alternative treatment options in hyperbilirubinemia, including immunoglobulins, albumin, phenobarbital, metalloporphyrins, zinc, clofibrate and prebiotics. Larger trials are warranted to evaluate EPO, folate and vitamin E in neonates. Long-term follow-up studies are needed in HDFN, especially on thrombocytopenia, iron overload and cholestasis.

  11. Effects of Methanol Seed Extract of Aframomum melegueta (Alligator Pepper on Wistar Rats with 2,4-Dinitrophenylhydrazine-induced Hemolytic Anemia

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    Damilola A. Omoboyowa

    2017-04-01

    Full Text Available The prevalence of parasitic infections such as malaria, which leads to decrease in hematological indices, the major cause of anemia, constitutes a serious health challenge in many developing countries such as Nigeria. This study investigated the effect of methanol seed extract of Aframomum melegueta on selected hematological indices of 2,4-dinitrophenylhydrazine (2,4-DNPH-induced anemic rats model. The toxicity study and qualitative phytochemical screening of the extract were carried out using standard procedure. Twenty Wistar rats were grouped into five of four rats each (n = 4. Group I: Normal control; Group II: Negative control; Group III: administered 20 ml/kg b.w. of Astifer (Standard drug; Group IV and V were administered 200 and 400 mg/kg b.w. of the extract, respectively. The animals of Groups II to V were induced with 2,4-dinitrophenylhydrazine (20 mg/kg b.w. once daily for seven consecutive days; their blood samples were collected by ocular puncture into heparinized capillary tubes for hematological analysis and animals with packed cell volume (PCV ≥ 30% reduction were considered anemic for the study. The result of the qualitative phytochemical analysis showed that the methanol extract tested positive to alkaloids, carbohydrate, saponins, flavonoids, steroids, terpenoids, and anthraquinones. Acute toxicity and lethality studies on methanol extract showed an oral LD50 equal or less than 5000 mg/kg b.w. in mice. The rats administered 20 ml/kg b.w. of Astifer showed significant (P 0.05 decrease in hemoglobin count, RBC, platelet, neutrophils and lymphocyte count compared with the normal control animals. The rats administered 400 mg/kg b.w. of A. melegueta showed significant (P 0.05 lower PCV, RBC, WBC count, and lymphocyte count compared with anemic rats administered with 0.3 ml of normal saline. It can be concluded that Aframomum melegueta seed has beneficial immunological and hematological properties in Wistar rats and possessed

  12. Severe anemia is associated with poor tumor oxygenation in head and neck squamous cell carcinomas

    International Nuclear Information System (INIS)

    Becker, Axel; Stadler, Peter; Lavey, Robert S.; Haensgen, Gabriele; Kuhnt, Thomas; Lautenschlaeger, Christine; Feldmann, Horst Juergen; Molls, Michael; Dunst, Juergen

    2000-01-01

    Purpose: To investigate the relationship between tumor oxygenation and the blood hemoglobin (Hb) concentration in patients with squamous cell carcinoma of the head and neck (SCCHN). Methods and Materials: A total of 133 patients with SCCHN underwent pretreatment polarographic pO 2 measurements of their tumors. In 66 patients measurements were also made in sternocleidomastoid muscles. The patients were divided into three groups according to their Hb concentration--severe anemia (Hb 2 . Conclusion: Our data suggest that a low Hb concentration and cigarette smoking contribute to inadequate oxygenation of SCCHN and thus for increased radioresistance. Consequently, Hb correction and abstinence from smoking may significantly improve tumor oxygenation

  13. Anti-HI can cause a severe delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease patients.

    Science.gov (United States)

    Ibanez, Clara; Habibi, Anoosha; Mekontso-Dessap, Armand; Chadebech, Philippe; Chami, Btissam; Bierling, Philippe; Galactéros, Frédéric; Rieux, Claire; Nataf, Joëlle; Bartolucci, Pablo; Peyrard, Thierry; Pirenne, France

    2016-07-01

    Delayed hemolytic transfusion reaction (DHTR) is a life-threatening condition in sickle cell disease (SCD) patients that is frequently complicated by hyperhemolysis. Antibodies resulting from antigen disparity between donors of European ancestry and patients of African ancestry are common, but situations involving antibodies not classically of clinical significance are also encountered. Anti-HI is generally considered to be an innocuous naturally occurring antibody. We describe two cases of hyperhemolysis with anti-HI and provide details of the reported cases. Both SCD patients were polyimmunized and belonged to blood group B. They developed anti-HI that was reactive at 37°C, after the transfusion of group O red blood cell units matched for all known and produced antibodies classically considered to be clinically significant. Both patients developed DHTR with hyperhemolysis. In the first case, a pregnant woman, a second transfusion was unavoidable and the patient died from cardiac arrest. The state of the second patient improved without the need for further transfusion. Three other cases of DHTR with anti-HI have been described in the literature in SCD patients. The two additional cases reported here definitively demonstrate that anti-HI is dangerous in SCD patients. As a result, ABO-identical matching (including A1 status) must be considered in SCD patients with anti-HI. © 2016 AABB.

  14. Zinc and antioxidant vitamin deficiency in patients with severe sickle cell anemia

    International Nuclear Information System (INIS)

    Hasanato, R.M. W.

    2006-01-01

    Patients with severe sickle cell anemia (SCA) have a higher potential for oxidative damage due to chronic redox imbalance in red blood cells that often leads to hemolysis, endothelial injury and recurrent vaso-occlusive episodes. This study evaluated the plasma levels of Vitamin A, C and E as indicators of antioxidants status. In addition, serum levels of zinc and copper were also estimated. Twenty-five adult patients with severe sickle cell anemia (12 males and 13 females aged 29.72+-12.94 years) and 25 matched controls were studied. Plasma levels of vitamin A, C and E were measured by HPLC technique. Serum zinc and copper levels were measured by atomic absorption spectrometry. There was significant decrease in plasma levels of vitamins A, C and E and in serum levels of zinc in patients with SCA as compared with controls (P<0.0001). Serum copper levels were significantly elevated compared with controls (P<0.0001). These findings emphasized the significant deficiencies of the antioxidant vitamins A, C and E and the trace element zinc along with the significant elevation of serum copper in patients with severe sickle cell disease. Further studies are needed to find out whether supplementation of antioxidant vitamins and zinc may ameliorate some sickle cell disease complications. (author)

  15. Severe hemorrhage from the umbilical cord at birth: a preventable cause of neonatal shock.

    Science.gov (United States)

    Singh, Neetu; Suresh, Gautham

    2013-01-01

    Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe that umbilical cord bleeding from errors in cord clamping could be an important cause of acute blood loss in the delivery room and that it may result in significant clinical morbidity, especially in extremely premature infants.

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... to moderate iron-deficiency anemia, or red blood cell transfusion for severe iron-deficiency anemia. You may ... body needs iron to make healthy red blood cells. Iron-deficiency anemia usually develops over time because ...

  17. Outcome of children with severe acquired aplastic anemia treated with rabbit antithymocyte globulin and cyclosporine A

    Directory of Open Access Journals (Sweden)

    Marlene Pereira Garanito

    2014-09-01

    Full Text Available Objective: To evaluate the outcome of children with severe acquired aplastic anemia treated with rabbit antithymocyte globulin and cyclosporine as first-line treatment at this institution. Methods: Retrospective analysis of 26 pediatric patients with aplastic anemia, treated between 1996 and 2011 with rabbit antithymocyte globulin plus cyclosporine. Results: The overall response rate at six months was 34.6% (9/26, and the cumulative incidence of relapse was 26.5% (95% confidence interval [CI]: 1.4%-66% at 5 years. The cumulative incidence of clonal evolution after immunosuppressive therapy was 8.3% (95% CI: 0.001%-53.7% at five years with both clonal evolutions in non -responders who acquired monosomy 7 karyotype. The overall survival at five years was 73.6% (95% CI: 49.2%-87.5%. Conclusions: The present results confirm the poor response rate with rabbit antithymocyte globulin as first therapy in pediatrics patients, similar to what has been reported for patients of all ages. This confirmation is problematic in Brazil, given the lack of horse antithymocyte globulin in many markets outside the United States. Resumo: Objetivo: Avaliar o resultado de crianças com anemia aplástica grave adquirida tratadas com globulina antitimocítica de coelho e ciclosporina como tratamento inicial em nosso instituto. Métodos: Análise retrospectiva de 26 pacientes pediátricos com anemia aplástica tratados entre 1996 e 2011 com globulina antitimocítica de coelho e ciclosporina. Resultados: A taxa de resposta geral em seis meses foi de 34,6% (9/26, e a incidência acumulada de recorrência foi de 26,5% (intervalo de confiança [IC] de 95%,1,4%-66% em cinco anos. A incidência acumulada de evolução clonal após a terapia imunossupressora foi de 8,3% (IC 95%, 0,001%-53,7% em cinco anos, com ambas as evoluções clonais em pacientes sem resposta que adquiriram o cariótipo com monossomia 7. A sobrevida geral em cinco anos foi de 73,6% (IC 95%, 49

  18. Hemolytic disease of the fetus and newborn: managing the mother, fetus, and newborn.

    Science.gov (United States)

    Delaney, Meghan; Matthews, Dana C

    2015-01-01

    Hemolytic disease of the fetus and newborn (HDFN) affects 3/100 000 to 80/100 000 patients per year. It is due to maternal blood group antibodies that cause fetal red cell destruction and in some cases, marrow suppression. This process leads to fetal anemia, and in severe cases can progress to edema, ascites, heart failure, and death. Infants affected with HDFN can have hyperbilirubinemia in the acute phase and hyporegenerative anemia for weeks to months after birth. The diagnosis and management of pregnant women with HDFN is based on laboratory and radiographic monitoring. Fetuses with marked anemia may require intervention with intrauterine transfusion. HDFN due to RhD can be prevented by RhIg administration. Prevention for other causal blood group specificities is less studied. © 2015 by The American Society of Hematology. All rights reserved.

  19. Sickle cell anemia and mitral valve replacement. Case report.

    Science.gov (United States)

    Bomfim, V; Ribeiro, A; Gouvea, F; Pereira, J; Björk, V

    1989-01-01

    An 8-year-old black boy with sickle cell disease and severe hemolytic anemia crisis (95% hemoglobin S) also had mitral incompetence due to rheumatic valve disease. A 27 mm monostrut Björk-Shiley valve prosthesis was implanted after partial exchange transfusions had reduced the hemoglobin S to less than 40%. High-flow normothermic perfusion was used during extracorporeal circulation, with care taken to avoid hypoxia and acidosis. Postoperative recovery was uneventful.

  20. Severe neonatal anemia from fetomaternal hemorrhage: report from a multihospital health-care system.

    Science.gov (United States)

    Christensen, R D; Lambert, D K; Baer, V L; Richards, D S; Bennett, S T; Ilstrup, S J; Henry, E

    2013-06-01

    The incidence of fetomaternal hemorrhage that is severe enough to cause neonatal anemia is not known. Owing to its relative rarity, much of the literature describing this condition is in the form of case reports and small case series. We performed a large, muiticentered, sequential, case series to determine the incidence, antecedents and outcomes. From the multicentered databases of Intermountain Healthcare, we obtained records of all neonates with hematocrit (Hct) hemorrhage. Among 219,853 live births, 24 had anemia with evidence of fetomaternal hemorrhage (incidence estimate, 1 per 9160 live births). The initial Hgb ranged from 1.4 to 10.2 g dl(-1) (Hct 29.8%). The initial Hgb was neonatal Hgb was hemorrhage (IVH). The adverse outcomes of death, IVH, periventricular leukomalacia, bronchopulmonary dysplasia or hypoxic-ischemic encephalopathy were common; occurring in 71% (17 of the 24), including all with an initial Hgb hemorrhage is a rare but sometimes devastating condition. Those with fetomaternal hemorrhage and an initial Hgb of <5 g dl(-1) are expected to need resuscitation at birth, to receive emergent transfusion support and to be at risk for death and major morbidities. Antenatal suspicion of this diagnosis should occur when absent fetal movement is reported. Improvements in rapid diagnosis are needed to prepare first responders and transfusion services.

  1. Assessing the sensitivity of human skin hyperspectral responses to increasing anemia severity levels

    Science.gov (United States)

    Baranoski, Gladimir V. G.; Dey, Ankita; Chen, Tenn F.

    2015-09-01

    Anemia is a prevalent medical condition that seriously affects millions of people all over the world. In many regions, not only its initial detection but also its monitoring are hindered by limited access to laboratory facilities. This situation has motivated the development of a wide range of optical devices and procedures to assist physicians in these tasks. Although noticeable progress has been achieved in this area, the search for reliable, low-cost, and risk-free solutions still continues, and the strengthening of the knowledge base about this disorder and its effects is essential for the success of these initiatives. We contribute to these efforts by closely examining the sensitivity of human skin hyperspectral responses (within and outside the visible region of the light spectrum) to reduced hemoglobin concentrations associated with increasing anemia severity levels. This investigation, which involves skin specimens with distinct biophysical and morphological characteristics, is supported by controlled in silico experiments performed using a predictive light transport model and measured data reported in the biomedical literature. We also propose a noninvasive procedure to be employed in the monitoring of this condition at the point-of-care.

  2. A Giant Brunneroma Causing Gastrointestinal Bleeding and Severe Anemia Requiring Transfusion and Surgery

    Directory of Open Access Journals (Sweden)

    Nicola C. Frenkel

    2017-01-01

    Full Text Available Brunner’s gland hamartoma, also called hyperplasia, adenoma, and Brunneroma, is an extremely rare benign proliferative lesion of Brunner’s glands in the duodenum. While being mostly small and asymptomatic, they can result in gastrointestinal bleeding and obstruction. We report the case of a 54-year-old man presenting with melena and severe anemia requiring blood transfusion. CT scans showed a large mass of 8 cm in diameter, presumably arising in the duodenum. Endoscopic biopsies were not conclusive. As we were unable to determine the nature of the mass preoperatively and due to the severe symptoms, its size, and the uncertain malignant potential, a classic Whipple procedure was performed. The resected specimen showed extensive proliferation of Brunner’s glands without signs of malignancy.

  3. Severe anemia caused by babesiosis in a maned wolf (Chrysocyon brachyurus).

    Science.gov (United States)

    Phair, Kristen A; Carpenter, James W; Smee, Nicole; Myers, Carl B; Pohlman, Lisa M

    2012-03-01

    An 8-yr-old, captive, spayed, female maned wolf (Chrysocyon brachyurus) developed progressive lethargy and weakness over a 24-hr period. Clinical signs included vomiting, recumbency, horizontal nystagmus, possible blindness, pale icteric mucus membranes, and port-wine colored urine. A complete blood cell count revealed severe anemia (packed cell volume [PCV], 6%) and intraerythrocytic piroplasms consistent with a Babesia species. Polymerase chain reaction testing later confirmed babesiosis. The wolf was treated with imidocarb dipropionate, antibiotics, and fluid therapy. A whole-blood transfusion from a sibling maned wolf also was performed. Despite aggressive treatment, the wolf failed to improve and was euthanized. To the authors' knowledge, this is the first documented case of babesiosis in a captive maned wolf in North America. Surveillance of infectious diseases in captive and wild maned wolf populations should be expanded to include screening for Babesia species. Tick control also should be implemented to prevent and decrease transmission of the disease to this endangered species.

  4. Total lymphoid irradiation and cyclophosphamide conditioning prior to bone marrow transplantation for patients with severe aplastic anemia

    International Nuclear Information System (INIS)

    Ramsay, N.K.; Kim, T.H.; McGlave, P.; Goldman, A.; Nesbit, M.E. Jr.; Krivit, W.; Woods, W.G.; Kersey, J.H.

    1983-01-01

    A preparative regimen, consisting of total lymphoid irradiation and cyclophosphamide, was utilized in 40 patients with severe aplastic anemia undergoing allogeneic marrow transplantation. This regimen was successful in decreasing rejection in these previously transfused patients, as only one patient rejected the marrow graft. Twenty-nine of the 40 transplanted patients are surviving from 1.5 to 59 mo, with a median follow-up of 24 mo. The actuarial survival rate for these heavily transfused patients with aplastic anemia is 72% at 2 yr. This preparative regimen is extremely effective in decreasing rejection following transplantation for severe aplastic anemia. Future efforts in this area must be aimed at the elimination of graft-versus-host disease and control of fatal infections

  5. Quantitative MRI shows cerebral microstructural damage in hemolytic-uremic syndrome patients with severe neurological symptoms but no changes in conventional MRI

    Energy Technology Data Exchange (ETDEWEB)

    Weissenborn, Karin; Worthmann, Hans; Heeren, Meike [Hannover Medical School, Clinic for Neurology, Hannover (Germany); Bueltmann, Eva; Donnerstag, Frank; Giesemann, Anja M.; Goetz, Friedrich; Lanfermann, Heinrich; Ding, Xiao-Qi [Hannover Medical School, Institute of Diagnostic and Interventional Neuroradiology, Hannover (Germany); Kielstein, Jan; Schwarz, Anke [Hannover Medical School, Clinic for Nephrology and Hypertension, Hannover (Germany)

    2013-07-15

    Severe neurological symptoms in Shiga toxin-producing Escherichia coli infection associated hemolytic-uremic syndrome (STEC-HUS) are often accompanied by none or only mild alterations of cerebral magnetic resonance imaging (MRI). This study aims to analyze if quantitative MRI is able to reveal cerebral pathological alterations invisible for conventional MRI. In nine patients with STEC-HUS associated severe neurological symptoms but inconspicuous cerebral MRI findings maps of the parameters T2 relaxation time, relative proton density (PD), apparent diffusion coefficient (ADC), and fractional anisotropy (FA) were generated. Quantitative values of these parameters were measured at the basal ganglia, thalamus, and white matter of the frontal and parietal lobe and compared to those of nine age- and sex-matched controls. Significant T2 prolongation (p < 0.01) was found in the basal ganglia of all patients compared to controls. PD and ADC were not significantly altered. A significant reduction of FA in patients was seen at caput nuclei caudati (p < 0.01). Prolonged T2 relaxation time indicates cerebral microstructural damages in these patients despite their inconspicuous MRI findings. T2 relaxometry could be used as a complementary tool for the assessment of metabolic-toxic brain syndromes. (orig.)

  6. Long-term outcome of pregnancy complicating with severe aplastic anemia under supportive care.

    Science.gov (United States)

    Chen, Kuan-Ju; Chang, Yao-Lung; Chang, Horng; Su, Shen-Yuan; Peng, Hsiu-Huei; Chang, Shuenn-Dyh; Chao, An-Shine

    2017-10-01

    Pregnancy associated with aplastic anemia (AA) is a rare and heterogeneous disorder. We aimed to identify and evaluate the maternal and pregnant outcomes of pregnancy-associated severe AA treated with supportive care. A 25-year retrospective study was conducted at in a single center between 1990 and 2014 with pregnancy associated severe AA. In addition, relevant published cases of antenatally diagnosed pregnancy-associated severe AA after 1990 were identified by PubMed. The main goal was to determine the impact of various risk factors on maternal and fetal outcomes. 15 women with 18 pregnancies were enrolled. With addition of the published reports in literature, a total of 36 cases were included for reference review. Univariate analysis showed that low platelet counts (<2.0 × 10 9 /L), bone marrow hypocellularity (<25%), and late diagnosis during pregnancy were predictors of poor maternal outcomes (P < 0.05). The complication rate of pregnancy outcomes was 53.3%, including preterm delivery, small gestational age (SGA), preterm premature ruptured of membranes (PPROM) and preeclampsia. This study identified the risk factors of mortality and morbidity in pregnant women with severe AA, as well as the obstetrical complications associated with neonatal outcome. Copyright © 2017. Published by Elsevier B.V.

  7. Hemolytic disease of the fetus and newborn due to anti-Ge3: combined antibody-dependent hemolysis and erythroid precursor cell growth inhibition.

    Science.gov (United States)

    Blackall, Douglas P; Pesek, Gina D; Montgomery, Matthew M; Oza, Krishna K; Arndt, Patricia A; Garratty, George; Shahcheraghi, Ali; Denomme, Gregory A

    2008-10-01

    The Gerbich (Ge) antigens are a collection of high-incidence antigens carried on the red blood cell membrane glycoproteins, glycophorins C and D. Antibodies against these antigens are uncommon, and there have been only rare case reports of hemolytic disease of the fetus and newborn due to anti-Ge. In this case report, we present a neonate with severe anemia and hyperbilirubinemia due to anti-Ge3. Routine and special laboratory studies undertaken in this case suggested two mechanisms for the patient's hemolysis and persistent anemia. Antibody-dependent hemolysis was associated with early-onset hyperbilirubinemia, anemia, and a mild reticulocytosis, and inhibition of erythroid progenitor cell growth was associated with late anemia and normal bilirubin and reticulocyte values. Though rare, anti-Ge3 can be a dangerous antibody in pregnancy. Affected neonates may require intensive initial therapy and close follow-up for at least several weeks after delivery.

  8. Hemolytic uremic syndrome and hypertensive crisis post dengue hemorrhagic fever: a case report

    Directory of Open Access Journals (Sweden)

    Mervin Tri Hadianto

    2011-12-01

    Full Text Available Hemolytic-uremic syndrome (HUS clinically manifests as acute renal failure, hemolytic anemia and thrombocytopenia. Acute renal failure with oliguria, hypertension, and proteinuria usually develops in affected patients.1,2 In children under 15 years of age, typical HUS occurs at a rate of 0.91 cases per 100,000 population.3 The initial onset of this disease usually happens in children below 3 years of age. Incidence is similar in boys and girls. Seasonal variation occurs, with HUS peaking in the summer and fall. In young children, spontaneous recovery is common. In adults, the probability of recovery is low when HUS is associated with severe hypertension.2

  9. [High-throughput genotyping multiplex ligation-dependent probe amplification for assisting diagnosis in a case of anti-Di(a)-induced severe hemolytic disease of the newborn].

    Science.gov (United States)

    Ji, Yanli; Mo, Chunyan; Wei, Ling; Zhou, Xiuzhen; Zhang, Runqing; Zhao, Yang; Luo, Hong; Wang, Zhen; Luo, Guangping

    2012-02-01

    To report a rare case of hemolytic disease of the newborn (HDN) with kernicterus caused by anti-Di(a) diagnosed using high-throughput genotyping multiplex ligation-dependent probe amplification (MLPA). Conventional serological methods were used to detect the antibodies related with HDN. The genotypes of more than 40 red blood cell antigens for the newborn and her parents were obtained using the high-throughput MLPA assay. The antibody titers were tested using a standard serological method. The unknown antibody against the low-frequency antigens was predicted based on the primary serological tests. The genotyping results for more than 40 red blood cell antigens of the newborn and her parents showed incompatible antigens of MNS and Diego blood group system, indicating the existence of anti-N or anti-Di(a). Further serological tests confirmed anti-Di(a) existence in the plasma of the newborn and her mother. The titer of anti-Di(a) in the mother's plasma was 1:32. Severe HDN including kernicterus can result from anti-Di(a). High-throughput genotyping MLPA assay can help type some rare antigens in complicated cases. The reagent red cell panels including Di(a)-positive cells are necessary in routine antibody screening test in Chinese population.

  10. Loss-of-Function FANCL Mutations Associate with Severe Fanconi Anemia Overlapping the VACTERL Association.

    Science.gov (United States)

    Vetro, Annalisa; Iascone, Maria; Limongelli, Ivan; Ameziane, Najim; Gana, Simone; Della Mina, Erika; Giussani, Ursula; Ciccone, Roberto; Forlino, Antonella; Pezzoli, Laura; Rooimans, Martin A; van Essen, Antoni J; Messa, Jole; Rizzuti, Tommaso; Bianchi, Paolo; Dorsman, Josephine; de Winter, Johan P; Lalatta, Faustina; Zuffardi, Orsetta

    2015-05-01

    The diagnosis of VACTERL syndrome can be elusive, especially in the prenatal life, due to the presence of malformations that overlap those present in other genetic conditions, including the Fanconi anemia (FA). We report on three VACTERL cases within two families, where the two who arrived to be born died shortly after birth due to severe organs' malformations. The suspicion of VACTERL association was based on prenatal ultrasound assessment and postnatal features. Subsequent chromosome breakage analysis suggested the diagnosis of FA. Finally, by next-generation sequencing based on the analysis of the exome in one family and of a panel of Fanconi genes in the second one, we identified novel FANCL truncating mutations in both families. We used ectopic expression of wild-type FANCL to functionally correct the cellular FA phenotype for both mutations. Our study emphasizes that the diagnosis of FA should be considered when VACTERL association is suspected. Furthermore, we show that loss-of-function mutations in FANCL result in a severe clinical phenotype characterized by early postnatal death. © 2015 WILEY PERIODICALS, INC.

  11. Association of severe thrombocytopenia and poor prognosis in pregnancies with aplastic anemia.

    Directory of Open Access Journals (Sweden)

    Jae Eun Shin

    Full Text Available PURPOSE: We sought to estimate the risks of adverse obstetric outcomes and disease outcomes associated with severe thrombocytopenia in pregnant women with aplastic anemia (AA. METHODS: In a retrospective study, we compared demographics, clinical characteristics, laboratory results, and outcomes between severe thrombocytopenia (ST and non-severe thrombocytopenia (non-ST groups comprising pregnant women with AA. RESULTS: Of 61 AA patients, 43 (70% were diagnosed as AA before pregnancy and 18 (30% were AA during pregnancy. The ST group exhibited lower gestational age at nadir of platelet count (26.0 versus 37.0 weeks, p<0.001 and at delivery (37.3 versus 39.1 weeks, p = 0.008, and a higher rate of bleeding gums (33.8 versus 7.7%, p = 0.015 than the non-ST group. In addition, the ST group exhibited more transfusions during pregnancy (72.7 versus 15.4%, p<0.001 and postpartum period (45.0 versus 2.7%, p<0.001, and more bone marrow transplant after delivery (25.0 versus 0.0%, p<0.001 than the non-ST group. The ST group had a higher odds ratio of composite disease complications (OR, 9.63; 95% CI, 2.82-32.9; p<0.001 and composite obstetric complications (OR, 6.78; 95% CI, 2.11-21.8; p = 0.001 than the non-ST group. CONCLUSIONS: Severe thrombocytopenia is more associated with obstetric and disease complications than is non-severe thrombocytopenia in pregnant women with AA.

  12. Diagnóstico laboratorial da anemia hemolítica auto-imune: características do teste manual direto do PolybreneÒ Laboratory diagnosis of auto-immune hemolytic anemia: characteristics of the manual direct test of PolybreneTM

    Directory of Open Access Journals (Sweden)

    G.W. Braga

    1998-03-01

    Full Text Available O teste manual direto do PolybreneÒ (TDP e o teste de Coombs direto (TCD foram utilizados para a detecção de IgG na superfície de hemácias de pacientes com diagnóstico clínico e laboratorial de anemia hemolítica auto-imune (AHAI. OBJETIVO: Comparar a sensibilidade e especificidade do TPD e do TCD no diagnóstico da AHAI. MÉTODO: Foram estudados 18 pacientes com diagnóstico clínico-laboratorial de AHAI. Como indivíduos controles, foram testados 20 doadores de sangue assintomáticos e 20 pacientes com anemia falciforme. RESULTADOS: O TCD foi positivo em 14 pacientes e negativo em quatro indivíduos, enquanto o TDP foi positivo em 17 pacientes e negativo em um indivíduo que apresentava TCD positivo devido a fixação de complemento (C3d nas hemácias. Todos os eluatos positivos realizados com a técnica de diclorometano revelaram anticorpos quentes com especificidade "anti-Rh". A sensibilidade do TDP (94% para detectar fixação de IgG in vivo foi significantemente maior (pThe direct manual PolybreneTM test (DPT and the direct antiglobulin tests (DAT were employed to detect antibody sensitizing red blood cell (RCB in patients with clinical and laboratorial findings of autoimmune hemolytic anemia (AIHA. PURPOSE: To compare the sensitivity and specificity of DPT and DAT in the diagnosis of AIHA. METHODS: Eighteen consecutive patients with diagnosis of AIHA were evaluated. The control group consisted of 20 normal volunteers blood donors and 20 patients with sickle cell anemia. All patients and controls were submitted to DPT and DAT. All DAT positive samples were further tested using monospecific reagents ( anti-IgG heavy chain and anti-C3d. Positive samples for either DPT or DAT were evaluated by eluate technique using. The dichloromethane (DCM. RESULTS: The DAT was positive in 14 patients and negative in 4 subjects, while the DPT was positive in 17 patients and negative in 1 individual who had a positive DAT owing to complement (C3d. All

  13. Hemolysis is associated with low reticulocyte production index and predicts blood transfusion in severe malarial anemia.

    Directory of Open Access Journals (Sweden)

    Rolf Fendel

    Full Text Available BACKGROUND: Falciparum Malaria, an infectious disease caused by the apicomplexan parasite Plasmodium falciparum, is among the leading causes of death and morbidity attributable to infectious diseases worldwide. In Gabon, Central Africa, one out of four inpatients have severe malarial anemia (SMA, a life-threatening complication if left untreated. Emerging drug resistant parasites might aggravate the situation. This case control study investigates biomarkers of enhanced hemolysis in hospitalized children with either SMA or mild malaria (MM. METHODS AND FINDINGS: Ninety-one children were included, thereof 39 SMA patients. Strict inclusion criteria were chosen to exclude other causes of anemia. At diagnosis, erythrophagocytosis (a direct marker for extravascular hemolysis, EVH was enhanced in SMA compared to MM patients (5.0 arbitrary units (AU (interquartile range (IR: 2.2-9.6 vs. 2.1 AU (IR: 1.3-3.9, p<0.01. Furthermore, indirect markers for EVH, (i.e. serum neopterin levels, spleen size enlargement and monocyte pigment were significantly increased in SMA patients. Markers for erythrocyte ageing, such as CD35 (complement receptor 1, CD55 (decay acceleration factor and phosphatidylserine exposure (annexin-V-binding were investigated by flow cytometry. In SMA patients, levels of CD35 and CD55 on the red blood cell surface were decreased and erythrocyte removal markers were increased when compared to MM or reconvalescent patients. Additionally, intravascular hemolysis (IVH was quantified using several indirect markers (LDH, alpha-HBDH, haptoglobin and hemopexin, which all showed elevated IVH in SMA. The presence of both IVH and EVH predicted the need for blood transfusion during antimalarial treatment (odds ratio 61.5, 95% confidence interval (CI: 8.9-427. Interestingly, this subpopulation is characterized by a significantly lowered reticulocyte production index (RPI, p<0.05. CONCLUSIONS: Our results show the multifactorial pathophysiology of SMA

  14. Early intravenous immunoglobin (two-dose regimen) in the management of severe Rh hemolytic disease of newborn--a prospective randomized controlled trial.

    Science.gov (United States)

    Elalfy, Mohsen Saleh; Elbarbary, Nancy Samir; Abaza, Heba Wegdan

    2011-04-01

    Phototherapy is the standard treatment in moderately severe hemolytic disease of newborn (HDN), whereas exchange transfusion (ET) is the second line in progressive cases. Intravenous immunoglobin (IVIG) has been suggested to decrease the need for ET. We aimed at assessing the efficacy of early two-dose regimens of IVIG to avoid unnecessary ET in severe Rh HDN. The study included 90 full-term neonates with Rh incompatibility unmodified by antenatal treatment and not eligible for early ET and which were randomly assigned into one of three groups: group (I), treated by conventional method; groups IIa and IIb received IVIG once at 12 h postnatal age if PT was indicated, in a dose of 0.5 and 1 g/kg, respectively. Analysis revealed 11 neonates (22%) in the conventional group and 2 (5%) in the intervention group who administered low-dose IVIG at 12 h, while none in group IIb required exchange transfusion (p = 0.03). Mean bilirubin levels were significantly lower during the first 96 h in the intervention group compared to the conventional group (p < 0.0001). Shorter duration of phototherapy (52.8 ± 12.39 h) and hospital stay (3.25 ± 0.71 days) in the IVIG group compared to conventional group (84 ± 12.12 h and 4.72 ± 0.78 days, p < 0.0001, respectively) were observed. We conclude that IVIG administration at 12 h was effective in the treatment of severe Rh HDN; the low-dose IVIG (0.5 g/kg) was as effective as high dose (1 g/kg) in reducing the duration of phototherapy and hospital stay, but less effective in avoiding exchange transfusion.

  15. The first description of severe anemia associated with acute kidney injury and adult minimal change disease: a case report

    Directory of Open Access Journals (Sweden)

    Qian Yimei

    2009-01-01

    Full Text Available Abstract Introduction Acute kidney injury in the setting of adult minimal change disease is associated with proteinuria, hypertension and hyperlipidemia but anemia is usually absent. Renal biopsies exhibit foot process effacement as well as tubular interstitial inflammation, acute tubular necrosis or intratubular obstruction. We recently managed a patient with unique clinical and pathological features of minimal change disease, who presented with severe anemia and acute kidney injury, an association not previously reported in the literature. Case presentation A 60-year-old Indian-American woman with a history of hypertension and diabetes mellitus for 10 years presented with progressive oliguria over 2 days. Laboratory data revealed severe hyperkalemia, azotemia, heavy proteinuria and progressively worsening anemia. Urine eosinophils were not seen. Emergent hemodialysis, erythropoietin and blood transfusion were initiated. Serologic tests for hepatitis B, hepatitis C, anti-nuclear antibodies, anti-glomerular basement membrane antibodies and anti-neutrophil cytoplasmic antibodies were negative. Complement levels (C3, C4 and CH50 were normal. Renal biopsy unexpectedly displayed 100% foot process effacement. A 24-hour urine collection detected 6.38 g of protein. Proteinuria and anemia resolved during six weeks of steroid therapy. Renal function recovered completely. No signs of relapse were observed at 8-month follow-up. Conclusion Adult minimal change disease should be considered when a patient presents with proteinuria and severe acute kidney injury even when accompanied by severe anemia. This report adds to a growing body of literature suggesting that in addition to steroid therapy, prompt initiation of erythropoietin therapy may facilitate full recovery of renal function in acute kidney injury.

  16. Exchange transfusion of least incompatible blood for severe hemolytic disease of the newborn due to anti-Rh17.

    Science.gov (United States)

    Li, Bi-juan; Jiang, Yuan-jun; Yuan, Fen; Ye, Hong-xing

    2010-02-01

    HDN attributed to the rare Rh variants has become more and more significant caused by anti-D, but the compatible blood is usually very difficult to obtain when exchange transfusion is required. We treated a 10-hour neonate of O, D + C + c - E - e+ blood group with severe HDN due to anti-Rh17 with least incompatible blood typed O, D + C - c + E + e-. The neonatal hemolysis was relieved obviously and bilirubin was reduced gradually after exchange transfusion. The infant was discharged in good health 13 days after birth with 135.0 g/L, 28.0 micromol/L and 10.7 micromol/L of Hb, total bilirubin and direct bilirubin, respectively. No sequelae were observed in a three-year follow-up. The result suggesting that the least incompatible blood is an alternative choice for exchange transfusion in severe HDN due to anti-Rh17 in case that Rh17 antigen-negative blood is unavailable.

  17. Shigella sonnei and hemolytic uremic syndrome: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Casey Adams

    2017-01-01

    Full Text Available Hemolytic uremic syndrome (HUS is a well-described process that is known to cause severe renal dysfunction, thrombocytopenia, and anemia. HUS is typically associated with toxins (shiga-like and shigella toxin found in strains of E. coli and Shigella spp [1–3]. We present a case of a 27 year-old man with jaundice, thrombocytopenia, and renal dysfunction who was found to have HUS in the setting of Shigella sonnei infection. Outside of developing countries, cases of HUS related to S. sonnei are largely unreported.

  18. NEW DATA REGARDING THE DEGREES OF SEVERITY OF ANEMIA IN MULTIPLE MYELOMA

    Directory of Open Access Journals (Sweden)

    Mihai Bularda

    2013-08-01

    Full Text Available This paper presents the results of a comparative analysis on the severity of anemia, in the light of the depletion of hemoglobin (Hb levels, hematocrit (Ht and of number of erythrocytes (E from multiple myeloma patients’ blood, depending on gender and age. The analysis was done on four groups of patients, men and women, aged under 60 and even 60. The hematologic failure highlighted in these patients was marked by a decrease in all variants up to 58,7-90,6% of the  normal physiological mean values of Hb levels, up to 57,4-80% of  Ht levels and up to 59,2-88,4% of red cells numbers. Moreover, it has been noticed that both in the case of Hb and Ht and E, the weighting factor of patients with normal individual values is null  (100% subnormal values in men aged under 60. It increases up to 9.1% in patients aged over 60. When it comes to women, the weighting factor of normal individual values reaches 18,2 - 45,5 % of total number of studied cases.

  19. Blueberry muffin rash, hyperbilirubinemia, and hypoglycemia: a case of hemolytic disease of the fetus and newborn due to anti-Kp(a).

    Science.gov (United States)

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

    2011-05-01

    Hemolytic disease of the fetus and newborn occurs when maternal IgG antibodies cross the placenta and cause hemolysis of fetal red blood cells. Kp(a) 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-Kp(a) have typically had minimal clinical consequences. We report a critically ill neonate who presented with purpura, respiratory failure, severe liver dysfunction, hyperbilirubinemia, hypoglycemia and anemia. This case report broadens the spectrum of neonatal disease associated with anti-Kp(a), addresses the evaluation of hemolysis with liver failure in a neonate, and emphasizes the importance of screening for antibodies to low frequency red blood cell antigens in suspected hemolytic disease of the fetus and newborn.

  20. Pernicious anemia

    Science.gov (United States)

    ... malabsorption); Anemia - intrinsic factor; Anemia - IF; Anemia - atrophic gastritis ... of pernicious anemia include: Weakened stomach lining (atrophic gastritis) An autoimmune condition in which the body's immune ...

  1. Comparable Outcomes after HLA-Matched Sibling and Alternative Donor Hematopoietic Cell Transplantation for Children with Fanconi Anemia and Severe Aplastic Anemia.

    Science.gov (United States)

    Ebens, Christen L; DeFor, Todd E; Tryon, Rebecca; Wagner, John E; MacMillan, Margaret L

    2018-04-01

    Fanconi anemia (FA)-associated severe aplastic anemia (SAA) requires allogeneic hematopoietic cell transplantation (HCT) for cure. With the evolution of conditioning regimens over time, outcomes of alternative donor HCT (AD-HCT) have improved dramatically. We compared outcomes of HLA-matched sibling donor HCT (MSD-HCT; n = 17) and AD-HCT (n = 57) performed for FA-associated SAA at a single institution between 2001 and 2016. Overall survival at 5 years was 94% for MSD-HCT versus 86% for AD-HCT, neutrophil engraftment was 100% versus 95%, platelet recovery was 100% versus 89%, grade II-IV acute graft-versus-host disease (GVHD) was 6% versus 12%, grade III-IV acute GVHD was 6% versus 4%, and chronic GVHD was 0 versus 7%, with no statistically significant differences by type of transplant. The use of UCB was associated with decreased rates of neutrophil recovery in AD-HCT and platelet recovery in both MSD-HCT and AD-HCT. A trend toward a higher serious infection density before day +100 post-HCT was observed in AD-HCT compared with MSD-HCT (P = .02). These data demonstrate that AD-HCT should be considered at the same time as MSD-HCT for patients with FA-associated SAA. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  2. Current outcome of HLA identical sibling versus unrelated donor transplants in severe aplastic anemia

    DEFF Research Database (Denmark)

    Bacigalupo, Andrea; Socié, Gerard; Hamladji, Rose Marie

    2015-01-01

    We have analyzed 1448 patients with acquired aplastic anemia grafted between 2005 and 2009, and compared outcome of identical sibling (n=940) versus unrelated donor (n=508) transplants. When compared to the latter, sibling transplants were less likely to be performed beyond 180 days from diagnosis.......04). In conclusion, in multivariate analysis, the outcome of unrelated donor transplants for acquired aplastic anemia, is currently not statistically inferior when compared to sibling transplants, although patients are at greater risk of acute and chronic graft-versus-host disease. The use of peripheral blood grafts...

  3. Total lymphoid irradiation based conditioning for hematopoietic stem cell transplantation in severe aplastic anemia

    International Nuclear Information System (INIS)

    Lee, Yun Hee; Kim, Ji Yoon; Choi, Byung Ock; Ryu, Mi Ryeong; Chung, Su Mi

    2012-01-01

    To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

  4. Total lymphoid irradiation based conditioning for hematopoietic stem cell transplantation in severe aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yun Hee; Kim, Ji Yoon; Choi, Byung Ock; Ryu, Mi Ryeong; Chung, Su Mi [Dept. of Radiation Oncology, The Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

    2012-12-15

    To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

  5. Hemolytic uremic syndrome after bone marrow transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Arai, Ayako; Sakamaki, Hisashi; Tanikawa, Shu [Tokyo Metropolitan Komagome Hospital (Japan)] [and others

    1998-06-01

    One hundred and thirteen patients who underwent autologous or allogeneic bone marrow transplantation (BMT) were investigated for the subsequent development of hemolytic uremic syndrome (HUS). HUS developed in seven patients (four males and three females, five acute lymphocytic leukemia (ALL), one acute myelogenous leukemia, one non-Hodgkin`s lymphoma) between 36-196 days after BMT. Four patients were recipients of autologous BMT and three were those of allogeneic BMT. Six patients were preconditioned with the regimens including fractionated total body irradiation (TBI). ALL and preconditioning regimen with TBI were suspected to be the risk factors for the development of HUS. Cyclosporin A (CSP) administration was discontinued in three patients who had been given CSP for graft-versus-host disease prophylaxis. Predonisolone was given to the three patients and plasma exchange was performed in one patient. Both hemolytic anemia and thrombocytopenia were resolved in virtually all patients, while creatinine elevation has persisted along with hypertension in one patient. (author)

  6. Unrelated alternative donor transplantation for severe acquired aplastic anemia: a study from the French Society of Bone Marrow Transplantation and Cell Therapies and the EBMT Severe Aplastic Anemia Working Party.

    Science.gov (United States)

    Devillier, Raynier; Dalle, Jean-Hugues; Kulasekararaj, Austin; D'aveni, Maud; Clément, Laurence; Chybicka, Alicja; Vigouroux, Stéphane; Chevallier, Patrice; Koh, Mickey; Bertrand, Yves; Michallet, Mauricette; Zecca, Marco; Yakoub-Agha, Ibrahim; Cahn, Jean-Yves; Ljungman, Per; Bernard, Marc; Loiseau, Pascale; Dubois, Valérie; Maury, Sébastien; Socié, Gérard; Dufour, Carlo; Peffault de Latour, Regis

    2016-07-01

    Unrelated allogeneic transplantation for severe aplastic anemia is a treatment option after immunosuppressive treatment failure in the absence of a matched sibling donor. Age, delay between disease diagnosis and transplantation, and HLA matching are the key factors in transplantation decisions, but their combined impact on patient outcomes remains unclear. Using the French Society of Bone Marrow Transplantation and Cell Therapies registry, we analyzed all consecutive patients (n=139) who underwent a first allogeneic transplantation for idiopathic severe aplastic anemia from an unrelated donor between 2000 and 2012. In an adjusted multivariate model, age over 30 years (Hazard Ratio=2.39; P=0.011), time from diagnosis to transplantation over 12 months (Hazard Ratio=2.18; P=0.027) and the use of a 9/10 mismatched unrelated donor (Hazard Ratio=2.14; P=0.036) were independent risk factors that significantly worsened overall survival. Accordingly, we built a predictive score using these three parameters, considering patients at low (zero or one risk factors, n=94) or high (two or three risk factors, n=45) risk. High-risk patients had significantly shorter survival (Hazard Ratio=3.04; Paplastic anemia. Copyright© Ferrata Storti Foundation.

  7. Inborn anemias in mice. Progress report to accompany twenty-first renewal proposal, 1 May 1975--30 April 1976

    Energy Technology Data Exchange (ETDEWEB)

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

    1976-05-15

    Progress is reported on studies on hereditary anemias of mice. At present under study are four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, and the autoimmune hemolytic anemia of NZB. Each of these blood dyscrasias is caused by the action of a unique mutant gene, each of 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.

  8. Upfront haploidentical transplant for acquired severe aplastic anemia: registry-based comparison with matched related transplant.

    Science.gov (United States)

    Xu, Lan-Ping; Jin, Song; Wang, Shun-Qing; Xia, Ling-Hui; Bai, Hai; Gao, Su-Jun; Liu, Qi-Fa; Wang, Jian-Min; Wang, Xin; Jiang, Ming; Zhang, Xi; Wu, De-Pei; Huang, Xiao-Jun

    2017-01-21

    Haploidentical donor (HID) hematopoietic stem cell transplantation (HSCT) is an alternative treatment method for severe aplastic anemia (SAA) patients lacking suitable identical donors and those who are refractory to immunosuppressive therapy (IST). The current study evaluated the feasibility of upfront haploidentical HSCT in SAA patients. We conducted a multicenter study based on a registry database. One hundred fifty-eight SAA patients who underwent upfront transplantation between June 2012 and September 2015 were enrolled. Eighty-nine patients had haploidentical donors (HIDs), and 69 had matched related donors (MRDs) for HSCT. The median times for myeloid engraftment in the HID and MRD cohorts were 12 (range, 9-20) and 11 (range, 8-19) days, with a cumulative incidence of 97.8 and 97.1% (P = 0.528), respectively. HID recipients had an increased cumulative incidence of grades II-IV acute graft-versus-host disease (aGVHD) (30.3 vs. 1.5%, P < 0.001), grades III-IV aGVHD (10.1 vs. 1.5%, P = 0.026), and chronic GVHD (cGVHD) (30.6 vs. 4.4%, P < 0.001) at 1 year but similar extensive cGVHD (3.4 vs. 0%, P = 0.426). The three-year estimated overall survival (OS) rates were 86.1 and 91.3% (P = 0.358), while the three-year estimated failure-free survival (FFS) rates were 85.0 and 89.8% (P = 0.413) in the HID and MRD cohorts, respectively. In multivariate analysis, survival outcome for the entire population was significantly adversely associated with increased transfusions and poor performance status pre-SCT. We did not observe differences in primary engraftment and survival outcomes by donor type. Haploidentical SCT as upfront therapy was an effective and safe option for SAA patients, with favorable outcomes in experienced centers.

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... contribute to differences in disease severity and how patients respond to treatment. The NHLBI Strategic Vision highlights ... Anemia in Chronic Kidney Disease (National Institute of Diabetes and Digestive and Kidney Diseases) Avoiding Anemia (National ...

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

    Directory of Open Access Journals (Sweden)

    Venu Jain

    2013-05-01

    Full Text Available 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.

  11. Major Burden of Severe Anemia from Non-Falciparum Malaria Species in Southern Papua: A Hospital-Based Surveillance Study

    Science.gov (United States)

    Douglas, Nicholas M.; Lampah, Daniel A.; Kenangalem, Enny; Simpson, Julie A.; Poespoprodjo, Jeanne R.; Sugiarto, Paulus; Anstey, Nicholas M.; Price, Ric N.

    2013-01-01

    Background The burden of anemia attributable to non-falciparum malarias in regions with Plasmodium co-endemicity is poorly documented. We compared the hematological profile of patients with and without malaria in southern Papua, Indonesia. Methods and Findings Clinical and laboratory data were linked for all patients presenting to a referral hospital between April 2004 and December 2012. Data were available on patient demographics, malaria diagnosis, hemoglobin concentration, and clinical outcome, but other potential causes of anemia could not be identified reliably. Of 922,120 patient episodes (837,989 as outpatients and 84,131 as inpatients), a total of 219,845 (23.8%) were associated with a hemoglobin measurement, of whom 67,696 (30.8%) had malaria. Patients with P. malariae infection had the lowest hemoglobin concentration (n = 1,608, mean = 8.93 [95% CI 8.81–9.06]), followed by those with mixed species infections (n = 8,645, mean = 9.22 [95% CI 9.16–9.28]), P. falciparum (n = 37,554, mean = 9.47 [95% CI 9.44–9.50]), and P. vivax (n = 19,858, mean = 9.53 [95% CI 9.49–9.57]); p-value for all comparisons anemia (hemoglobin anemia (adjusted odds ratio [AOR] 3.25 [95% CI 2.99–3.54]); AORs for severe anaemia associated with P. falciparum, P. vivax, and P. malariae were 2.11 (95% CI 2.00–2.23), 1.87 (95% CI 1.74–2.01), and 2.18 (95% CI 1.76–2.67), respectively, panemia was attributable to non-falciparum infections compared with 15.1% (95% CI 13.9%–16.3%) for P. falciparum monoinfections. Patients with severe anemia had an increased risk of death (AOR = 5.80 [95% CI 5.17–6.50]; panemia in early infancy, mixed P. vivax/P. falciparum infections are associated with a greater hematological impairment than either species alone, and in adulthood P. malariae, although rare, is associated with the lowest hemoglobin concentration. These findings highlight the public health importance of integrated genus-wide malaria

  12. Successful management of severe hemolytic disease of the fetus due to anti-Jsb using intrauterine transfusions with serial maternal blood donations: a case report and a review of the literature.

    Science.gov (United States)

    Al Riyami, Arwa Z; Al Salmani, Moza; Al Hashami, Sabria; Al Mahrooqi, Sabah; Al Hinai, Sumaiya; Al Balushi, Halima; Al Riyami, Nihal; Gowri, V; Al Dughaishi, Tamima; Al Hosni, Saif; Al-Khabori, Murtadha; Al-Farsi, Khalil; Al Huneini, Mohammed; Alkindi, Salam

    2014-01-01

    The management of pregnant women with anti-Jsb is challenging due to the paucity of antigen-negative blood for fetal and neonatal transfusion. A 29-year-old woman with anti-Jsb was referred for assessment of recurrent fetal losses. With the presence of the sister as a historically matched donor, she was planned for active surveillance for fetal anemia during pregnancy. The fetus remained well until 21 weeks of gestation when signs of fetal anemia and early hydrops fetalis were noted. Anti-Jsb titer was at 128. The sister's red blood cells (RBCs) were cross-match incompatible. Urgent intrauterine transfusion (IUT) was performed with washed irradiated maternal RBCs, donated after cessation of heparin. The mother was given intravenous iron (IV-Fe) and continued on weekly recombinant human erythropoietin (rHu-EPO). Repeated IUTs were needed every 1 to 3 weeks. Throughout a 7-week period, three maternal donations were performed with total donated whole blood volume of 1250 mL, supporting four IUTs. At 29 weeks of gestation, the procedure was complicated by umbilical cord hematoma necessitating urgent cesarean section. A male newborn was delivered, transfused at birth, and subsequently treated with phototherapy and five top-up transfusions. This case represents a successful example of managing hemolytic disease of the fetus due to a rare antibody using maternal blood. It also supports previous data on safety of maternal donations during pregnancy and the use of combination of rHu-EPO and IV-Fe as a supportive measure. © 2013 American Association of Blood Banks.

  13. First-Line Matched Related Donor Hematopoietic Stem Cell Transplantation Compared to Immunosuppressive Therapy in Acquired Severe Aplastic Anemia

    Science.gov (United States)

    Peinemann, Frank; Grouven, Ulrich; Kröger, Nicolaus; Bartel, Carmen; Pittler, Max H.; Lange, Stefan

    2011-01-01

    Introduction Acquired severe aplastic anemia (SAA) is a rare and progressive disease characterized by an immune-mediated functional impairment of hematopoietic stem cells. Transplantation of these cells is a first-line treatment option if HLA-matched related donors are available. First-line immunosuppressive therapy may be offered as alternative. The aim was to compare the outcome of these patients in controlled trials. Methods A systematic search was performed in the bibliographic databases MEDLINE, EMBASE, and The Cochrane Library. To show an overview of various outcomes by treatment group we conducted a meta-analysis on overall survival. We evaluated whether studies reported statistically significant factors for improved survival. Results 26 non-randomized controlled trials (7,955 patients enrolled from 1970 to 2001) were identified. We did not identify any RCTs. Risk of bias was high except in 4 studies. Young age and recent year of treatment were identified as factors for improved survival in the HSCT group. Advanced age, SAA without very severe aplastic anemia, and combination of anti-lymphocyte globulin with cyclosporine A were factors for improved survival in the IST group. In 19 studies (4,855 patients), summary statistics were sufficient to be included in meta-analysis. Considerable heterogeneity did not justify a pooled estimate. Adverse events were inconsistently reported and varied significantly across studies. Conclusions Young age and recent year of treatment were identified as factors for improved survival in the transplant group. Advanced age, SAA without very severe aplastic anemia, and combination of anti-lymphocyte globulin with cyclosporine A were factors for improved survival in the immunosuppressive group. Considerable heterogeneity of non-randomized controlled studies did not justify a pooled estimate. Adverse events were inconsistently reported and varied significantly across studies. PMID:21541024

  14. Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    Hyun Jin Kim

    2017-03-01

    Full Text Available Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL, respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL. She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.

  15. Tissue Factor and Thrombin in Sickle Cell Anemia

    OpenAIRE

    Chantrathammachart, Pichika; Pawlinski, Rafal

    2012-01-01

    Sickle cell anemia is an inherited hematologic disorder associated with hemolytic and vaso-occlusive complications. An activation of coagulation is also a prominent feature of sickle cell anemia. Growing evidence indicates that coagulation may contribute to the inflammation and vascular injury in sickle cell anemia. This review focuses on tissue factor expression and its contribution to the activation of coagulation, thrombosis and vascular inflammation in sickle cell anemia.

  16. Severe Aplastic Anemia following Acute Hepatitis from Toxic Liver Injury: Literature Review and Case Report of a Successful Outcome

    Directory of Open Access Journals (Sweden)

    Kamran Qureshi

    2014-01-01

    Full Text Available Hepatitis associated aplastic anemia (HAAA is a rare syndrome in which severe aplastic anemia (SAA complicates the recovery of acute hepatitis (AH. HAAA is described to occur with AH caused by viral infections and also with idiopathic cases of AH and no clear etiology of liver injury. Clinically, AH can be mild to fulminant and transient to persistent and precedes the onset SAA. It is assumed that immunologic dysregulation following AH leads to the development of SAA. Several observations have been made to elucidate the immune mediated injury mechanisms, ensuing from liver injury and progressing to trigger bone marrow failure with the involvement of activated lymphocytes and severe T-cell imbalance. HAAA has a very poor outcome and often requires bone marrow transplant (BMT. The findings of immune related myeloid injury implied the use of immunosuppressive therapy (IST and led to improved survival from HAAA. We report a case of young male who presented with AH resulting from the intake of muscle building protein supplements and anabolic steroids. The liver injury slowly resolved with supportive care and after 4 months of attack of AH, he developed SAA. He was treated with IST with successful outcome without the need for a BMT.

  17. Acute myocardial infarction and pulmonary embolism in a young man with pernicious anemia-induced severe hyperhomocysteinemia

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    Hofmann Marion A

    2009-05-01

    Full Text Available Abstract A 27 year-old man who presented to the hospital with progressive lower extremity weakness, developed an acute ST elevation myocardial infarction on his second hospital day. Primary angioplasty to the left anterior descending coronary artery was performed. Due to persistent dyspnea, the patient underwent a diagnostic chest computed tomography which confirmed multiple small pulmonary emboli. Laboratory analysis revealed a megaloblastic anemia with a reduced vitamin B12 level and positive titers for antibodies against intrinsic factor, establishing a diagnosis of pernicious anemia. Screening for hypercoaguable markers documented an isolated severely elevated homocysteine levels (105 μmol/l. No other significant risk factors for coronary artery disease including a family history of premature atherosclerosis were identified. This case illustrates the importance of testing for hyperhomocysteinemia as part of a workup for atherothrombotic disease, especially in patients without other significant risk factors. The severity of hyperhomocysteinemia found in our patient is unusual for patients with vitamin B12 malabsorption and raises the question of whether the widely practiced folic acid fortification in the United States may mask or even worsen vitamin B12 deficiency over time, leading to more severe cases of vitamin B12 deficiency and hyperhomocysteinemia than seen in the past.

  18. Cotransplantation of haploidentical hematopoietic and umbilical cord mesenchymal stem cells for severe aplastic anemia: Successful engraftment and mild GVHD

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    Wu Yamei

    2014-01-01

    Full Text Available Haploidentical hematopoietic stem-cell transplantation (haplo-HSCT is associated with an increased risk of graft failure and severe graft-versus-host disease (GVHD. Mesenchymal stromal cells (MSCs have been shown to support in vivo normal hematopoiesis and to display potent immunesuppressive effects. We cotransplanted the culture-expanded third-party donor-derived umbilical cord MSCs (UC-MSCs in 21 young people with severe aplastic anemia (SAA undergoing haplo-HSCT without T-cell-depleted. We observed that all patients had sustained hematopoietic engraftment without any adverse UC-MSC infusion-related events. Furthermore, we did not observe any increase in severe aGVHD. These data suggest that UC-MSCs, possibly thanks to their potent immunosuppressive effect on allo-reactive host T lymphocytes escaping the preparative regimen, reduce the risk of graft failure and severe GVHD in haplo-HSCT.

  19. A noninvasive method for the prediction of fetal hemolytic disease

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    E. N. Kravchenko

    2017-01-01

    Full Text Available Objective: to improve the diagnosis of fetal hemolytic disease.Subjects and methods. A study group consisted of 42 pregnant women whose newborn infants had varying degrees of hemolytic disease. The women were divided into 3 subgroups according to the severity of neonatal hemolytic disease: 1 pregnant women whose neonates were born with severe hemolytic disease (n = 14; 2 those who gave birth to babies with moderate hemolytic disease (n = 11; 3 those who delivered infants with mild hemolytic disease (n = 17. A comparison group included 42 pregnant women whose babies were born without signs of hemolytic disease. Curvesfor blood flow velocity in the middle cerebral artery were analyzed in a fetus of 25 to 39 weeks’ gestation.Results. The peak systolic blood flow velocity was observed in Subgroup 1; however, the indicator did not exceed 1.5 MoM even in severe fetal anemic syndrome. The fetal middle artery blood flow velocity rating scale was divided into 2 zones: 1 the boundary values of peak systolic blood flow velocity from the median to the obtained midscore; 2 the boundary values of peak systolic blood flow velocity of the obtained values of as high as 1.5 MoM.Conclusion. The value of peak systolic blood flow velocity being in Zone 2, or its dynamic changes by transiting to this zone can serve as a prognostic factor in the development of severe fetal hemolytic disease. 

  20. Characteristics of severe anemia and its association with malaria in young children of the Kassena-Nankana District of northern Ghana.

    Science.gov (United States)

    Owusu-Agyei, Seth; Fryauff, David J; Chandramohan, Daniel; Koram, Kwadwo A; Binka, Fred N; Nkrumah, Francis K; Utz, Greg C; Hoffman, Stephen L

    2002-10-01

    Severe anemia is thought to be the principal underlying cause of malaria death in areas of intense seasonal malaria transmission such as the Kassena-Nankana District of northern Ghana. Factors associated with severe anemia in young children, 6-24 months old, were elucidated by analyzing results of 2 malaria-associated anemia surveys (1996, 2000), separated by 4 years, but conducted in the same community and at the same seasonal time point. Age-adjusted comparison confirmed that the proportion of severely anemic children and overall mean hemoglobin (Hb) levels in the November 2000 sample were significantly improved over those of the 1996 sample (17.5 versus 26.4%, P = 0.03; Hb 7.5 versus 6.9 g/dL, P = 0.002). Weight-for-age Z-scores also indicated a significant improvement in the 2000 sample (-1.93 versus -2.20, P or = 6.0 g/dL, those with severe anemia (Hb < 6.0 g/dL) were older, more frequently parasitemic (odds ratio [OR], 1.60; 95% confidence interval [CI], 1.08-2.35), more often febrile (OR, 2.44; 95% CI, 1.71-3.48), and predominantly male (OR, 1.50; 95% CI, 1.05-2.13). An association was identified in both surveys between severe anemia and residence in the northern part of the district, but no clear link was observed in relation to irrigation. Blood transfusions, a likely surrogate index of severe anemia in young children, followed a distinct seasonal pattern. Evidence suggests that dramatic peaks and troughs of severe anemia are regular and possibly predictable events that may be used to gauge the health and survival of young children in this area.

  1. Selective engraftment of the granulocyte compartment after allogeneic bone marrow transplantation in a patient with severe aplastic anemia.

    Science.gov (United States)

    Barriga, F J; Legues, M E; Bertin, P

    1996-05-01

    We present a patient with severe aplastic anemia who had partial engraftment with full chimerism after allogeneic bone marrow transplantation from an HLA identical sibling. A 3-year-old girl with severe aplastic anemia (SAA) received a bone marrow transplantation (BMT) from an HLA identical brother 9 months after her diagnosis. Before BMT she was red blood cell tranfusion dependent, had an absolute neutrophil count (ANC) of 1,000-1,500 x 10(9)/1 and a platelet count of 15-19,000 x 10(9)/1. She was conditioned with 800 cGy total body irradiation (TBI) and cyclophosphamide and received 3X10(8) nucleated cells/kg. She reached an ANC of 1500 x 10(9)/1 on day +35 but her reticulocyte and platelet counts did not recover. A bone marrow aspirate and biopsy post BMT showed hypoplasia with marked decrease in megakaryocyte and red blood cell precursors. The granulocyte compartment showed a left shift with predominance of promyelocytes and myelocytes. The karyotype showed full chimerism (46,XY) with no 46,XX metaphases. This case illustrates the possibility of a bone marrow microenvironment defect as the cause of SAA.

  2. Microcephalic osteodysplastic primordial dwarfism (MOPD type I with severe anemia and MRI brain findings of MOPD type II

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    Rabah M. Shawky

    2017-10-01

    Full Text Available We report a 4 month old male, 4th in order of birth of healthy consanguineous Egyptian parents with typical characteristics of microcephalic osteodysplastic primordial dwarfism most probably belongs to type I (MOPD I. The patient had intrauterine growth retardation, sparse scalp hair, sparse eyebrows and eyelashes, high arched palate, micrognathia, low set ears, short neck, clenched fists, groove between thumb and palm of hand, arachnodactyly, flexion contractures of elbow and knee. He also had thin dry skin with marked decreased subcutaneous fat and prominent superficial veins over chest and abdomen and mild hypertrichosis over lower back and buttocks. However, the patient had severe anemia and MRI brain findings revealed global hypovolemic brain changes in the form of dilated ventricles and widened cortical sulci, multiple old vascular insults and aneurismal dilatation of right internal carotid artery (ICA which are consistent with MOPD II. Keywords: Microcephalic osteodysplastic primordial dwarfism type I, Intrauterine growth retardation, Dilatation of internal carotid artery, Severe anemia, Skeletal anomalies, Subcutaneous fat, Superficial veins on skin, Aneurysm of internal carotid artery

  3. Influence of lime juice on the severity of sickle cell anemia.

    Science.gov (United States)

    Adegoke, Samuel Ademola; Shehu, Umar Abdullahi; Mohammed, Lasisi Oluwafemi; Sanusi, Yunusa; Oyelami, Oyeku Akibu

    2013-06-01

    The pain in sickle cell anemia (SCA) is often triggered by dehydration, acidosis, and fever that are usually due to malaria. Intake of lime juice was recently demonstrated to facilitate clearance of the malaria parasite. It was therefore sought to determine whether regular intake of lime juice will ameliorate crisis, especially recurrent bone pain. In this preliminary, open-labeled, randomized study, the effects of lime juice on the clinical and some laboratory characteristics of children with SCA were tested. Among the 113 children with SCA studied in two hospitals, the 58 receiving lime treatment had lower rates of significant painful episodes than the 55 without lime (37 versus 83 crises in 6 months, and 0.64±0.11 versus 1.51±0.34 average rates per child, plime did not cause any significant side-effect. Regular intake of lime juice may be of great therapeutic and nutritional relevance in children with SCA.

  4. Nationwide survey on the use of eltrombopag in patients with severe aplastic anemia: a report on behalf of the French Reference Center for Aplastic Anemia.

    Science.gov (United States)

    Lengline, Etienne; Drenou, Bernard; Peterlin, Pierre; Tournilhac, Olivier; Abraham, Julie; Berceanu, Ana; Dupriez, Brigitte; Guillerm, Gaelle; Raffoux, Emmanuel; de Fontbrune, Flore Sicre; Ades, Lionel; Balsat, Marie; Chaoui, Driss; Coppo, Paul; Corm, Selim; Leblanc, Thierry; Maillard, Natacha; Terriou, Louis; Socié, Gerard; de Latour, Regis Peffault

    2018-02-01

    Few therapeutic options are available for patients with aplastic anemia who are ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag was recently shown to produce trilineage responses in refractory patients. However, the effects of real-life use of this drug remain unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic Anemia on patients with relapsed/refractory aplastic anemia, and patients ineligible for antithymocyte globulin or transplantation, who received eltrombopag for at least 2 months. Forty-six patients with aplastic anemia were given eltrombopag without prior antithymocyte globulin treatment (n=11) or after antithymocyte globulin administration (n=35) in a relapsed/refractory setting. Eltrombopag (median daily dose 150 mg) was introduced 17 months (range, 8-50) after the diagnosis of aplastic anemia. At last followup, 49% were still receiving treatment, 9% had stopped due to a robust response, 2% due to toxicity and 40% due to eltrombopag failure. Before eltrombopag treatment, all patients received regular transfusions. The overall rates of red blood cell and platelet transfusion independence were 7%, 33%, 46% and 46% at 1, 3, 6 months and last follow-up. Responses were slower to develop in antithymocyte treatment-naïve patients. In patients achieving transfusion independence, hemoglobin concentration and platelet counts improved by 3 g/dL (interquartile range, 1.4-4.5) and 42×10 9 /L (interquartile range, 11-100), respectively. Response in at least one lineage (according to National Institutes of Health criteria) was observed in 64% of antithymocyte treatment-naïve and 74% of relapsed/refractory patients, while trilineage improvement was observed in 27% and 34%, respectively. We found high rates of hematologic improvement and transfusion independence in refractory aplastic anemia patients but also in patients ineligible for antithymocyte globulin receiving first

  5. Effects and Predictive Factors of Immunosuppressive Therapy Combined with Umbilical Cord Blood Infusion in Patients with Severe Aplastic Anemia.

    Science.gov (United States)

    Zhang, Xia; Li, Zhangzhi; Geng, Wei; Song, Bin; Wan, Chucheng

    2018-07-01

    To investigate the efficacy and safety of umbilical cord blood (UCB) infusion (UCBI) plus immunosuppressive therapy (IST) treatment in comparison to IST treatment, as well as predictive factors for clinical responses, in severe aplastic anemia (SAA) patients. Totally, 93 patients with SAA were enrolled in this cohort study. In the IST group, rabbit antithymocyte globulin (r-ATG) combined with cyclosporine A (CsA) was administered, while in the IST+UBCI group, r-ATG, CsA, and UCB were used. After 6 months of treatment, UCBI+IST achieved a higher complete response (CR) rate (p=0.002) and an elevated overall response rate (ORR) (p=0.004), compared to IST. Regarding hematopoietic recovery at month 6, platelet responses in the UCBI+IST group were better than those in the IST group (p=0.002), and UCBI+IST treatment facilitated increasing trends in absolute neutrophil count (ANC) response (p=0.056). Kaplan-Meier curves illuminated UCBI+IST achieved faster ANC response (paplastic anemia (VSAA) and ANC could predict clinical responses as well. However, Cox proportional hazard regression indicated that VSAA (p=0.003), but not UCBI+IST, affected OS. Safety profiles showed that UCBI+IST therapy did not elevate adverse events, compared with IST treatment. UCBI+IST achieved better clinical responses and hematopoietic recovery than IST, and was well tolerated in SAA patients. © Copyright: Yonsei University College of Medicine 2018.

  6. Modified immunosuppressive therapy with porcine antilymphocyte globulin plus delayed cyclosporine A in children with severe aplastic anemia.

    Science.gov (United States)

    Cui, Qingya; Sha, Pingping; Chen, Haifei; Shen, Hongshi; Qin, Longmei; Li, Zhengyang; Wu, Tianqin; Wang, Zhaoyue

    2018-01-01

    Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CsA) is the standard treatment for children with severe aplastic anemia (SAA) with no human leukocyte antigen-matched siblings. Due to the unavailability of horse ATG in China, porcine antilymphocyte globulin (p-ALG), which is less expensive and more effective than rabbit ATG, is widely used. We sought to evaluate the efficacy and safety profile of modified IST with p-ALG plus delayed CsA at day 21 in 50 SAA children. Eighteen SAA patients who progressed from nonsevere aplastic anemia (NSAA) were classified as SAA-II; the other 32 patients were classified as SAA-I. Overall response (OR) rates at 3, 6 and 12 months were 56, 64 and 62%, respectively. The 10-year overall survival (OS) rate and disease-free survival (DFS) rate were 80 and 56%. The OR, OS and DFS rates in the SAA-I group were clearly better than those in the SAA-II group. Death rate from infection within 30 days was 4%. Modified IST with p-ALG plus delayed CsA is a reliable and well-tolerated treatment for children with SAA, and reduces early death due to infection. Modified IST is more suitable for children with SAA-I.

  7. Hemolytic disease of the newborn- anti c antibody induced hemolysis.

    Science.gov (United States)

    Murki, Srinivas; Kandraju, Hemasree; Devi, Surekha A

    2012-02-01

    Hemolytic disease in the newborn, as a cause of early jaundice, is not uncommon. This is mostly due to Rh (D), ABO incompatibility and rarely due to other minor blood group incompatibility. The authors report two cases of Rh anti c isoimmunization presenting as significant early neonatal jaundice within the 20 h of life. Both the babies were treated with intensive phototherapy. One baby underwent exchange transfusion and the other required packed cell transfusion for anemia.

  8. Outcome of Allogeneic Stem Cell Transplantation for Patients Transformed to Myelodysplastic Syndrome or Leukemia from Severe Aplastic Anemia: A Report from the MDS Subcommittee of the Chronic Malignancies Working Party and the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation

    NARCIS (Netherlands)

    Hussein, A.A.; Halkes, C.M.; Socie, G.; Tichelli, A.; Borne, P.A. von dem; Schaap, M.N.; Foa, R.; Ganser, A.; Dufour, C.; Bacigalupo, A.; Locasciulli, A.; Aljurf, M.; Peters, C.; Robin, M.; Biezen, A.A. van; Volin, L.; Witte, T.J. de; Marsh, J.; Passweg, J.R.; Kroger, N.; et al.,

    2014-01-01

    One hundred and forty patients who had undergone hematopoietic stem cell transplantation (HSCT) for myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML) transformation after treatment of severe aplastic anemia (SAA) were identified in the European Group for Blood and Marrow

  9. Hematological outcome in neonatal alloimmune hemolytic disease

    NARCIS (Netherlands)

    Rath, Mirjam Eva Aafke

    2013-01-01

    This thesis focuses on several aspects related to the hematological outcome of infants with hemolytic disease of the fetus and newborn (HDFN) due to red blood cell alloimmunization, including pathogenesis and management of the disease. The presence of leukocytopenie and thrombocytopenia support the

  10. First-line treatment for severe aplastic anemia in children: bone marrow transplantation from a matched family donor versus immunosuppressive therapy.

    Science.gov (United States)

    Yoshida, Nao; Kobayashi, Ryoji; Yabe, Hiromasa; Kosaka, Yoshiyuki; Yagasaki, Hiroshi; Watanabe, Ken-Ichiro; Kudo, Kazuko; Morimoto, Akira; Ohga, Shouichi; Muramatsu, Hideki; Takahashi, Yoshiyuki; Kato, Koji; Suzuki, Ritsuro; Ohara, Akira; Kojima, Seiji

    2014-12-01

    The current treatment approach for severe aplastic anemia in children is based on studies performed in the 1980s, and updated evidence is required. We retrospectively compared the outcomes of children with acquired severe aplastic anemia who received immunosuppressive therapy within prospective trials conducted by the Japanese Childhood Aplastic Anemia Study Group or who underwent bone marrow transplantation from an HLA-matched family donor registered in the Japanese Society for Hematopoietic Cell Transplantation Registry. Between 1992 and 2009, 599 children (younger than 17 years) with severe aplastic anemia received a bone marrow transplant from an HLA-matched family donor (n=213) or immunosuppressive therapy (n=386) as first-line treatment. While the overall survival did not differ between patients treated with immunosuppressive therapy or bone marrow transplantation [88% (95% confidence interval: 86-90) versus 92% (90-94)], failure-free survival was significantly inferior in patients receiving immunosuppressive therapy than in those undergoing bone marrow transplantation [56% (54-59) versus 87% (85-90); Paplastic anemia. Copyright© Ferrata Storti Foundation.

  11. Severe anemia in young children after high and low malaria transmission seasons in the Kassena-Nankana district of northern Ghana.

    Science.gov (United States)

    Koram, K A; Owusu-Agyei, S; Utz, G; Binka, F N; Baird, J K; Hoffman, S L; Nkrumah, F K

    2000-06-01

    Malaria and anemia accounted for 41% and 18% respectively of hospital deaths in the Kassena-Nankana district of northern Ghana during 1996. We measured hemoglobin (Hb), malaria prevalence, and anthropometric indices of 6--24-month-old infants and young children randomly selected from this community at the end of the high (May-October, n = 347) and low (November-April, n = 286) malaria transmission seasons. High transmission season is characterized by rainfall (the equivalent of 800-900 mm/yr.), while the remaining months receive less than 50 mm/yr. Severe anemia, defined as Hb < 6.0 g/dL, was 22.1% at the end of the high transmission season compared to 1.4% at the end of the low transmission season (Odds Ratio [OR] = 20.1; 95% CI: 7.1-55.3). Parasitemia was 71% and 54.3% at these time points (OR = 2.1; 95% CI: 1.5-2.9). Nutritional anemia appeared to have little impact upon this seasonal difference since anthropometric indices were comparable. Although the relative contributions of other causes of severe anemia were not assessed, repeated malaria infections may be a primary determinant of severe anemia among infants and young children during the high transmission season.

  12. A case of asymptomatic pancytopenia with clinical features of hemolysis as a presentation of pernicious anemia

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    Venkateswara K. Kollipara

    2016-09-01

    Full Text Available Pernicious anemia is an autoimmune disease with a variety of clinical presentations. We describe a case of pernicious anemia presenting with pancytopenia with hemolytic features. Further workup revealed very low vitamin B12 levels and elevated methylmalonic acid. It is important for a general internist to identify pernicious anemia as one of the cause of pancytopenia and hemolytic anemia to avoid extensive workup. Pernicious anemia can present strictly with hematological abnormalities without neurological problems or vice versa as in our case.

  13. Severe Anemia with Hemoperitoneum as a First Presentation for Multinodular Hepatocellular Carcinoma: A Rare Event in Western Countries

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    Thein Swe

    2016-01-01

    Full Text Available Hemoperitoneum due to spontaneous rupture of hepatocellular carcinoma is a life-threatening and rare condition in western countries with an incidence of less than 3% because of early detection of cirrhosis and neoplasm. Here, we describe a case of a 66-year-old male patient with altered mental status with hemorrhagic shock. Computed tomography scan of abdomen revealed hemoperitoneum and mass in liver. Patient underwent resection of liver tumor and biopsy revealed multinodular hepatocellular carcinoma. A high degree of suspicion is required where severe anemia and hemoperitoneum can be a first presentation for hepatocellular carcinoma especially in patients with chronic hepatitis C infection. Early diagnosis is crucial since mortality rates remain high for untreated cases.

  14. Secondary benefit of maintaining normal transcranial Doppler velocities when using hydroxyurea for prevention of severe sickle cell anemia.

    Science.gov (United States)

    Ghafuri, Djamila Labib; Chaturvedi, Shruti; Rodeghier, Mark; Stimpson, Sarah-Jo; McClain, Brandi; Byrd, Jeannie; DeBaun, Michael R

    2017-07-01

    In a retrospective cohort study, we tested the hypothesis that when prescribing hydroxyurea (HU) to children with sickle cell anemia (SCA) to prevent vaso-occlusive events, there will be a secondary benefit of maintaining low transcranial Doppler (TCD) velocity, measured by imaging technique (TCDi). HU was prescribed for 90.9% (110 of 120) of children with SCA ≥5 years of age and followed for a median of 4.4 years, with 70% (n = 77) receiving at least one TCDi evaluation after starting HU. No child prescribed HU had a conditional or abnormal TCDi measurement. HU initiation for disease severity prevention decreases the prevalence of abnormal TCDi velocities. © 2016 Wiley Periodicals, Inc.

  15. Safety of Sofosbuvir and Ribavirin Combination Therapy in a Patient Who Developed Anemia due to Ribavirin

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    Hirokazu Suii

    2017-01-01

    Full Text Available Interferon (IFN and ribavirin (RBV combination therapy was previously the standard of care for treatment of hepatitis C virus (HCV genotype 2 infection. But, it often induced hemolytic anemia. In 2014, sofosbuvir (SOF was approved for the treatment of chronic HCV genotype 2 in Japan. SOF/RBV therapy is more effective against genotype 2 than IFN/RBV therapy. We report a case of a 74-year-old woman with chronic HCV genotype 2b infection. She received five treatments including RBV and IFN therapy before SOF was approved and all of them were ineffective. Therapies that included RBV induced severe anemia and led to discontinuation of treatment. With pegylated IFN/RBV therapy, the maximum change in hemoglobin (Hb from baseline was −3.7 g/dL. However, SOF/RBV therapy was effective and she achieved sustained virologic response (SVR with a maximum change in Hb from baseline of only −1.2 g/dL. We also found reticulocyte count was very low during treatment in this case and speculate it was one of the reasons that she developed hemolytic anemia with RBV. In conclusion, SOF/RBV therapy is effective and allowed the patient to achieve SVR. An SOF/RBV regimen is safe and effective for patients who have or are at risk of anemia induced by RBV.

  16. Prevalence and severity of anemia and iron deficiency: cross-sectional studies in adolescent schoolgirls in western Kenya

    NARCIS (Netherlands)

    Leenstra, T.; Kariuki, S. K.; Kurtis, J. D.; Oloo, A. J.; Kager, P. A.; ter Kuile, F. O.

    2004-01-01

    Objective: Anemia is a major public health concern in preschool children and pregnant women in the developing world. While many studies have examined these two at-risk groups, there is a paucity of data on anemia in adolescents living in developing countries in the complex ecologic context of

  17. Real-time PCR Demonstrates Ancylostoma duodenale Is a Key Factor in the Etiology of Severe Anemia and Iron Deficiency in Malawian Pre-school Children

    NARCIS (Netherlands)

    Jonker, Femkje A. M.; Calis, Job C. J.; Phiri, Kamija; Brienen, Eric A. T.; Khoffi, Harriet; Brabin, Bernard J.; Verweij, Jaco J.; van Hensbroek, Michael Boele; van Lieshout, Lisette

    2012-01-01

    Background: Hookworm infections are an important cause of (severe) anemia and iron deficiency in children in the tropics. Type of hookworm species (Ancylostoma duodenale or Necator americanus) and infection load are considered associated with disease burden, although these parameters are rarely

  18. Severe Hemorrhage from the Umbilical Cord at Birth: A Preventable Cause of Neonatal Shock

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    Neetu Singh

    2013-01-01

    Full Text Available Posthemorrhagic anemia is a rare but important cause of anemia in neonates, second only to hemolytic anemia of newborn. Most cases of posthemorrhagic anemia are reported from fetomaternal hemorrhage or umbilical cord accidents in utero. This case report describes a preterm infant who developed severe anemia and shock immediately after delivery related to an acute hemorrhage through patent umbilical cord vessels secondary to a tear in the umbilical cord at the site of cord clamping. We believe that umbilical cord bleeding from errors in cord clamping could be an important cause of acute blood loss in the delivery room and that it may result in significant clinical morbidity, especially in extremely premature infants.

  19. Kell hemolytic disease of the fetus. Combination treatment with plasmapheresis and intrauterine blood transfusion.

    Science.gov (United States)

    Lakhwani, S; Machado, P; Pecos, P; Coloma, M; Rebollo, S; Raya, J M

    2011-08-01

    We report the case of a 36-year old pregnant woman with a Kell alloimmunization (anti-K1), probably secondary to a previous blood transfusion, and a severe hemolytic disease of the fetus. Once the first fetal blood transfusion by cordocentesis was performed, we started treatment with repeated plasmapheresis to maintain anti-K1 titer below 1:32. With this scheme we did not need to perform a second intrauterine fetal blood transfusion and only mild anemia was found in the newborn. Taking into account that the rate of serious complications with plasmapheresis is lower than that related with intrauterine blood transfusion, this could be an alternative approach to repeated transfusions. Copyright © 2011 Elsevier Ltd. All rights reserved.

  20. Intrauterine transfusion and non-invasive treatment options for hemolytic disease of the fetus and newborn - review on current management and outcome.

    Science.gov (United States)

    Zwiers, Carolien; van Kamp, Inge; Oepkes, Dick; Lopriore, Enrico

    2017-04-01

    Hemolytic disease of the fetus and newborn (HDFN) remains a serious pregnancy complication which can lead to severe fetal anemia, hydrops and perinatal death. Areas covered: This review focusses on the current prenatal management, treatment with intrauterine transfusion (IUT) and promising non-invasive treatment options for HDFN. Expert commentary: IUTs are the cornerstone in prenatal management of HDFN and have significantly improved perinatal outcome in the past decades. IUT is now a relatively safe procedure, however the risk of complications is still high when performed early in the second trimester. Non-invasive management using intravenous immunoglobulin may be a safe alternative and requires further investigation.

  1. Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia Crise aplástica devido à infecção por parvovirus humano B19 em anemia hemolítica hereditária

    Directory of Open Access Journals (Sweden)

    R. C. N. Cubel

    1992-10-01

    Full Text Available Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.IgM específica anti-B19 foi demonstrada nos soros de três crianças apresentando aplasia transitória de medula. Um menino de dois anos de idade vivendo no Rio de Janeiro e sendo portador de anemia falciforme, apresentou a crise durante Agosto de 1990. Dois irmãos vivendo em Santa Maria - RS, desenvolveram crise de aplasia em Maio de 1991, quando foram também diagnosticados como portadores de microesferocitose. IgM anti-B19 não foi detectada no soro de uma terceira criança, desta mesma família, a qual primeiramente apresentou crise de aplasia.

  2. Predictors of early treatment discontinuation and severe anemia in a Brazilian cohort of hepatitis C patients treated with first-generation protease inhibitors

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    N. Miotto

    2016-01-01

    Full Text Available The aim of this study was to determine risk factors for adverse events (AE-related treatment discontinuation and severe anemia among patients with chronic hepatitis C virus (HCV genotype 1 infection, treated with first-generation protease inhibitor (PI-based therapy. We included all patients who initiated treatment with PI-based therapy at a Brazilian university hospital between November 2013 and December 2014. We prospectively collected data from medical records using standardized questionnaires and used Epi Info 6.0 for analysis. Severe anemia was defined as hemoglobin ≤8.5 mg/dL. We included 203 patients: 132 treated with telaprevir (TVR and 71 treated with boceprevir (BOC. AE-related treatment discontinuation rate was 19.2% and anemia was the main reason (38.5%. Risk factors for treatment discontinuation were higher comorbidity index (OR=1.85, CI=1.05-3.25 for BOC, and higher bilirubin count (OR=1.02, CI=1.01-1.04 and lower BMI (OR=0.98, CI=0.96-0.99 for TVR. Severe anemia occurred in 35 (17.2% patients. Risk factors for this outcome were lower estimated glomerular filtration rate (eGFR; OR=0.95, CI=0.91-0.98 for patients treated with TVR, and higher comorbidity index (OR=2.21, CI=1.04-4.67 and ribavirin dosage (OR=0.84, CI=0.72-0.99 for those treated with BOC. Fifty-five (57.3% patients treated with TVR and 15 (27.3% patients treated with BOC achieved sustained virological response (SVR. Among patients who received TVR and interrupted treatment due to AE (n=19, only 26.3% (n=5 achieved SVR (P=0.003. Higher number of comorbidities, lower eGFR and advanced liver disease are associated with severe anemia and early treatment cessation, which may compromise SVR achievement.

  3. Neurocognitive Deficits in Children With Sickle Cell Disease Are Associated With the Severity of Anemia

    NARCIS (Netherlands)

    Hijmans, Channa T.; Grootenhuis, Martha A.; Oosterlaan, Jaap; Heijboer, Harriët; Peters, Marjolein; Fijnvandraat, Karin

    2011-01-01

    Background. Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological

  4. Interesting case of G6PD deficiency anemia with severe hemolysis

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    Anupam Chhabra

    2013-01-01

    Full Text Available Severe hemolysis was observed in a critically ill patient with G6Pd deficiency where the causative trigger could not be identified. We describe one young patient with severe hemolysis treated with two cycles of plasmapheresis which proved to be an effective tool in the treatment. The patient presented with diffuse pain abdomen, vomiting, yellowish discoloration of sclera and skin and acute breathlessness. Hemoglobin 5.4 mg/dl and total (T serum bilirubin 17.08 mg/dl: Direct (D 4.10 mg/dl and Indirect (I 12.98 mg/dl. Subsequently patient started passing black color urine. As the patient developed severe hemolysis and the trigger agent of hemolysis was unknown, two cycles of plasmapheresis were performed with the aim to remove unknown causative agent. Consequently no trace of hemolysis was found and patient stabilized. Plasmapheresis can be used to treat G6PD deficient patients with severe hemolysis due to unidentified trigger agent.

  5. Study of alpha hemoglobin stabilizing protein expression in patients with β thalassemia and sickle cell anemia and its impact on clinical severity.

    Science.gov (United States)

    Mahmoud, Hanan Mohamed; Shoeib, Ahmed Al-Saiid Hamed; Abd El Ghany, Shereen Mohamed; Reda, Marwa Mohamed; Ragab, Iman Ahmed

    2015-12-01

    The α hemoglobin stabilizing protein (AHSP) binds α-Hb and prevents its precipitation limiting free α-Hb toxicities. Our aim was to study AHSP expression in β thalassemia syndromes in relation to their clinical severity and to compare it with its level in sickle cell anemia. We compared patients with β-thalassemia (n=37) (β-thalassemia major (BTM) (n=19) and β-thalassemia intermedia (BTI) (n=18)) with 12 patients with sickle cell anemia as regards clinical severity, age at presentation, transfusion dependency, mean pre-transfusion hemoglobin level, use of hydroxyurea and AHSP expression by real time quantitative PCR. Median (and IQR) AHSP expression was significantly higher in patients with sickle cell anemia 2275 (3898) compared to thalassemia 283 (718), P=0.001, with no significant difference between BTM and BTI (P=0.346). It was also significantly higher in non-transfusion dependent patients with β thalassemia (NTDT) compared to transfusion dependent ones (P=0.019), and in patients on hydroxyurea therapy (Psickle cell anemia versus thalassemia, with no significant difference between BTM and BTI. Expression was higher in patients with NTDT and on hydroxyurea therapy. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Anti-Mur as the most likely cause of mild hemolytic disease of the newborn.

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    Bakhtary, Sara; Gikas, Anastasia; Glader, Bertil; Andrews, Jennifer

    2016-05-01

    Although rare in the United States, anti-Mur is relatively common in Southeast Asia and has been reported to have clinical significance in Chinese and Taiwanese populations. The infant was full term and the second child of a Chinese mother and Vietnamese father, presenting with jaundice. He was clinically diagnosed with immune-mediated hemolytic anemia. The direct antiglobulin test indicated that the infant's red blood cells were coated only with anti-IgG. Anti-Mur was identified in the maternal serum and the neonate's plasma. The father was found to be positive for the Mur antigen. The cause of the infant's hemolytic anemia was determined to be most likely anti-Mur. Since anti-Mur is implicated in causing hemolytic disease of the newborn, it is important to recognize this antibody more commonly found in Asian patients in the United States as the Mur+ phenotype has a higher prevalence in this population. © 2016 AABB.

  7. Real-time PCR Demonstrates Ancylostoma duodenale Is a Key Factor in the Etiology of Severe Anemia and Iron Deficiency in Malawian Pre-school Children

    Science.gov (United States)

    Jonker, Femkje A. M.; Calis, Job C. J.; Phiri, Kamija; Brienen, Eric A. T.; Khoffi, Harriet; Brabin, Bernard J.; Verweij, Jaco J.; van Hensbroek, Michael Boele; van Lieshout, Lisette

    2012-01-01

    Background Hookworm infections are an important cause of (severe) anemia and iron deficiency in children in the tropics. Type of hookworm species (Ancylostoma duodenale or Necator americanus) and infection load are considered associated with disease burden, although these parameters are rarely assessed due to limitations of currently used diagnostic methods. Using multiplex real-time PCR, we evaluated hookworm species-specific prevalence, infection load and their contribution towards severe anemia and iron deficiency in pre-school children in Malawi. Methodology and Findings A. duodenale and N. americanus DNA loads were determined in 830 fecal samples of pre-school children participating in a case control study investigating severe anemia. Using multiplex real-time PCR, hookworm infections were found in 34.1% of the severely anemic cases and in 27.0% of the non-severely anemic controls (panemia (adjusted odds ratio: 2.49 (95%CI 1.16–5.33) and 9.04 (95%CI 2.52–32.47) respectively). Iron deficiency (assessed through bone marrow examination) was positively associated with intensity of A. duodenale infection (adjusted odds ratio: 3.63 (95%CI 1.18–11.20); 16.98 (95%CI 3.88–74.35) and 44.91 (95%CI 5.23–385.77) for low, moderate and high load respectively). Conclusions/Significance This is the first report assessing the association of hookworm load and species differentiation with severe anemia and bone marrow iron deficiency. By revealing a much higher than expected prevalence of A. duodenale and its significant and load-dependent association with severe anemia and iron deficiency in pre-school children in Malawi, we demonstrated the need for quantitative and species-specific screening of hookworm infections. Multiplex real-time PCR is a powerful diagnostic tool for public health research to combat (severe) anemia and iron deficiency in children living in resource poor settings. PMID:22514750

  8. Acute Systolic Heart Failure Associated with Complement-Mediated Hemolytic Uremic Syndrome

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    John L. Vaughn

    2015-01-01

    Full Text Available Complement-mediated hemolytic uremic syndrome (otherwise known as atypical HUS is a rare disorder of uncontrolled complement activation that may be associated with heart failure. We report the case of a 49-year-old female with no history of heart disease who presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given her normal ADAMSTS13 activity, evidence of increased complement activation, and renal biopsy showing evidence of thrombotic microangiopathy, she was diagnosed with complement-mediated HUS. She subsequently developed acute hypoxemic respiratory failure secondary to pulmonary edema requiring intubation and mechanical ventilation. A transthoracic echocardiogram showed evidence of a Takotsubo cardiomyopathy with an estimated left ventricular ejection fraction of 20%, though ischemic cardiomyopathy could not be ruled out. Treatment was initiated with eculizumab. After several failed attempts at extubation, she eventually underwent tracheotomy. She also required hemodialysis to improve her uremia and hypervolemia. After seven weeks of hospitalization and five doses of eculizumab, her renal function and respiratory status improved, and she was discharged in stable condition on room air and independent of hemodialysis. Our case illustrates a rare association between acute systolic heart failure and complement-mediated HUS and highlights the potential of eculizumab in stabilizing even the most critically-ill patients with complement-mediated disease.

  9. Prospective study of rabbit antithymocyte globulin and cyclosporine for aplastic anemia from the EBMT Severe Aplastic Anaemia Working Party.

    Science.gov (United States)

    Marsh, Judith C; Bacigalupo, Andrea; Schrezenmeier, Hubert; Tichelli, Andre; Risitano, Antonio M; Passweg, Jakob R; Killick, Sally B; Warren, Alan J; Foukaneli, Theodora; Aljurf, Mahmoud; Al-Zahrani, H A; Höchsmann, Britta; Schafhausen, Philip; Roth, Alexander; Franzke, Anke; Brummendorf, Tim H; Dufour, Carlo; Oneto, Rosi; Sedgwick, Philip; Barrois, Alain; Kordasti, Shahram; Elebute, Modupe O; Mufti, Ghulam J; Socie, Gerard

    2012-06-07

    Rabbit antithymocyte globulin (rATG; thymoglobulin, Genzyme) in combination with cyclosporine, as first-line immunosuppressive therapy, was evaluated prospectively in a multicenter, European, phase 2 pilot study, in 35 patients with aplastic anemia. Results were compared with 105 age- and disease severity-matched patients from the European Blood and Marrow Transplant registry, treated with horse ATG (hATG; lymphoglobulin) and cyclosporine. The primary end point was response at 6 months. At 3 months, no patients had achieved a complete response to rATG. Partial response occurred in 11 (34%). At 6 months, complete response rate was 3% and partial response rate 37%. There were 10 deaths after rATG (28.5%) and 1 after subsequent HSCT. Infections were the main cause of death in 9 of 10 patients. The best response rate was 60% for rATG and 67% for hATG. For rATG, overall survival at 2 years was 68%, compared with 86% for hATG (P = .009). Transplant-free survival was 52% for rATG and 76% for hATG (P = .002). On multivariate analysis, rATG (hazard ratio = 3.9, P = .003) and age more than 37 years (hazard ratio = 4.7, P = .0008) were independent adverse risk factors for survival. This study was registered at www.clinicaltrials.gov as NCT00471848.

  10. Increased apoptosis and peripheral blood mononuclear cell suppression of bone marrow mesenchymal stem cells in severe aplastic anemia.

    Science.gov (United States)

    Chao, Yu-Hua; Lin, Chiao-Wen; Pan, Hui-Hsien; Yang, Shun-Fa; Weng, Te-Fu; Peng, Ching-Tien; Wu, Kang-Hsi

    2018-06-05

    Although immune-mediated pathogenesis is considered an important aspect of severe aplastic anemia (SAA), its underlying mechanisms remain unclear. Mesenchymal stem cells (MSCs) are essential to the formation of specialized microenvironments in the bone marrow (BM), and MSC insufficiency can trigger the development of SAA. To find MSC alterations in the SAA BM, we compared BM MSCs from five children with SAA and five controls. Peripheral blood mononuclear cells (PBMCs) were cocultured with MSCs to evaluate the supportive effects of MSCs on hematopoiesis. Cytometric bead array immunoassay was used to determine cytokine excretion by MSCs. The immune functions of MSCs and their conditioned medium (CM) were evaluated by PBMC proliferation assays. SAA MSCs were characterized by a high percentage of cells in the abnormal sub-G1 phase of the cell cycle, which suggests an increased rate of apoptosis in SAA MSCs. In comparison with control MSCs, PBMCs cocultured with SAA MSCs displayed significantly reduced PBMC proliferation (P = 0.009). Aberrant cytokine profiles were secreted by SAA MSCs, with increased concentrations of interleukin-6, interferon-γ, tumor necrosis factor-α, and interleukin-1β in the CM. PBMC proliferation assays demonstrated additional immunosuppressive effects of SAA MSCs (P = 0.016) and their CM (P = 0.013). Our data revealed increased apoptosis and PBMC suppression of SAA MSCs. The alterations of MSCs may contribute to the formation of functionally abnormal microenvironments in SAA BM. © 2018 Wiley Periodicals, Inc.

  11. Reduced intensity conditioning, combined transplantation of haploidentical hematopoietic stem cells and mesenchymal stem cells in patients with severe aplastic anemia.

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    Xiao-Hong Li

    Full Text Available We examined if transplantation of combined haploidentical hematopoietic stem cells (HSC and mesenchymal stem cells (MSC affected graft failure and graft-versus-host disease (GVHD in patients with severe aplastic anemia (SAA. Patients with SAA-I (N = 17 received haploidentical HSCT plus MSC infusion. Stem cell grafts used a combination of granulocyte colony-stimulating factor (G-CSF-primed bone marrow and G-CSF-mobilized peripheral blood stem cells of haploidentical donors and the culture-expanded third-party donor-derived umbilical cord MSCs (UC-MSCs, respectively. Reduced intensity conditioning consisted of fludarabine (30 mg/m2·d+cyclosphamide (500 mg/m2·d+anti-human thymocyte IgG. Transplant recipients also received cyclosporin A, mycophenolatemofetil, and CD25 monoclonal antibody. A total of 16 patients achieved hematopoietic reconstitution. The median mononuclear cell and CD34 count was 9.3×10(8/kg and 4.5×10(6/kg. Median time to ANC was >0.5×10(9/L and PLT count >20×10(9/L were 12 and 14 days, respectively. Grade III-IV acute GVHD was seen in 23.5% of the cases, while moderate and severe chronic GVHD were seen in 14.2% of the cases. The 3-month and 6-month survival rates for all patients were 88.2% and 76.5%, respectively; mean survival time was 56.5 months. Combined transplantation of haploidentical HSCs and MSCs on SAA without an HLA-identical sibling donor was safe, effectively reduced the incidence of severe GVHD, and improved patient survival.

  12. APLASTIC ANEMIA

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    Ni Made Dharma Laksmi

    2013-07-01

    Full Text Available Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 Aplastic Anemia describes a disorder of the clinical syndrome is marked by a deficiency of red blood cells, neutrophils, monocytes and platelets in the absence of other forms of bone marrow damage. Aplastic anemia is classified as a rare disease in developed countries the incidence of 3-6 cases / 1 million inhabitants / year. The exact cause of someone suffering from aplastic anemia also can not be established with certainty, but there are several sources of potential risk factors. Prognosis or course of the disease varies widely aplastic anemia, but without treatment generally gives a poor prognosis /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:"Times New Roman"; mso-bidi-theme-font:minor-bidi;}

  13. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study.

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    Deepika S Darbari

    Full Text Available BACKGROUND: Frequent painful vaso-occlusive crises (VOCs were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD over twenty years ago. Modern therapies for sickle cell anemia (SCA like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort. METHODS: Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation. RESULTS: Higher baseline hematocrit (p = 0.0008, ferritin (p = 0.005, and HDL cholesterol (p = 0.01 were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03. Higher tricuspid regurgitatant jet velocity (TRV (RR 2.41, 95% CI 1.5-3.9, p < 0.0001, elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001 and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001 were also independent risk factors for mortality. CONCLUSIONS: Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies. TRIAL REGISTRATION: ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/

  14. Predictors of early mortality after rabbit antithymocyte globulin as first-line treatment in severe aplastic anemia.

    Science.gov (United States)

    Atta, Elias H; Lima, Carlos B L; Dias, Danielle S P; Clé, Diego V; Bonduel, Mariana M; Sciuccati, Gabriela B; Medeiros, Larissa A; Oliveira, Michel M; Salvino, Marco A; Garanito, Marlene P; Blum Fonseca, Patricia B; Saad, Sara Teresinha O; Calado, Rodrigo T; Scheinberg, Phillip

    2017-11-01

    Despite being recommended as first-line immunosuppressive therapy in severe aplastic anemia (SAA), horse antithymocyte globulin (ATG) is still unavailable in many countries outside the USA. Rabbit ATG is more lymphocytoxic than horse ATG, and this might result in a higher incidence of severe infections and early mortality. This study was designed to identify the risk factors for early mortality and overall survival (OS) after rabbit ATG in patients with SAA. We retrospectively reviewed 185 patients with SAA who underwent rabbit ATG and cyclosporine. The incidence of death in 3 months following rabbit ATG therapy was 15.1% (28/185). Early mortality was mainly related to infectious complications, despite adequate antibiotic and/or antifungal treatment. Age > 35 years (odds ratio [OR] 5.06, P = 0.001) and baseline absolute neutrophil count (ANC) ≤ 0.1 × 10 9 /L (OR 7.64, P  35 years (OR 1.88, P = 0.03), baseline ANC ≤ 0.1 × 10 9 /L (OR 2.65, P < 0.001), and lack of response to rabbit ATG (OR 11.40, P < 0.001) were independently associated with mortality. Alternative strategies are needed for the treatment of SAA patients in countries were horse ATG is unavailable, particularly for those at high risk for early mortality after rabbit ATG due to a higher age and very low pre-treatment neutrophil count.

  15. Severe Painful Vaso-Occlusive Crises and Mortality in a Contemporary Adult Sickle Cell Anemia Cohort Study

    Science.gov (United States)

    Darbari, Deepika S.; Wang, Zhengyuan; Kwak, Minjung; Hildesheim, Mariana; Nichols, James; Allen, Darlene; Seamon, Catherine; Peters-Lawrence, Marlene; Conrey, Anna; Hall, Mary K.; Kato, Gregory J.; Taylor VI, James G.

    2013-01-01

    Background Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort. Methods Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation. Results Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality. Conclusions Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies. Trial Registration ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/ PMID:24224021

  16. Ribavirin-induced anemia in hepatitis C virus patients undergoing combination therapy.

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    Sheeja M Krishnan

    2011-02-01

    Full Text Available The current standard of care for hepatitis C virus (HCV infection - combination therapy with pegylated interferon and ribavirin - elicits sustained responses in only ∼50% of the patients treated. No alternatives exist for patients who do not respond to combination therapy. Addition of ribavirin substantially improves response rates to interferon and lowers relapse rates following the cessation of therapy, suggesting that increasing ribavirin exposure may further improve treatment response. A key limitation, however, is the toxic side-effect of ribavirin, hemolytic anemia, which often necessitates a reduction of ribavirin dosage and compromises treatment response. Maximizing treatment response thus requires striking a balance between the antiviral and hemolytic activities of ribavirin. Current models of viral kinetics describe the enhancement of treatment response due to ribavirin. Ribavirin-induced anemia, however, remains poorly understood and precludes rational optimization of combination therapy. Here, we develop a new mathematical model of the population dynamics of erythrocytes that quantitatively describes ribavirin-induced anemia in HCV patients. Based on the assumption that ribavirin accumulation decreases erythrocyte lifespan in a dose-dependent manner, model predictions capture several independent experimental observations of the accumulation of ribavirin in erythrocytes and the resulting decline of hemoglobin in HCV patients undergoing combination therapy, estimate the reduced erythrocyte lifespan during therapy, and describe inter-patient variations in the severity of ribavirin-induced anemia. Further, model predictions estimate the threshold ribavirin exposure beyond which anemia becomes intolerable and suggest guidelines for the usage of growth hormones, such as erythropoietin, that stimulate erythrocyte production and avert the reduction of ribavirin dosage, thereby improving treatment response. Our model thus facilitates, in

  17. A retrospective comparison of cyclophosphamide plus antithymocyte globulin with cyclophosphamide plus busulfan as the conditioning regimen for severe aplastic anemia

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    L.V.M. Ommati

    2009-03-01

    Full Text Available Allogeneic hematopoietic stem cell transplantation (AHSCT is the treatment of choice for young patients with severe aplastic anemia (SAA. The association of antithymocyte globulin (ATG and cyclophosphamide (CY is the most frequently used conditioning regimen for this disease. We performed this retrospective study in order to compare the outcomes of HLA-matched sibling donor AHSCT in 41 patients with SAA receiving cyclophosphamide plus ATG (ATG-CY, N = 17 or cyclophosphamide plus busulfan (BU-CY, N = 24. The substitution of BU for ATG was motivated by the high cost of ATG. There were no differences in the clinical features between the two groups, including age, gender, cytomegalovirus status, ABO match, interval between diagnosis and transplant, and number of total nucleated cells infused. No differences were observed in the time to neutrophil and platelet engraftment, or in the risk of veno-occlusive disease and hemorrhage. However, there was a higher risk of mucositis in the BU-CY group (71 vs 24%, P = 0.004. There were no differences in the incidence of neutrophil and platelet engraftment, acute and chronic graft-versus-host disease, and transplant-related mortality. There was a higher incidence of late rejection in the ATG-CY group (41 vs 4%, P = 0.009. Although the ATG-CY group had a longer follow-up (101 months than the BU-CY group (67 months, P = 0.04, overall survival was similar between the groups (69 vs 58%, respectively, P = 0.32. We conclude that the association BU-CY is a feasible option to the conventional ATG-CY regimen in this population.

  18. Long-term outcome of 25 children and adolescents with severe aplastic anemia treated with antithymocyte globulin

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    de-Medeiros C.R.

    2000-01-01

    Full Text Available Severe aplastic anemia (SAA is probably an immune-mediated disorder, and immunosuppressive therapy is recommended for patients with no available donor for bone marrow transplant. Between October 1984 and November 1987, 25 consecutive children and adolescents with SAA with no HLA-compatible marrow donor received equine antithymocyte globulin (ATG (15 mg kg-1 day-1 for 10 days. The patients were evaluated 6 weeks, 6 months, and 12 months after starting ATG treatment. Thereafter, patients were evaluated yearly until July 1998. Median age was 10 years (range, 1.5-20 years, granulocyte counts on referral ranged from 0.032 to 1.4 x 10(9/l (median 0.256 x 10(9/l, and 12 patients had granulocyte counts <0.2 x 10(9/l. At a median follow-up of 9.6 years (range, 8.6-11.8 years, 10 patients (40% remained alive with good marrow function. No morphologic evidence of hematological clonal disorders has been observed, although two patients probably have acquired clonal chromosomal abnormalities (trisomy 8 and del(6q21, respectively. Responses to ATG were observed between 6 weeks and 6 months from the start of treatment in 60% of evaluable patients. The response rate was not different in patients whose granulocyte count at diagnosis was <0.2 x 10(9/l, or in those who were <10 years of age. This study supports the view that, when compared with supportive measures, ATG is an effective treatment for children or adolescents with SAA. Although these results are inferior to those reported for marrow transplantation or more intensive immunosuppressive regimens, these patients who responded to ATG are long-term survivors with stable peripheral blood counts and a low rate of relapse.

  19. Outcomes of Optimized over Standard Protocol of Rabbit Antithymocyte Globulin for Severe Aplastic Anemia: A Single-Center Experience

    Science.gov (United States)

    Ge, Meili; Shao, Yingqi; Huang, Jinbo; Huang, Zhendong; Zhang, Jing; Nie, Neng; Zheng, Yizhou

    2013-01-01

    Background Previous reports showed that outcome of rabbit antithymocyte globulin (rATG) was not satisfactory as the first-line therapy for severe aplastic anemia (SAA). We explored a modifying schedule of administration of rATG. Design and Methods Outcomes of a cohort of 175 SAA patients, including 51 patients administered with standard protocol (3.55 mg/kg/d for 5 days) and 124 cases with optimized protocol (1.97 mg/kg/d for 9 days) of rATG plus cyclosporine (CSA), were analyzed retrospectively. Results Of all 175 patients, response rates at 3 and 6 months were 36.6% and 56.0%, respectively. 51 cases received standard protocol had poor responses at 3 (25.5%) and 6 months (41.2%). However, 124 patients received optimized protocol had better responses at 3 (41.1%, P = 0.14) and 6 (62.1%, P = 0.01). Higher incidences of infection (57.1% versus 37.9%, P = 0.02) and early mortality (17.9% versus 0.8%, P<0.001) occurred in patients received standard protocol compared with optimized protocol. The 5-year overall survival in favor of the optimized over standard rATG protocol (76.0% versus. 50.3%, P<0.001) was observed. By multivariate analysis, optimized protocol (RR = 2.21, P = 0.04), response at 3 months (RR = 10.31, P = 0.03) and shorter interval (<23 days) between diagnosis and initial dose of rATG (RR = 5.35, P = 0.002) were independent favorable predictors of overall survival. Conclusions Optimized instead of standard rATG protocol in combination with CSA remained efficacious as a first-line immunosuppressive regimen for SAA. PMID:23554855

  20. Severe atypical herpes zoster as an initial symptom of fatal myelodysplastic syndrome with refractory anemia and blast excess (RAEB II

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    Wollina U

    2017-05-01

    Full Text Available Uwe Wollina,1 Gesina Hansel,1 Anja Baunacke,1 Georgi Tchernev2 1Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany; 2Department of Dermatology and Dermatologic Surgery, Medical Institute of Ministry of Interior (MVR, Sofia, Bulgaria Abstract: Herpes zoster is a common disease caused due to varicella zoster virus (VZV infection with increasing incidence by age. If the patient has a severe, extended, or treatment-recalcitrant course of herpes zoster, this must be a red flag to search for underlying pathologies. Here, we report about a 64-year-old male patient with diabetes, who came to our emergency department because of general malaise, fever, chills, and a pronounced nuchal and facial swelling on the left side. Based on herpetiform-grouped vesicles and yellowish crusts, an impetiginized facial herpes zoster was diagnosed, and combined antiviral and antibiotic treatment was initiated. He was HIV negative. Despite intensified treatment, his situation worsened. We observed blasts in peripheral blood, but bone marrow biopsy was initially denied. Some days later after deterioration of his disease, he accepted further diagnostics. A myelodysplastic syndrome with blast excess (refractory anemia and blast excess II, RAEB II could be confirmed. The following translocations were detected: t(2;12(p13; q13 and t(6;9(p22;q34. REAB II has an unfortunate prognosis. Cytoreductive treatment was initiated by the hemato-oncologist. Unfortunately, the patient deceased due to septic shock. Keywords: herpes zoster, varicella zoster virus, myelodysplastic syndrome, sepsis, emergency

  1. Gastrointestinal Stromal Tumor of the Stomach with Narrow Stalk-Like Based, Uneven Protruding Appearance Presenting with Severe Acute Anemia despite Small Size

    Directory of Open Access Journals (Sweden)

    Tomomitsu Tahara

    2010-03-01

    Full Text Available We report the case of a 56-year-old woman who had a gastrointestinal stromal tumor (GIST of the stomach. She was admitted to our hospital for epigastric pain, nausea, and severe acute anemia (hemoglobin level 4.3 g/dl. Esophagogastroduodenoscopy revealed a narrow stalk-like based, hemorrhagic and uneven protruding lesion in the lesser curvature of the gastric upper corpus. Although the tumor was less than 2 cm in diameter and was probably a benign GIST according to histology, laparoscopy-assisted local resection was needed because the patient had continuous severe anemia and epigastric pain. Histological assessment showed that the elongated spindle-like tumor cells originated from the intrinsic muscle layer, and was shown with growth to the mucosal side, cropping out to the surface in most areas of the protruding lesion. Only a small part of the tumor was within nontumoral gastric mucosa. Most of the tumor cells demonstrated immunoreactivity for KIT and CD34 in the cytoplasm but not for αSMA, S100, and desmin. Mitotic activity (0/50 high power field and the labeling index for MIB-1 (about 1% were low. The GIST of the stomach described in this report was a rare case with a narrow stalk-like based, uneven protruding mass presenting with severe acute anemia despite small size.

  2. Unexpected Anemia and Reticulocytopenia in an Adolescent With Sickle Cell Anemia Receiving Chronic Transfusion Therapy.

    Science.gov (United States)

    Blauel, Emily R; Grossmann, Lily T; Vissa, Madhav; Miller, Scott T

    2015-10-01

    In a patient with sickle cell disease receiving chronic transfusion, exacerbation of anemia with reticulocytopenia must prompt consideration of a delayed hemolytic transfusion reaction with hyperhemolysis, as further transfusion may worsen this condition; definitive diagnosis is sometimes difficult. Anemia evolving during parvovirus B19-induced erythroid hypoplasia (transient aplastic crisis) should be attenuated in chronic transfusion patients due to superior survival of transfused over endogenous red blood cells. A 16-year-old with sickle cell disease receiving chronic transfusion of modified intensity (goal to maintain hemoglobin S<50%) who developed symptomatic anemia with reticulocytopenia was later shown to have had transient aplastic crisis.

  3. Aplastic Anemia

    Science.gov (United States)

    Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... blood cells. There are different types, including Fanconi anemia. Causes include Toxic substances, such as pesticides, arsenic, ...

  4. Fanconi anemia

    Science.gov (United States)

    ... possibly given through a vein) to treat infections Blood transfusions to treat symptoms due to low blood counts ... have regular check-ups to screen for cancer. Alternative Names Fanconi's anemia; Anemia - Fanconi's Images Formed elements of blood References Bagby GC. Aplastic anemia and related bone ...

  5. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... making new blood cells. Visit our Aplastic Anemia Health Topic to learn more. ... recommend that you take iron supplements, also called iron pills or oral iron, by mouth once or several times a ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... A-Z Clinical Trials Publications and Resources Health Education and Awareness ... If your doctor diagnoses you with iron-deficiency anemia, your treatment will depend on the cause and severity of the condition. Your ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... diagnoses you with iron-deficiency anemia, your treatment will depend on the cause and severity of the ... of iron. The recommended daily amounts of iron will depend on your age, sex, and whether you ...

  8. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... symptoms. More severe iron-deficiency anemia may cause fatigue or tiredness, shortness of breath, or chest pain. ... in the hands and feet Difficulty concentrating Dizziness Fatigue, or feeling tired, is the most common symptom. ...

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... family history and genetics , lifestyle habits, or sex. Age You may be at increased risk for iron ... Signs, Symptoms, and Complications Iron-deficiency anemia can range from mild to severe. People with mild or ...

  10. Detection and Significance of CD4+CD25+CD127dim Regulatory T Cells in Individuals with Severe Aplastic Anemia

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    Weiwei Qi

    2015-09-01

    Full Text Available Objective: To investigate the relationship between CD4+CD25+CD127dim regulatory T cells (Tregs and immune imbalance in acquired severe aplastic anemia (SAA. Materials and Methods: The quantity of CD4+CD25+CD127dim Tregs in 44 SAA patients and 23 normal controls was measured by flow cytometry. Correlations between Tregs and T cell subsets, dendritic cell (DC subsets, granulocyte counts, and percentage of reticulocytes (RET% were analyzed. Results: The percentage of CD4+CD25+CD127dim Tregs in peripheral blood lymphocytes (PBLs of untreated patients was lower than in recovery patients and normal controls (0.83±0.44% vs. 2.91±1.24% and 2.18±0.55%, respectively, p<0.05. The percentage of CD4+CD25+CD127dim Tregs in CD4+ T lymphocytes of recovery patients was higher than that of untreated patients and normal controls (9.39±3.51% vs. 7.61±5.3% and 6.83±1.4%, respectively, p<0.05. The percentage of CD4+ T lymphocytes in PBLs of untreated patients was lower than in recovery patients and normal controls (13.55±7.37% vs. 31.82±8.43% and 32.12±5.88%, respectively, p<0.05. T cell subset (CD4+/CD8+ ratio was 0.41±0.24 in untreated patients, which was lower than in recovery patients (1.2±0.4 and normal controls (1.11±0.23 (p<0.05. DC subset (myeloid DC/plasmacytoid DC ratio, DC1/DC2 ratio was 3.08±0.72 in untreated patients, which was higher than in recovery patients (1.61±0.49 and normal controls (1.39±0.36 (p<0.05. The percentage of CD4+CD25+CD127dim Tregs in PBLs was positively associated with T cell subset (r=0.955, p<0.01 and negatively associated with DC subset (r=-0.765, p<0.01. There were significant positive correlations between CD4+CD25+CD127dim Tregs/PBL and granulocyte counts and RET% (r=0.739 and r=0.749, respectively, p<0.01. Conclusion: The decrease of CD4+CD25+CD127dim Tregs in SAA patients may cause excessive functioning of T lymphocytes and thus lead to hematopoiesis failure in SAA.

  11. Hemolytic potential of hydrodynamic cavitation.

    Science.gov (United States)

    Chambers, S D; Bartlett, R H; Ceccio, S L

    2000-08-01

    The purpose of this study was to determine the hemolytic potentials of discrete bubble cavitation and attached cavitation. To generate controlled cavitation events, a venturigeometry hydrodynamic device, called a Cavitation Susceptibility Meter (CSM), was constructed. A comparison between the hemolytic potential of discrete bubble cavitation and attached cavitation was investigated with a single-pass flow apparatus and a recirculating flow apparatus, both utilizing the CSM. An analytical model, based on spherical bubble dynamics, was developed for predicting the hemolysis caused by discrete bubble cavitation. Experimentally, discrete bubble cavitation did not correlate with a measurable increase in plasma-free hemoglobin (PFHb), as predicted by the analytical model. However, attached cavitation did result in significant PFHb generation. The rate of PFHb generation scaled inversely with the Cavitation number at a constant flow rate, suggesting that the size of the attached cavity was the dominant hemolytic factor.

  12. Combined deficiencies of 25-hydroxyvitamin D and anemia in preschool children with severe early childhood caries: A case-control study.

    Science.gov (United States)

    Deane, Shannon; Schroth, Robert J; Sharma, Atul; Rodd, Celia

    2018-05-01

    Severe early childhood caries (S-ECC) is common and has adverse affects on children's health. Children with S-ECC have been shown to have anemia or vitamin D deficiency. No studies have assessed the presence of combined deficiencies with S-ECC. The purpose of our study was to examine whether those with S-ECC had a higher prevalence of combined anemia and low 25-hydroxyvitamin D (25(OH)D) compared to controls. Covariates associated with elevated parathyroid hormone (PTH), previously noted in S-ECC, were examined. This is a re-analyses of a previously described cross-sectional case-control study; data were collected between 2009 and 2011. Children with S-ECC were recruited on the day of dental surgery and controls from the community. Blood was drawn for complete blood count, ferritin, 25(OH)D and PTH. Families completed a questionnaire. A total of 266 children participated (S-ECC n=144); the mean age was 40.8 ± 14.1 months. Children with S-ECC were more likely to have low 25(OH)D, hemoglobin, elevated PTH or iron-deficiency anemia compared to controls. Significant differences between groups were seen for a combined deficiency of low hemoglobin (<110 g/L) and 25(OH)D < 50 nmol/L; controls 0/114 versus S-ECC 15/140 (P<0.001). In an adjusted regression model, PTH was negatively associated with 25(OH)D (P<0.001) and higher income (P<0.02); it was positively associated with less regular milk consumption (P=0.001). Combined deficiencies of vitamin D and anemia are more prevalent in children with S-ECC; the etiology remains unclear. A detailed diet history is key in those with S-ECC to assess risks for deficiencies.

  13. Beta-hemolytic Streptococcal Bacteremia

    DEFF Research Database (Denmark)

    Nielsen, Hans Ulrik; Kolmos, Hans Jørn; Frimodt-Møller, Niels

    2002-01-01

    Bacteremia with beta-hemolytic Streptococci groups A, B, C and G has a mortality rate of approximately 20%. In this study we analyzed the association of various patient risk factors with mortality. Records from 241 patients with beta-hemolytic streptococcal bacteremia were reviewed with particular...... attention to which predisposing factors were predictors of death. A logistic regression model found age, burns, immunosuppressive treatment and iatrogenic procedures prior to the infection to be significant predictors of death, with odds ratios of 1.7 (per decade), 19.7, 3.6 and 6.8, respectively...

  14. Assesment, treatment and prevention of atypical hemolytic uremic syndrome

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    Azar Nickavar

    2013-01-01

    Full Text Available 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.

  15. Intravenous immunoglobulin in ABO and Rh hemolytic diseases of newborn.

    Science.gov (United States)

    Nasseri, Fatemeh; Mamouri, Gholam A; Babaei, Homa

    2006-12-01

    To evaluate whether the use of intravenous immunoglobulin in newborn infants with isoimmune hemolytic jaundice due to Rh and ABO incompatibility is an effective treatment in reducing the need for exchange transfusion. This study included all direct Coombs' test positive Rh and ABO isoimmunized babies, who admitted in the Neonatal Intensive Care Unit of Ghaem Hospital of Mashhad University of Medical Sciences, Iran, from October 2003 to October 2004. Significant hyperbilirubinemia was defined as rising by >or=0.5 mg/dl per hour. Babies were randomly assigned to received phototherapy with intravenous immunoglobulin (IVIg) 0.5 g/kg over 4 hours, every 12 hours for 3 doses (study group) or phototherapy alone (control group). Exchange transfusion was performed in any group if serum bilirubin exceeded >or=20mg/dl or rose by >or=1mg/dl/h. A total of 34 babies were eligible for this study (17 babies in each group). The number of exchange transfusion, duration of phototherapy and hospitalization days, were significant shorter in the study group versus control group. When we analyzed the outcome results in ABO and Rh hemolytic disease separately, the efficacy of IVIg was significantly better in Rh versus ABO isoimmunization. Late anemia was more common in the IVIg group 11.8% versus 0%, p=0.48. Adverse effects were not observed during IVIg administration. Administration of IVIg to newborns with significant hyperbilirubinemia due to Rh hemolytic disease reduced the need for exchange transfusion but in ABO hemolytic disease there was no significant difference between IVIg and double surface blue light phototherapy.

  16. Dietary hemoglobin rescues young piglets from severe iron deficiency anemia: Duodenal expression profile of genes involved in heme iron absorption.

    Directory of Open Access Journals (Sweden)

    Robert Staroń

    Full Text Available Heme is an efficient source of iron in the diet, and heme preparations are used to prevent and cure iron deficiency anemia in humans and animals. However, the molecular mechanisms responsible for heme absorption remain only partially characterized. Here, we employed young iron-deficient piglets as a convenient animal model to determine the efficacy of oral heme iron supplementation and investigate the pathways of heme iron absorption. The use of bovine hemoglobin as a dietary source of heme iron was found to efficiently counteract the development of iron deficiency anemia in piglets, although it did not fully rebalance their iron status. Our results revealed a concerted increase in the expression of genes responsible for apical and basolateral heme transport in the duodenum of piglets fed a heme-enriched diet. In these animals the catalytic activity of heme oxygenase 1 contributed to the release of elemental iron from the protoporphyrin ring of heme within enterocytes, which may then be transported by the strongly expressed ferroportin across the basolateral membrane to the circulation. We hypothesize that the well-recognized high bioavailability of heme iron may depend on a split pathway mediating the transport of heme-derived elemental iron and intact heme from the interior of duodenal enterocytes to the bloodstream.

  17. Pregnancy Complications: Anemia

    Science.gov (United States)

    ... online community Home > Complications & Loss > Pregnancy complications > Anemia Anemia E-mail to a friend Please fill in ... anemia at a prenatal care visit . What causes anemia? Usually, a woman becomes anemic (has anemia) because ...

  18. Anemia (For Parents)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Anemia KidsHealth / For Parents / Anemia What's in this article? ... Deficiency Anemia in My Kids? Print What Is Anemia? Anemia is when the level of healthy red ...

  19. Hemolytic disease of the fetus and newborn caused by anti-Lan.

    Science.gov (United States)

    Brooks, Sarah; Squires, Jerry E

    2014-05-01

    Antibodies to the high-incidence red blood cell (RBC) antigen Lan (Langereis) are typically immunoglobulin G and have been shown to fix complement and cause hemolysis of Lan antigen-positive RBCs. Only three cases of hemolytic disease of the fetus and newborn (HDFN) have been reported involving anti-Lan and all have been characterized as "mild." A 26-year-old Hispanic female presented in her fifth pregnancy for routine obstetric care. Due to progressively rising anti-Lan titers, middle cerebral artery (MCA) Dopplers were performed. At 32 weeks of gestation, the antibody titer had reached 128; the MCA Doppler indicated that fetal anemia was severe. An intrauterine transfusion with Lan antigen-negative RBCs was performed and a viable infant was delivered 25 days later. Three cases of HDFN associated with anti-Lan have been previously reported. While these cases have been associated with somewhat variable serologic findings, none have resulted in fetal demise or severe symptomatology requiring pre- or postnatal intervention other than routine phototherapy. The current report, however, suggests that in some instances anti-Lan can result in a more severe form of HDFN requiring more aggressive prenatal therapy. In spite of previous case reports suggesting that anti-Lan is associated with relatively mild HDFN, this case suggests that in some instances, this antibody can cause severe HDFN requiring prenatal intervention. © 2013 American Association of Blood Banks.

  20. Evaluation of Neonatal Hemolytic Jaundice: Clinical and Laboratory Parameters

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    Anet Papazovska Cherepnalkovski

    2015-12-01

    CONCLUSIONS: The laboratory profile in ABO/Rh isoimmunisation cases depicts hemolytic mechanism of jaundice. These cases carry a significant risk for early and severe hyperbilirubinemia and are eligible for neurodevelopmental follow-up. Hematological parameters and blood grouping are simple diagnostic methods that assist the etiological diagnosis of neonatal hyperbilirubinemia.

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

    Science.gov (United States)

    Starcević, Mirta; Mataija, Marina; Sović, Dragica; Dodig, Javorka; Matijević, Ratko; Kukuruzović, Monika

    2011-03-01

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

  2. ABO incompatibility hemolytic disease following exchange transfusion 96 newborn

    Directory of Open Access Journals (Sweden)

    Khatami S.F

    2007-09-01

    Full Text Available Background: ABO incompatibility hemolytic disease of the newborn is a common cause of clinical jaundice and causes two-thirds of the hemolytic disease in newborns. This study was undertaken to determine the frequency of ABO incompatibility hemolytic disease and its complications in newborns undergoing exchange transfusion.Methods: This prospective and descriptive study was performed in jaundiced newborn infants during a three-year period. Inclusion criteria were: maternal blood type O, newborn blood type A or B, rising indirect hyperbilirubinemia in the first two days of life, positive immunohematologic test for newborns and exchange transfusion. Exclusion criteria were: incomplete information, other accompanying diseases that induce hyperbilirubinemia. All newborn infants received phototherapy before and after exchange transfusion. We did not use intravenous immunoglobulin, hemoxygenase inhibitor drugs and blood products before exchange transfusion.Results: Double-volume exchange transfusion via umbilical cord catheter was performed in 96 patients, 19 (20% of whom suffered from ABO incompatibility. Of these 19 newborns, two-thirds (13 were preterm infants. The minimum level of serum bilirubin was 10 mg/dl and the maximum serum bilirubin level was 35 mg/dl. In six patients (32% serum bilirubin levels were >25mg/dl. The most common blood group was type A for newborns. Immunohematologic tests were positive in 84% of the mothers. ABO incompatibility hemolytic disease was the fourth and second most common reasons for blood exchange transfusion in preterm and term infants, respectively. Laboratory complications were more common than clinical complications. The etiology of 48% of the alloimmunization and 42% of the hemolytic disease in these newborns was ABO incompatibility.Conclusions: Mothers with blood group O and newborns with blood group A or B with positive immunohematologic tests in first hours of life are at high risk for hemolytic disease

  3. Intravenous immunoglobulin G (IVIG) therapy for significant hyperbilirubinemia in ABO hemolytic disease of the newborn.

    Science.gov (United States)

    Miqdad, A M; Abdelbasit, O B; Shaheed, M M; Seidahmed, M Z; Abomelha, A M; Arcala, O P

    2004-09-01

    Although intravenous immunoglobulin G (IVIG) therapy has been reported in hyperbilirubinemia of Rh hemolytic disease, its use in ABO hemolytic disease has been reported in only a few studies. In our institute we have observed that almost 30% of babies with hyperbilirubinemia due to ABO hemolytic disease required exchange transfusion. To determine whether administration of IVIG to newborns with significant hyperbilirubinemia due to ABO hemolytic disease would reduce the need for exchange transfusion as a primary goal in these babies. This was a prospective study involving all newborns with significant hyperbilirubinemia due to direct Coombs-positive ABO hemolytic disease. All healthy term babies with ABO hemolytic disease with positive direct Coombs test in the period between 2000 and 2002 were identified. Significant hyperbilirubinemia was defined as hyperbilirubinemia requiring phototherapy and/or rising by 8.5 micromol/l per h (0.5 mg/dl per h) or more to require exchange transfusion. Babies were randomly assigned into two groups: group 1 (study group) received phototherapy plus IVIG (500 mg/kg); and group 2 (control group) received phototherapy alone. Exchange transfusion was carried out in any group if at any time the bilirubin level reached 340 micromol/l (20 mg/dl) or more, or rose by 8.5 micromol/l per h (0.5 mg/dl per h) in group 2. A total of 112 babies were enrolled over 2 years, 56 in each group. Exchange transfusion was carried out in four babies in the study group, while 16 babies in the control group required exchange. Late anemia was not of concern in either group. No adverse effects related to IVIG administration were recorded. Administration of IVIG to newborns with significant hyperbilirubinemia due to ABO hemolytic disease with positive direct Coomb's test reduces the need for exchange transfusion without producing immediate adverse effects.

  4. Specific features of a neonatal period in infants following intrauterine intravascular blood transfusion for fetal hemolytic disease

    Directory of Open Access Journals (Sweden)

    A. V. Ivanova

    2015-01-01

    Full Text Available The paper gives data on the characteristics of a neonatal period in infants following intrauterine blood transfusion for Rh-induced fetal hemolytic disease. It is shown that the early diagnosis and detection of the signs of fetal hemolytic disease, and intrauterine intravascular blood transfusion may prolong pregnancy, ensure the birth of a baby with normal anthropometric indicators, optimize his/her neonatal period and prognosis of severe hemolytic disease in the fetus and newborn.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1980-08-01

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

  6. T-cell receptor Vbeta CDR3 oligoclonality frequently occurs in childhood refractory cytopenia (MDS-RC) and severe aplastic anemia

    DEFF Research Database (Denmark)

    Vries, A.C. de; Langerak, A.W.; Verhaaf, B.

    2008-01-01

    (Very) severe acquired aplastic anemia ((v)SAA) and myelodysplastic syndrome (MDS) are rare diseases in childhood. (V)SAA is a bone marrow (BM) failure syndrome characterized by immune-mediated destruction of hematopoietic progenitors. MDS is a malignant clonal stem cell disorder, of which...... the hypoplastic variant is, in case of absence of a cytogenetic clone, difficult to separate from (v)SAA. Recently, studies provided a molecular signature of autoimmunity in adult (v)SAA, by showing oligoclonality based on the length of the TCR Vbeta CDR3 region. We investigated retrospectively the frequency...... and the discriminative value of TCR Vbeta CDR3 oligoclonality in pediatric (v)SAA and MDS patients. Peripheral blood (PB) and/or BM mononuclear cell samples of pediatric patients with (v)SAA (n=38), refractory cytopenia (MDS-RC) (n=28) and 18 controls were analysed via TCR Vbeta heteroduplex PCR analysis of extracted...

  7. An attempt to induce transient immunosuppression pre-erythrocytapheresis in a girl with sickle cell disease, a history of severe delayed hemolytic transfusion reactions and need for hip prosthesis

    Directory of Open Access Journals (Sweden)

    Alessandro Cattoni

    2013-06-01

    Full Text Available We report on a case of delayed hemolytic transfusion reaction (DHTR occurred 7 days after an erythrocytapheresis or eritroexchange procedure (EEX treated with rituximab and glucocorticoids in a 15-years old patient with sickle cell disease. EEX was performed despite a previous diagnosis of alloimmunization, in order to reduce hemoglobin S rate before a major surgery for avascular necrosis of the femoral head. A first dose of rituximab was administered before EEX. However, rituximab couldn’t prevent DHTR that occurred with acute hemolysis, hemoglobinuria and hyper-bilirubinemia. A further dose of rituximab and three boli of methylprednisolone were given after the onset of the reaction. It is likely that the combined use of rituximab and steroids managed to gradually improve both patient’s general conditions and hemoglobin levels. Nor early or late side effects were registered in a 33-months follow-up period. This report suggests the potential effectiveness and safety of rituximab in combination with steroids in managing and mitigating the symptoms of delayed post-transfusional hemolytic reactions in alloimmunized patients affected by sickle cell disease with absolute need for erythrocytapheresis.

  8. [Microalbuminuria in pediatric patients diagnosed with hemolytic uremic syndrome].

    Science.gov (United States)

    Cubillos C, María Paz; Del Salas, Paulina; Zambrano, Pedro O

    2015-01-01

    Hemolytic uremic syndrome (HUS) is characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. It is the leading cause of acute kidney failure in children under 3 years of age. A variable number of patients develop proteinuria, hypertension, and chronic renal failure. To evaluate the renal involvement in pediatric patients diagnosed with HUS using the microalbumin/creatinine ratio. Descriptive concurrent cohort study that analyzed the presence of microalbuminuria in patients diagnosed with HUS between January 2001 and March 2012, who evolved without hypertension and normal renal function (clearance greater than 90ml/min using Schwartz formula). Demographic factors (age, sex), clinical presentation at time of diagnosis, use of antibiotics prior to admission, and need for renal replacement therapy were evaluated. Of the 24 patients studied, 54% were male. The mean age at diagnosis was two years. Peritoneal dialysis was required in 45%, and 33% developed persistent microalbuminuria. Antiproteinuric treatment was introduce in 4 patients, with good response. The mean follow-up was 6 years (range 6 months to 11 years). The serum creatinine returned to normal in all patients during follow up. The percentage of persistent microalbuminuria found in patients with a previous diagnosis of HUS was similar in our group to that described in the literature. Antiproteinuric treatment could delay kidney damage, but further multicenter prospective studies are necessary. Copyright © 2015. Publicado por Elsevier España, S.L.U.

  9. Advanced Prostate Cancer Presenting as Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    R. Ramos

    2013-01-01

    Full Text Available Introduction. Hemolytic uremic syndrome (HUS is characterized by endothelial dysfunction, consumption thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. HUS generally has a dismal prognosis, except when associated with gastroenteritis caused by verotoxin-producing bacteria. Cancer associated HUS is uncommon, and there are only scarce reports on prostate cancer presenting with HUS. Case Presentation. A 72-year-old man presented to the emergency department with oliguria, hematuria, and hematemesis. Clinical evaluation revealed acute renal failure, hemolysis, normal blood-clotting studies, and prostate-specific antigen value of 1000 ng/mL. The patient was started on hemodialysis, ultrafiltration with plasma exchange, and androgen blockade with bicalutamide and completely recovered from HUS. The authors review the 14 published cases on this association. Conclusion. The association of HUS and prostate cancer occurs more frequently in patients with high-grade, clinically advanced prostate cancer. When readily recognized and appropriately treated, HUS does not seem to worsen prognosis in prostate cancer patients.

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

    Directory of Open Access Journals (Sweden)

    Olivia Boyer

    2011-01-01

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

  11. Your Guide to Anemia

    Science.gov (United States)

    ... Inherited Causes l Folate or iron deficiency l Fanconi anemia from poor diet l Shwachman-Diamond l Demand ... cells, leading to aplastic anemia. These conditions include Fanconi anemia, Shwachman-Diamond syndrome, dyskeratosis congenita, Diamond- Blackfan anemia, ...

  12. About Anemia (For Kids)

    Science.gov (United States)

    ... Safe Videos for Educators Search English Español About Anemia KidsHealth / For Kids / About Anemia What's in this ... to every cell in your body. What Is Anemia? Anemia happens when a person doesn't have ...

  13. Hemolytic disease of the fetus and newborn in the molecular era.

    Science.gov (United States)

    Fasano, Ross M

    2016-02-01

    Maternal-fetal red cell antigen incompatibility can lead to alloimmunization, maternal immunoglobulin transplacental transfer, and hemolytic disease of the fetus and newborn (HDFN). The use of routine antenatal anti-D prophylaxis (RAADP) has sharply decreased the incidence of and mortality from HDFN due to RhD allosensitization. The ability to identify pregnancies/fetuses at risk of HDFN has significantly improved due to paternal molecular RHD zygosity testing, and non-invasive fetal molecular diagnostics for detecting putative antigen(s) (notably RhD) in fetuses utilizing cff-DNA in maternal plasma. Fetal RHD genotyping using cff-DNA has become increasingly accurate for fetal RHD detection, prompting some countries to implement targeted RAADP through mass screening programs of RhD-negative pregnant women. Along with middle cerebral artery Doppler ultrasonography for predicting fetal anemia, non-invasive fetal molecular diagnostics have greatly decreased the need for invasive diagnostic procedures in pregnancies at risk for severe HDFN. This review highlights these molecular advancements in HDFN-related prenatal diagnostics. Copyright © 2015 Elsevier Ltd. All rights reserved.

  14. Absence of hemolytic disease of fetus and newborn despite maternal high-titer IgG anti-Ku.

    Science.gov (United States)

    Kakaiya, R M; Whaley, A; Howard-Menk, C; Rami, J; Papari, M; Campbell-Lee, S; Malecki, Z

    2010-01-01

    Anti-Ku seen in K(o) (Kell-null) individuals has previously been shown to cause severe hemolytic transfusion reactions. Maternal anti-Ku can cause none or moderate to severe hemolytic disease of the fetus and newborn (HDFN). In two of four previously described HDFN cases, intrauterine transfusions were required because of severe anemia. We report a case in which maternal anti-Ku did not cause HDFN. Standard serologic methods were used for RBC antibody screening and identification, adsorption and elution of RBC antibodies, and antigen typing. A gravida 3, para 3 (G3P3) woman was first evaluated in 2006 and was found to have an IgG RBC antibody that reacted against all panel RBCs in the anti-human globulin phase. A panel of RBCs treated with DTT did not react with the antibody. The antibody failed to react with one example of K(o) RBCs. The patient’s RBCs typed negative for the following Kell blood group antigens: KEL1, KEL2, KEL3, KEL4, KEL6, KEL7, KEL11, KEL13, and KEL18. These results established the presence of anti-Ku in maternal serum. The newborn was group A, D+ and required phototherapy for hyperbilirubinemia, but did not require transfusion. The woman was seen again in January 2010 during the third trimester (G4P3). At this time, anti-Ku titer was 256. She delivered a healthy group O, D+ baby boy at 37 weeks' gestation. Cord RBCs were 4+ for IgG by DAT. An eluate reacted with all RBCs tested, but did not react when tested against a panel of DTT-treated RBCs. K(o) phenotype is rare to begin with, and the maternal anti-Ku formation may require more than one pregnancy. Therefore, cases that can be evaluated for anti-Ku–related HDFN are rare. Our case contributes to serologic and clinical aspects of such rare cases.

  15. Cancer-related anemia

    International Nuclear Information System (INIS)

    Abdel-Rzaeq, Hikmat N.

    2004-01-01

    Anemia is the most common hematological abnormality in cancer patients is often under-recognized and undertreated. The pathogenesis of cancer anemia is complex and most of time multifactorial; involving factors related to the tumor itself or its therapy. While anemia can be present in a wide range of symptoms, involing almost every organ, it is beleived that it contributes much to cancer-related-fatigue, one of the most common symptoms in cancer patients. In addition there is increasing evidence to suggest that anemia is an independent factor adversely affecting tumor reponse and patient survival. While blood transfusion was the only option to treat cancer related anemia, the use of recombinant human erythropoietin (rHuEPO) is becomig the new standard of care, more so with the recent studies demonstrating the feasibility of a sigle weekly injection .Things are even getting better with the recent approval of a new form of rHuEPO; Darbepoetin an analogue with a 3-fold longer half-life. In addition to its effects in raising homoglobin, several well controlled studies demonstrated decrease in transfusion requirementsand better qualify of life assessed objectively using standard assesments scales. (author)

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Heart and Vascular Diseases Precision Medicine Activities Obesity, Nutrition, and Physical Activity Population and Epidemiology Studies Women’s ... making new blood cells. Visit our Aplastic Anemia Health Topic to learn more. ... recommend that you take iron supplements, also called iron pills or oral iron, by mouth once or several times a ...

  17. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... of the body. When your heart has to work harder, this can lead to several conditions: irregular heartbeats called arrhythmias , a heart murmur , an ... chronic conditions, iron-deficiency anemia can make their condition worse or result in treatments not working as well. Look for Diagnosis will discuss any ...

  18. Worse outcome and more chronic GVHD with peripheral blood progenitor cells than bone marrow in HLA-matched sibling donor transplants for young patients with severe acquired aplastic anemia.

    NARCIS (Netherlands)

    Schrezenmeier, H.; Passweg, J.R.; Marsh, J.C.; Bacigalupo, A.; Bredeson, C.N.; Bullorsky, E.; Camitta, B.M.; Champlin, R.E.; Gale, R.P.; Fuhrer, M.; Klein, J.P.; Locasciulli, A.; Oneto, R.; Schattenberg, A.V.M.B.; Socie, G.; Eapen, M.

    2007-01-01

    We analyzed the outcome of 692 patients with severe aplastic anemia (SAA) receiving transplants from HLA-matched siblings. A total of 134 grafts were peripheral blood progenitor cell (PBPC) grafts, and 558 were bone marrow (BM) grafts. Rates of hematopoietic recovery and grades 2 to 4 chronic

  19. Anticardiolipin antibodies in classic pediatric hemolytic-uremic syndrome: a possible pathogenic role.

    Science.gov (United States)

    Ardiles, L G; Olavarría, F; Elgueta, M; Moya, P; Mezzano, S

    1998-01-01

    Anticardiolipin (aCL) antibodies have been associated with thrombocytopenia, hemolytic anemia and an increased risk of thrombosis in different vascular locations, even in the absence of lupus. The classic hemolytic-uremic syndrome is a postinfectious acute renal failure characterized by hemolytic anemia, thrombocytopenia and the presence of widespread glomerular thrombosis in the kidney, with pathogenic mechanisms that remain to be identified. In order to establish the frequency of aCL antibodies in this syndrome and to identify a possible role in the pathogenesis and clinical manifestations, 17 patients were studied during the reactant phase of the disease looking for an association between the presence of aCL antibodies (isotypes IgG, IgA and IgM) and the main clinical variables of the syndrome. In 8 patients IgG aCL was present, 2 patients had IgM aCL, and 1 had IgA antibodies on the solid-phase ELISA aCL assays, but no association could be demonstrated with the clinical variables studied. Although it might correspond to an epiphenomenon related to the triggering intestinal infection, a pathogenic role cannot be discarded and additional studies should be performed.

  20. Immunoglobulin transfusion in hemolytic disease of the newborn: place in therapy

    OpenAIRE

    Mundy CA; Bhatia J

    2015-01-01

    Cynthia A Mundy, Jatinder Bhatia Department of Pediatrics, Division of Neonatology, Georgia Regents University, Children's Hospital of Georgia, GA, USA Abstract: Hemolytic disease of the newborn continues to be a common neonatal disorder that requires a comprehensive understanding on the part of those caring for infants. Common treatments include hydration and phototherapy. Exchange transfusion is used in severe hemolytic disease, but infants undergoing this treatment are exposed to ...

  1. Anemia and survival in human immunodeficiency virus

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Mocroft, Amanda

    2003-01-01

    The prospective, multicenter cohort study EuroSIDA has previously reported on predictors and outcomes of anemia in patients infected with human immunodeficiency virus. In a Cox proportional-hazards model with serial measures of CD4+ cell count, plasma viral load, and degrees of anemia fitted...... as time-dependent variables, the relative hazard of death increased markedly for patients with anemia versus no anemia. A clinical scoring system was developed and validated for patients receiving highly active antiretroviral therapy using the most recent laboratory measures. Mild and severe anemia were...... independently (Panemia. The mechanisms underlying why hemoglobin is such a strong prognostic...

  2. Pernicious Anemia Associated Cobalamin Deficiency and Thrombotic Microangiopathy: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Farhanah Yousaf

    2017-01-01

    Full Text Available A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam. Presence of hemolytic anemia, schistocytosis, thrombocytopenia, and elevated lactate dehydrogenase (LDH was suggestive of thrombotic thrombocytopenic purpura (TTP. However, presence of leukopenia, macrocytes, and an inadequate reticulocyte response to the degree of anemia served as initial clues to an alternative diagnosis. Two and one units of packed red blood cells were transfused on day 1 and day 3, respectively. In addition, one unit of platelets was transfused on day 2. Daily therapeutic plasma exchange (TPE was initiated and continued until ADAMTS-13 result ruled out TTP. A low cobalamin (vitamin B12 level was evident at initial laboratory work-up and subsequent testing revealed positive intrinsic factor-blocking antibodies supporting a diagnosis of pernicious anemia with severe cobalamin deficiency. Hematological improvement was observed following vitamin B12 supplementation. The patient was discharged and markedly improved on day 9 with outpatient follow-up for cobalamin supplementation.

  3. Predictors of anemia in preschool children: Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project

    Science.gov (United States)

    Aaron, Grant J; Huang, Jin; Varadhan, Ravi; Temple, Victor; Rayco-Solon, Pura; Macdonald, Barbara

    2017-01-01

    Background: A lack of information on the etiology of anemia has hampered the design and monitoring of anemia-control efforts. Objective: We aimed to evaluate predictors of anemia in preschool children (PSC) (age range: 6–59 mo) by country and infection-burden category. Design: Cross-sectional data from 16 surveys (n = 29,293) from the Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project were analyzed separately and pooled by category of infection burden. We assessed relations between anemia (hemoglobin concentration anemia (hemoglobin concentration anemia with concomitant iron deficiency (defined as an inflammation-adjusted ferritin concentration anemia in >50% of surveys. Associations between breastfeeding and anemia were attenuated by controlling for child age, which was negatively associated with anemia. The most consistent predictors of severe anemia were malaria, poor sanitation, and underweight. In multivariable pooled models, child age, iron deficiency, and stunting independently predicted anemia and severe anemia. Inflammation was generally associated with anemia in the high- and very high–infection groups but not in the low- and medium-infection groups. In PSC with anemia, 50%, 30%, 55%, and 58% of children had concomitant iron deficiency in low-, medium-, high-, and very high–infection categories, respectively. Conclusions: Although causal inference is limited by cross-sectional survey data, results suggest anemia-control programs should address both iron deficiency and infections. The relative importance of factors that are associated with anemia varies by setting, and thus, country-specific data are needed to guide programs. PMID:28615260

  4. Inborn anemias in mice. Progress report, 1 May 1977--31 July 1978

    Energy Technology Data Exchange (ETDEWEB)

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

    1978-08-01

    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 autoimmune hemolytic anemia of NZB. 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: characterization of peripheral blood values, determinations of radiosensitivity under a variety of conditions, measurements of iron metabolism and heme synthesis, histological and biochemical study of blood-forming tissue, functional tests of the stem cell component, examination of responses to erythroid stimuli, and transplantation of tissue between individuals of differently affected genotypes. Considerable effort is devoted to perfection of hematologic, cell culture, and transplant methods to make these techniques useful in dealing with special problems associated with abnormal function.

  5. A case of atypical hemolytic uremic syndrome as an early manifestation of acute lymphoblastic leukemia

    Directory of Open Access Journals (Sweden)

    Dong Kyun Han

    2010-02-01

    Full Text Available Hemolytic uremic syndrome (HUS is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a tendency to relapse with a poorer prognosis. HUS has been reported to be associated with acute lymphoblastic leukemia (ALL in children. The characteristics and the mechanisms underlying this condition are largely unknown. In this study, we describe the case of an 11-year-old boy in whom the diagnosis of ALL was preceded by the diagnosis of atypical HUS. Thus, patients with atypical HUS should be diagnosed for the possibility of developing ALL.

  6. Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child.

    Science.gov (United States)

    Alparslan, Caner; Yavaşcan, Önder; Kasap Demir, Belde; Atmiş, Bahriye; Karabay Bayazit, Aysun; Leblebisatan, Göksel; Öncel, Elif P; Alaygut, Demet; Mutlubaş, Fatma; Aksu, Nejat

    2018-03-23

    Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult. We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed. The patient was treated with eculizumab, agressive blood pressure control, and antiepileptic drugs. At the fifth month of follow-up, the patient was diagnosed with acute B-cell lymphoblastic leukemia with fever, bone pain, hepatosplenomegaly, and pancytopenia. After initiation of ALL treatment, he had no episodes of HUS, despite cessation of eculizumab. In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed.

  7. Recurrent atypical hemolytic uremic syndrome after renal transplantation: treatment with eculizumab

    Directory of Open Access Journals (Sweden)

    Ana B. Latzke

    2018-04-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare entity. It is characterized by a thrombotic microangiopathy (nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure, with a typical histopathology of thickening of capillary and arteriolar walls and an obstructive thrombosis of the vascular lumen. The syndrome is produced by a genetic or acquired deregulation of the alternative pathway of the complement system, with high rates of end stage renal disease, post-transplant recurrence, and high mortality. Mutations associated with factor H, factor B and complement C3 show the worst prognosis. Even though plasma therapy is occasionally useful, eculizumab is effective both for treatment and prevention of post-transplant recurrence. We describe here an adult case of congenital aHUS (C3 mutation under preventive treatment with eculizumab after renal transplantation, with neither disease recurrence nor drug-related adverse events after a 36-months follow-up.

  8. Hemolytic disease of the newborn caused by a new deletion of the entire beta-globin cluster.

    OpenAIRE

    Pirastu, M; Kan, Y W; Lin, C C; Baine, R M; Holbrook, C T

    1983-01-01

    We describe a new type of gamma delta beta-thalassemia in four generations of a family of Scotch-Irish descent. The proposita presented with hemolytic disease of the newborn, which was characterized by a microcytic anemia. Initial restriction endonuclease analysis of the DNA showed no grossly abnormal patterns, but studies of polymorphic restriction sites and gene dosage revealed an extensive deletion that removed all the beta- and beta-like globin genes from the affected chromosome. In situ ...

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Research Home / < Back To Health Topics / Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer en español Iron-deficiency ... iron-deficiency anemia. Blood tests to screen for iron-deficiency anemia To screen for iron-deficiency anemia, your doctor ...

  10. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... To Health Topics / Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer en español Iron-deficiency ... anemia. Blood tests to screen for iron-deficiency anemia To screen for iron-deficiency anemia, your doctor ...

  11. Iron-Deficiency Anemia

    Science.gov (United States)

    ... To Health Topics / Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer en español Iron-deficiency ... anemia. Blood tests to screen for iron-deficiency anemia To screen for iron-deficiency anemia, your doctor ...

  12. Long-term neurodevelopmental outcome after intrauterine transfusion for hemolytic disease of the fetus/newborn: the LOTUS study.

    Science.gov (United States)

    Lindenburg, Irene T; Smits-Wintjens, Vivianne E; van Klink, Jeanine M; Verduin, Esther; van Kamp, Inge L; Walther, Frans J; Schonewille, Henk; Doxiadis, Ilias I; Kanhai, Humphrey H; van Lith, Jan M; van Zwet, Erik W; Oepkes, Dick; Brand, Anneke; Lopriore, Enrico

    2012-02-01

    To determine the incidence and risk factors for neurodevelopmental impairment (NDI) in children with hemolytic disease of the fetus/newborn treated with intrauterine transfusion (IUT). Neurodevelopmental outcome in children at least 2 years of age was assessed using standardized tests, including the Bayley Scales of Infant Development, the Wechsler Preschool and Primary Scale of Intelligence, and the Wechsler Intelligence Scale for Children, according to the children's age. Primary outcome was the incidence of neurodevelopmental impairment defined as at least one of the following: cerebral palsy, severe developmental delay, bilateral deafness, and/or blindness. A total of 291 children were evaluated at a median age of 8.2 years (range, 2-17 years). Cerebral palsy was detected in 6 (2.1%) children, severe developmental delay in 9 (3.1%) children, and bilateral deafness in 3 (1.0%) children. The overall incidence of neurodevelopmental impairment was 4.8% (14/291). In a multivariate regression analysis including only preoperative risk factors, severe hydrops was independently associated with neurodevelopmental impairment (odds ratio, 11.2; 95% confidence interval, 1.7-92.7). Incidence of neurodevelopmental impairment in children treated with intrauterine transfusion for fetal alloimmune anemia is low (4.8%). Prevention of fetal hydrops, the strongest preoperative predictor for impaired neurodevelopment, by timely detection, referral and treatment may improve long-term outcome. Copyright © 2012 Mosby, Inc. All rights reserved.

  13. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... detect signs of iron-deficiency anemia and help rule out other types of anemia. Treatment will explain ... your blood. More testing may be needed to rule out other types of anemia. Tests for gastrointestinal ...

  14. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... you are diagnosed with iron-deficiency anemia. Risk Factors You may have an increased risk for iron- ... iron-deficiency anemia if you have certain risk factors , including pregnancy. To prevent iron-deficiency anemia, your ...

  15. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... view the colon directly. What if my doctor thinks something else is causing my iron-deficiency anemia? ... deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in premature ...

  16. What Is Anemia?

    Science.gov (United States)

    ... Intramural Research Home / Anemia Anemia Also known as Iron-poor blood , Low blood , ... you or your child diagnosed with Diamond-Blackfan anemia? The registry is collecting information from people with ...

  17. Fanconi Anemia Research Fund

    Science.gov (United States)

    ... Support Publications Fundraising News What is the Fanconi Anemia Research Fund? Fanconi anemia is an inherited disease that can lead to ... population. Lynn and Dave Frohnmayer started the Fanconi Anemia Research Fund, in 1989 to find effective treatments ...

  18. Anemia and Pregnancy

    Science.gov (United States)

    ... Advocacy Toolkit Home For Patients Blood Disorders Anemia Anemia and Pregnancy Your body goes through significant changes ... becoming anemic. back to top Is Pregnancy-Related Anemia Preventable? Good nutrition is the best way to ...

  19. Musculoskeletal manifestations in sickle cell anemia

    Directory of Open Access Journals (Sweden)

    Reddy Ravikanth

    2017-01-01

    Full Text Available Sickle cell anemia is an inherited hemoglobin disorder characterized by substitution of glutamic acid by valine at the sixth position of the beta globin chain. The sequence of events leads to pain crisis. Ischemia of the tissues resulting from decreased blood flow is believed to occur in pain crisis. Repeated or prolonged sickling causes red cell death in the form of hemolytic anemia. The majority of hospital admissions are due to painful crisis. These patients are at increased risk for both osteomyelitis and infarction of the long bones. Magnetic resonance imaging has been shown to be helpful in the diagnosis of early osteomyelitis and its differentiation from infarction in sickle cell disease patients with acute bone crisis. Others findings include dactylitis, medullary infarcts, diploic space widening, fish mouth vertebrae, and avascular necrosis. We present a case series on the various musculoskeletal manifestations of sickle cell disease.

  20. Hemolytic uremic syndrome associated with Plasmodium vivax malaria successfully treated with plasma exchange

    Directory of Open Access Journals (Sweden)

    V S Keskar

    2014-01-01

    Full Text Available We report a case of hemolytic uremic syndrome (HUS in an adult patient with Plasmodium vivax malaria. The patient presented with worsening anemia, persistent thrombocytopenia and acute kidney injury. HUS was diagnosed based on the high serum lactate dehydrogenase, elevated reticulocyte count and presence of schistocytes on peripheral blood smear. Kidney biopsy showed features of thrombotic microangiopathy. Complete hematological remission was achieved after five sessions of therapeutic plasma exchange. Renal function partially recovered and stabilized at discharge. Vivax malaria, generally considered benign, may be rarely associated with HUS.

  1. Correlative study on anemia and radiotherapy effects in nasopharyngeal carcinoma

    International Nuclear Information System (INIS)

    Chen Jinsheng; Jiang Yuanshi; Cao Xibiao; Zhan Yongzhong; Yang Liye; Chen Jianxiu; Chen Chengwu; Li Yang

    2003-01-01

    Objective: To study the effect of oxygen-carrying ability of blood efficacy of radiotherapy for patients with nasopharyngeal carcinoma. Methods: Altogether 161 cases of patients with nasopharyngeal carcinoma were classified according to severity of anemia, and Hb, RBC, MCH, HCT, MCV, MCHC and RDW were tested before, during and after radiotherapy. The patients were followed-up for up to 5 years, the relationship and mechanism among anemia, radiotherapy effects and survival rate was discussed. Results: The survival rate between anemia group and non-anemia group was different significantly (P<0.05). Anemia before radiotherapy, anemia appearance or anemia deterioration during radiotherapy were sensitive factors affecting radiotherapy results. The anemia more severe, the radiotherapy worse. Conclusion: Anemia-hypohemoglobinemia leads to decrease of oxygen-carrying capacity of blood, resulting in oxygen deficiency of tumor cells and their radiotherapy resistance. Therefore this method is worthy of further studies

  2. Reduced dose cyclophosphamide, fludarabine and antithymocyte globulin for sibling and unrelated transplant of children with severe and very severe aplastic anemia.

    Science.gov (United States)

    Chung, Nack-Gyun; Lee, Jae Wook; Jang, Pil-Sang; Jeong, Dae-Chul; Cho, Bin; Kim, Hack-Ki

    2013-06-01

    We evaluated the results of a novel conditioning regimen of reduced dose cyclophosphamide (Cy, 25 mg/kg for four days), fludarabine (Flu, 30 mg/m(2) for four days), and rabbit ATG (2.5 mg/kg for three days) for allogeneic transplant of children with SAA, implemented since January 2009. Overall, 23 patients were treated with this regimen (16 male, seven female), including 10 diagnosed with VSAA. Donors included eight-MSD and 15 UD (five-matched UD, and 10 mismatched UD). All patients showed neutrophil and platelet engraftment. Cumulative incidence of acute (grade 2 or above) and chronic GVHD was 26.1% and 8.7%, respectively. Estimated two-yr FFS and OS for the entire cohort was 90.3 ± 6.5%. Rates of TRM and graft failure were 5.3% and 4.3%, respectively. No difference in OS was found according to disease severity (SAA vs. VSAA, p = 0.184), or according to donor type (MSD vs. UD, p = 0.699). Excellent outcomes of patients with VSAA underscore the efficacy of allogeneic transplant as a means of expediting hematopoietic recovery. Improved survival of UD transplant reaffirms its role as a valid therapeutic alternative in the absence of MSD. © 2013 John Wiley & Sons A/S.

  3. [Ischemic Changes in the Electrocardiogram and Circulatory Collapse Accompanied by Severe Anemia Owing to the Delay of Red Blood Cell Concentrate Transfusion in Two Patients with Intraoperative Massive Bleeding].

    Science.gov (United States)

    Horiuchi, Toshinori; Noguchi, Teruo; Kurita, Naoko; Yamaguchi, Ayako; Takeda, Masafumi; Sha, Keiichi; Nagahata, Toshihiro

    2016-01-01

    We present two patients developing intraoperative massive bleeding and showed ischemic changes in the electrocardiogram and circulatory collapse accompanied by severe anemia owing to the delay of red blood cell concentrate transfusion. One patient underwent hepatectomy and the other pancreaticoduodenectomy. Their lowest hemoglobin concentration was around 2 g x dl(-1), and they showed ischemic changes in the electrocardiogram and severe decreases in blood pressure. The former received compatible red blood cell concentrate and the latter received uncrossmatched same blood group red blood cell concentrate immediately, and their electrocardiogram and blood pressure quickly improved. To avoid life-threatening anemia, emergency red blood cell concentrate transfusion including compatible different blood group transfusion should be applied for intraoperative massive bleeding.

  4. T cell costimulation blockade promotes transplantation tolerance in combination with sirolimus and post-transplantation cyclophosphamide for haploidentical transplantation in children with severe aplastic anemia.

    Science.gov (United States)

    Jaiswal, Sarita Rani; Bhakuni, Prakash; Zaman, Shamsuz; Bansal, Satish; Bharadwaj, Priyanka; Bhargava, Sneh; Chakrabarti, Suparno

    2017-08-01

    We conducted a pilot study employing extended T cell costimulation blockade (COSBL) with Abatacept along with sirolimus and post-transplantation cyclophosphamide (PTCy) in 10 patients (median age 12) with severe aplastic anemia (SAA). Nine patients engrafted in the COSBL group, compared to all 10 patients (median 14 vs 13days) treated on PTCy protocols without abatacept (CONTROL group). The incidence of acute graft-versus-host disease (GVHD) was 10.5% in the COSBL group compared to 50% in the CONTROL group (p=0.04). Chronic GVHD (12.5% vs 56%, p=0.02) and CMV reactivation (30% vs 80%, p=0.03) were also reduced in the COSBL group. T and NK cell subset analysis revealed higher CD56 bright CD16 - NK cells in the CONTROL group (p=0.004), but similar CD56 dim CD16 + NK cells in both groups at day+30. Tregs (CD4 + CD25 + CD127 dim/- FoxP3+) were markedly higher in the COSBL group at day+30 (8.4% vs 1.1%) and the trend was maintained through day+90 (p<0.01). The GVHD and Disease-free survival at one year in the COSBL group was 80% vs. 30% in the CONTROL group (p=0.05). Our preliminary findings suggest that COSBL in combination with PTCy and sirolimus might augment transplantation tolerance in children with SAA, probably due to synergistic effect on early recovery of Tregs. Copyright © 2017 Elsevier B.V. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Sami eAlasfar

    2014-12-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1-2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (Infection-induced HUS and is frequently characterized by relapses that leads to end stage renal disease (ESRD. For a long time, kidney transplantation for these patients was contraindicated because of high rate of recurrence and subsequent renal graft loss. The post-kidney transplantation recurrence rate largely depends on the pathogenetic mechanisms involved. However, over the past several years, advancements in the understanding and therapeutics of aHUS have allowed successful kidney transplantation in these patients. Eculizumab, which is a complement C5 antibody that inhibits complement factor 5a (C5a and subsequent formation of the membrane attack complex, has been used in prevention and treatment of post-transplant aHUS recurrence. In this paper, we present two new cases of aHUS patients who underwent successful kidney transplantation in our center with the use of prophylactic and maintenance eculizumab therapy that have not been published before. The purpose of reporting these two cases is to emphasize the importance of using eculizumab as a prophylactic therapy to prevent aHUS recurrence post transplant in high-risk patients. We will also review the current understanding of the genetics of aHUS, the pathogenesis of its recurrence after kidney transplantation, and strategies for prevention and treatment of post-transplant aHUS recurrence.

  6. The Student with Sickle Cell Anemia.

    Science.gov (United States)

    Tetrault, Sylvia M.

    1981-01-01

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

  7. CLASSIFICATION AND DIAGNOSTICS OF ANEMIA IN CHILDREN

    OpenAIRE

    A. G. Rumyantsev

    2011-01-01

    Anemia in children is one of the most frequent somatic diseases. Criteria of anemia diagnosis are strictly regulated as decrease of hemoglobin/erythrocytes level accompanies majority of infectious, inflammatory, autoimmune, hereditary diseases and, in several cases, it is estimated as transitory disease in some periods of children’s growth and development. The article presents main classification and differential diagnostic schemes of anemia. Diagnostics makes accent on laboratory analysis; t...

  8. An unusual cause of anemia and encephalopathy

    Directory of Open Access Journals (Sweden)

    Sanjeev Kumar Sharma

    2015-04-01

    Full Text Available The authors present here an interesting case of recent onset anemia that was associated with an encephalopathy of the unusual cause.Although severe anemia can theoretically result in anemic hypoxia and can then lead to hypoxic encephalopathy, it is not a primary cause of encephalopathy. More frequently anemia can contribute together with other multiple causes of encephalopathy, such as infections, metabolic abnormalities, trauma, hepatic dysfunction, hypertension, toxins.

  9. Neutropenia in infants with hemolytic disease of the newborn.

    Science.gov (United States)

    Blanco, Esther; Johnston, Donna L

    2012-06-01

    This study examined the incidence, outcome and risk factors of neutropenia in infants with hemolytic disease of the newborn (HDN). A retrospective chart review was performed on infants with HDN. Of 69 evaluable infants, 45% developed neutropenia. Only one infectious complication was recorded. In most instances the neutropenia resolved spontaneously, but in seven infants it persisted for a median of 397 days. Males were at higher risk for developing neutropenia, but severity of HDN, antibody specificity, or therapy were not significant risk factors. Neutropenia is a common feature of HDN, regardless of severity of disease, treatment received, or antibody specificity. Copyright © 2011 Wiley Periodicals, Inc.

  10. Anemia as a risk factor for childhood asthma

    Directory of Open Access Journals (Sweden)

    Ramakrishnan K

    2010-01-01

    Full Text Available Objective: This prospective-(cohort study was conducted to evaluate whether anemia is a risk factor for childhood asthma. Materials and Methods: Two hundred children in the age group of 2-18 years who attended the Outpatient Department with upper respiratory / lower respiratory tract infections were included in this study. One hundred children with anemia were taken as the study group and another 100, age - and sex-matched children without anemia were taken as the control.They were subjected to complete blood count (CBC C-reactive protein (CRP estimation, Mantoux test and chest X-ray. Pulmonary function tests (PFTs were performed on those above six years showing evidence of asthma. Peripheral smear, serum ferritin and serum iron-binding capacity were estimated for all anemic children. Results: Asthma was present in 74 (74% children in the study group and in 33 (33% children in the control group. Iron-deficiency anemia was present in 85 (85% anemia of chronic infection in 20 (20% and the other five (5% had hemolytic anemia. Anemia was found to be a risk factor for childhood asthma. Conclusion: Anemic children were 5.75 times more susceptible to asthmatic attacks when compared with nonanemic children.

  11. [Anemia in the elderly].

    Science.gov (United States)

    Maerevoet, M; Sattar, L; Bron, D; Gulbis, B; Pepersack, T

    2014-09-01

    Anaemia is a problem that affects almost 10% over 65 years and 20% over 85 years. There is no physiological anaemia in the elderly. Any anaemia expresses the existence of a pathological process, regardless of its severity. Anaemia in the elderly is always associated with a poor prognosis that is in terms of mortality, morbidity and risk of fragility. The diagnostic approach to anemia in the elderly is the same as in younger individual. There are many causes of anaemia; anaemia balance is a complex diagnostic process. Most anaemias are due to a deficiency, chronic inflammation or comorbidity. However, in the elderly, the etiology of anaemia is often multifactorial. In a number of cases remain unexplained anaemia. In a number of cases, anemia remain unexplained. Treatment of anaemia is the treatment of the cause, but specific therapeutic aspects to the elderly should be considered, as among other martial substitution or use of erythropoietin (EPO).

  12. Fanconi anemia.

    Science.gov (United States)

    Soulier, Jean

    2011-01-01

    Fanconi anemia (FA) is the most frequent inherited cause of BM failure (BMF). Fifteen FANC genes have been identified to date, the most prevalent being FANCA, FANCC, FANCG, and FANCD2. In addition to classical presentations with progressive BMF during childhood and a positive chromosome breakage test in the blood, atypical clinical and/or biological situations can be seen in which a FA diagnosis has to be confirmed or eliminated. For this, a range of biological tools have been developed, including analysis of skin fibroblasts. FA patients experience a strong selective pressure in the BM that predisposes to clonal evolution and to the emergence in their teens or young adulthood of myelodysplasia syndrome (MDS) and/or acute myeloid leukemia (AML) with a specific pattern of somatic chromosomal lesions. The cellular mechanisms underlying (1) the hematopoietic defect which leads to progressive BMF and (2) somatic clonal evolutions in this background, are still largely elusive. Elucidation of these mechanisms at the molecular and cellular levels should be useful to understand the physiopathology of the disease and to adapt the follow-up and treatment of FA patients. This may also ultimately benefit older, non-FA patients with aplastic anemia, MDS/AML for whom FA represents a model genetic condition.

  13. Complement Mutations in Diacylglycerol Kinase-ε–Associated Atypical Hemolytic Uremic Syndrome

    Science.gov (United States)

    Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

    2014-01-01

    kinase-ε and C3 mutations. Conclusions Data suggest that complement dysregulation influences the onset and disease severity in carriers of diacylglycerol kinase-ε mutations and that treatments on the basis of plasma infusions and complement inhibition are potentially useful in patients with combined diacylglycerol kinase-ε and complement mutations. A comprehensive understanding of the genetic component predisposing to atypical hemolytic uremic syndrome is, therefore, critical to guide an effective treatment. PMID:25135762

  14. Complement mutations in diacylglycerol kinase-ε-associated atypical hemolytic uremic syndrome.

    Science.gov (United States)

    Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

    2014-09-05

    the onset and disease severity in carriers of diacylglycerol kinase-ε mutations and that treatments on the basis of plasma infusions and complement inhibition are potentially useful in patients with combined diacylglycerol kinase-ε and complement mutations. A comprehensive understanding of the genetic component predisposing to atypical hemolytic uremic syndrome is, therefore, critical to guide an effective treatment. Copyright © 2014 by the American Society of Nephrology.

  15. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... anemia, your doctor may order the following blood tests to diagnose iron-deficiency anemia: Complete blood count (CBC) to ... than normal when viewed under a microscope. Different tests help your doctor diagnose iron-deficiency anemia. In iron-deficiency anemia, blood ...

  16. Iron-Deficiency Anemia

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    Full Text Available ... en español Iron-deficiency anemia is a common type of anemia that occurs if you do not ... iron-deficiency anemia and help rule out other types of anemia. Treatment will explain treatment-related complications ...

  17. Anemia (For Teens)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Anemia KidsHealth / For Teens / Anemia What's in this article? ... Enough Iron Print en español Anemia What Is Anemia? Lots of teens are tired. With all the ...

  18. Fetal Anemia and Hydrops Fetalis Associated with Homozygous Hb Constant Spring (HBA2: c.427T > C).

    Science.gov (United States)

    He, Yi; Zhao, Ying; Lou, Ji-Wu; Liu, Yan-Hui; Li, Dong-Zhi

    2016-01-01

    Hb Constant Spring (Hb CS, HBA2: c.427T > C) is a common nondeletional α-thalassemia (α-thal) that results from a nucleotide substitution at the termination codon of the α2-globin gene. Homozygosity for Hb CS (α(CS)α/α(CS)α) is relatively rare, and generally characterized with mild hemolytic anemia, jaundice, and splenomegaly. In this report we present a fetus with cardiomegaly, pericardial effusion, enlarged placenta and increased middle cerebral artery-peak systolic velocity (MCA-PSV) at 24 weeks' gestation. Fetal blood sampling revealed the severe anemia [hemoglobin (Hb) level being 4.8 g/dL] and Hb H (β4) disease-like hematological findings with Hb Bart's (γ4) level of 17.9%. DNA sequencing of the α-globin genes found that both partners were Hb CS carriers and the fetus was an Hb CS homozygote. Therefore, this was a rare case of homozygous Hb CS which demonstrated an unusual and serious anemia and hydrops fetalis in utero.

  19. Fatal hemolytic disease of the fetus and newborn associated with anti-Jr.

    Science.gov (United States)

    Peyrard, Thierry; Pham, Bach-Nga; Arnaud, Lionel; Fleutiaux, Sophie; Brossard, Yves; Guerin, Bénédicte; Desmoulins, Isabelle; Rouger, Philippe; Le Pennec, Pierre-Yves

    2008-09-01

    Jr(a) is a high-prevalence red cell (RBC) antigen. The clinical significance of anti-Jr(a) is controversial. When hemolytic disease of the fetus and newborn (HDFN) occurred, most reported cases were clinically mild. We report the first case of fatal HDFN due to anti-Jr(a). A 28-year-old Caucasian woman with transfusion history was monitored at the 29th week of pregnancy (G4P1). An ultrasound scan showed fetal cardiomegaly and hepatomegaly. An antibody directed against a high-prevalence antigen was detected, but without conclusive identification. An emergency cesarean section was performed at the 36th week. The newborn was hydropic and showed severe anemia. Death occurred 30 hours after birth. Serologic methods were performed to investigate the mother's RBCs and serum. An in vitro functional cellular assay and semiquantitative measurement of anti-Jr(a) were used to determine the clinical significance of the antibody. Anti-Jr(a) was identified in the serum and Jr(a-) phenotype was confirmed. The anti-Jr(a) titer was 1024, with predominant immunoglobulin (Ig)G1 and minor IgG4 subclasses. The functional cellular assay was consistent with an antibody unlikely to cause HDFN. Semiquantitative measurement of anti-Jr(a) showed a reactivity equivalent to a 25 IU per mL (5 microg/mL) concentration of anti-D, a value associated with a significant risk of HDFN. This is the first documented case of fatal HDFN due to anti-Jr(a). Therefore, we recommend close monitoring of pregnant women with a high-titer anti-Jr(a), especially those with an incompatible transfusion history and/or multiple pregnancies. This case report provides new arguments about the clinical significance of anti-Jr(a) in the transfusion setting.

  20. Reticulocyte parameters in hemoglobinopathies and iron deficiency anemia

    Directory of Open Access Journals (Sweden)

    Cortellazzi Laura C.

    2003-01-01

    Full Text Available Flow cytometric reticulocyte analysis allows the evaluation of reticulocyte maturity. New reticulocyte parameters have been used in the diagnosis and management of anemias, in the bone marrow transplant setting and in the monitoring of iron replacement or erythropoiet in therapy. Reticulocyte numbers and maturation levels have been studied in different hemoglobinopathies and the results have been correlated with the degree of ineffective erythropoiesis. In order to verify differences in reticulocyte parameters in various types of anemias and to test the absolute number of immature reticulocytes as a possible discriminating factor among various types of anemias, reticulocyte counts were performed on 219 samples from patients with sickle cell anemia (SS (n= 62, hemoglobin S trait (n=9, Sbeta thalassemia (n=7, hemoglobin SC disease (n=11, beta thalassemia trait (n=33 and iron deficiency anemia (n= 47, and non-anemic individuals (n= 50. Mean fluorescence index (MFI was defined as representative of the degree of reticulocyte immaturity and it was evaluated as a percentage and in absolute values. Reticulocyte counts and MFI values were significantly higher in SS, Sbeta thalassemic and SC groups when compared to controls, but not different among the three anemia groups. Patients with hemoglobin S trait, iron deficiency anemia and beta thalassemia trait showed reticulocyte parameters similar to the non-anemic group. There was no difference between the b thalassemic trait and iron deficiency anemia in relation to any parameters. MFI in absolute numbers were significantly higher in anemias that develop with the hemolytic process, although this was not evident in MFI percentage values. Our results showed that the erythoid expansion in sickle cell diseases (SS, SC and Sb thalassemia leads to an enhanced immature reticulocyte release from bone marrow and that the phenomena is more evident by the MFI counting in absolute figures than in percentages. We

  1. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2010-09-01

    Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

  2. [A comparative study of unrelated donor and matched-sibling donor allogeneic hematopoietic stem cell transplantation in children and adolescents with acquired severe aplastic anemia].

    Science.gov (United States)

    Zhou, J; Fu, Y W; Liang, L J; Wang, Q; Han, L J; Zu, Y L; Zhang, Yanli; Zhu, X H; Yu, F K; Fang, B J; Wei, X D; Song, Y P

    2016-12-01

    Objective: To evaluate the efficacy of unrelated donor allogeneic hematopoietic stem cell transplantation(URD allo-HSCT) for children and adolescents with severe aplastic anemia (SAA). Methods: Clinical data of 34 SAA children and adolescents undergoing allo-HSCT were retrospectively analyzed from October 2001 to October 2015. According to the source of donor, the patients were divided into matched sibling donor allo-HSCT group (MSD group) and unrelated donor group (URD group). The clinical outcome of SAA children and adolescents receiving URD allo-HSCT was assessed, and patients in MSD allo-HSCT group were enrolled as control at the same period. Results: The rate of hematopoietic reconstitution, the time of neutrophil and platelet engraftment, incidence of chimerism and graft rejection between two groups were not statistically different.The incidence of acute graft-versus-host disease (GVHD) in URD group was significantly higher than that in MSD group [42.9%(6/14) vs 10.5%(2/19), P =0.047]. The incidence of grade Ⅱ-Ⅳ acute GVHD and chronic GVHD in URD were higher than those in MSD group [21.4%(3/14) vs 5.3%(1/19), P =0.288; 35.7%(5/14) vs 5.3%(1/19), P =0.062, respectively], yet without significant difference between two groups. Other transplant-related complications including pulmonary complications, hemorrhagic cystitis, incidence of EBV and CMV reactivation and venous occlusive disease were comparable with two regimens. Estimated 5-years overall survival (OS) rate and disease free survival (DFS) rate were not statistically significant between URD group and MSD group [(84.4±6.6)% vs (89.4±7.1)%, (82.5±5.4)% vs (82.1±4.3)%; P =0.766, P =0.884, respectively]. Conclusions: By multivariate analysis, the outcome of URD allo-HSCT in SAA children and adolescent is similar to MSD allo-HSCT. It could be an alternative option as the first-line treatment for SAA children and adolescents without HLA matched sibling donors.

  3. Hemolytic disease in the newborn - history and prevention in the world and the Czech Republic.

    Science.gov (United States)

    Santavy, Jiri

    2010-06-01

    Hemolytic disease in the newborn with its typical signs and poor prognosis has been known for centuries. Historically it can be divided into three pathological states which are fetal hydrops (hydrops fetus universalis), neonatal jaundice (icterus neonati gravis familiaris) and fetal anemia (anemia neonati). Almost 70 reports with quite accurate descriptions were found up to the end of 19th century. The patho physiological basis of the condition began to be studied at the beginning of the last century and the development of our knowledge is an example of the cooperation between pathologists, pediatricians, hematologists and later, obstetricians, immunologists and geneticists. Despite all the advances in this field it remains a serious disease up to this time. It is not managed successfully in all cases and despite successful immunological prophylaxis there are cases when we need to administer intrauterine transfusion based on the information received by dopplerometric measurement of arteria cerebri perfusion and fetal blood sampling. Review of lover cited literature. The history of the hemolytic disease in the newborn, its condition and approaches to it has not been recently compiled in the Czech Republic.

  4. A Case of Hemolytic Disease of the Newborn due to Dia Antibody

    Science.gov (United States)

    Jethava, Ashif; Olivares, Esperanza; Shariatmadar, Sherry

    2015-01-01

    Anti-Dia is a clinically significant red cell antibody known to cause hemolytic disease of the newborn. Here, we report on a case of mild hemolytic disease of the newborn caused by Dia antibody. The mother had three prior pregnancies with no history of blood transfusion. She delivered a preterm 35-week-old female newborn by cesarean section. The neonate developed anemia and mild icterus on postnatal day five with hemoglobin of 9500 mg/dL and total bilirubin of 10 mg/dL. The direct antiglobulin test on the neonate's red blood cells was positive. The maternal serum and an eluate from the infant RBCs were negative in routine antibody detection tests but were positive using commercially prepared Di(a+) red cells. The neonate was discharged home in stable condition following treatment with erythropoietin and phototherapy. When a newborn has a positive DAT in the absence of major blood group incompatibility or commonly detected RBC antibodies, an antibody to a low frequency antigen such as Dia must be considered. Further immunohematology tests are required to determine presence of the antibody and the clinician must be alerted to closely monitor the infant for signs of anemia and hemolysis. PMID:26682081

  5. A Case of Hemolytic Disease of the Newborn due to Di (a) Antibody.

    Science.gov (United States)

    Jethava, Ashif; Olivares, Esperanza; Shariatmadar, Sherry

    2015-01-01

    Anti-Di(a) is a clinically significant red cell antibody known to cause hemolytic disease of the newborn. Here, we report on a case of mild hemolytic disease of the newborn caused by Di(a) antibody. The mother had three prior pregnancies with no history of blood transfusion. She delivered a preterm 35-week-old female newborn by cesarean section. The neonate developed anemia and mild icterus on postnatal day five with hemoglobin of 9500 mg/dL and total bilirubin of 10 mg/dL. The direct antiglobulin test on the neonate's red blood cells was positive. The maternal serum and an eluate from the infant RBCs were negative in routine antibody detection tests but were positive using commercially prepared Di(a+) red cells. The neonate was discharged home in stable condition following treatment with erythropoietin and phototherapy. When a newborn has a positive DAT in the absence of major blood group incompatibility or commonly detected RBC antibodies, an antibody to a low frequency antigen such as Di(a) must be considered. Further immunohematology tests are required to determine presence of the antibody and the clinician must be alerted to closely monitor the infant for signs of anemia and hemolysis.

  6. A Case of Hemolytic Disease of the Newborn due to Dia Antibody

    Directory of Open Access Journals (Sweden)

    Ashif Jethava

    2015-01-01

    Full Text Available Anti-Dia is a clinically significant red cell antibody known to cause hemolytic disease of the newborn. Here, we report on a case of mild hemolytic disease of the newborn caused by Dia antibody. The mother had three prior pregnancies with no history of blood transfusion. She delivered a preterm 35-week-old female newborn by cesarean section. The neonate developed anemia and mild icterus on postnatal day five with hemoglobin of 9500 mg/dL and total bilirubin of 10 mg/dL. The direct antiglobulin test on the neonate’s red blood cells was positive. The maternal serum and an eluate from the infant RBCs were negative in routine antibody detection tests but were positive using commercially prepared Di(a+ red cells. The neonate was discharged home in stable condition following treatment with erythropoietin and phototherapy. When a newborn has a positive DAT in the absence of major blood group incompatibility or commonly detected RBC antibodies, an antibody to a low frequency antigen such as Dia must be considered. Further immunohematology tests are required to determine presence of the antibody and the clinician must be alerted to closely monitor the infant for signs of anemia and hemolysis.

  7. Hemolytic Disease of the Newborn Due to Anti-c Isoimmunization: A Case Report.

    Science.gov (United States)

    Sheeladevi, C S; Suchitha, S; Manjunath, G V; Murthy, Srinivas

    2013-09-01

    The Rhesus (Rh) blood group is one of the most complex blood groups known in humans. It has remained of primary importance in obstetrics, being the main cause of hemolytic disease of the newborn (HDN). Anti-D causes the most severe form of HDN. Other Rh allo antibodies that are capable of causing severe HDN include anti-c, which clinically is the most important Rh antigen after the D antigen. We report a case of hemolytic disease of the newborn due to Rh anti-c in an infant of an Rh positive mother.

  8. Imaging Diagnosis of Neonatal Anemia: Report of Two Unusual Etiologies

    Directory of Open Access Journals (Sweden)

    Shabnam Bhandari Grover

    2013-01-01

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

  9. Immunoglobulin transfusion in hemolytic disease of the newborn: place in therapy

    Directory of Open Access Journals (Sweden)

    Mundy CA

    2015-06-01

    Full Text Available Cynthia A Mundy, Jatinder Bhatia Department of Pediatrics, Division of Neonatology, Georgia Regents University, Children's Hospital of Georgia, GA, USA Abstract: Hemolytic disease of the newborn continues to be a common neonatal disorder that requires a comprehensive understanding on the part of those caring for infants. Common treatments include hydration and phototherapy. Exchange transfusion is used in severe hemolytic disease, but infants undergoing this treatment are exposed to many adverse effects. Intravenous immunoglobulin is a newer strategy that is showing promise in the treatment of the disease. This review discusses the current use and future expectations of intravenous immunoglobulin therapy in newborns. Keywords: hyperbilirubinemia, ABO incompatibility, neonatal jaundice 

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

    OpenAIRE

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

    2013-01-01

    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 wit...

  11. Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

    Science.gov (United States)

    Lobo, Clarisse Lopes de Castro; do Nascimento, Emilia Matos; Abelha, Renato; Queiroz, Ana Maria Mach; Connes, Philippe; Cardoso, Gilberto Perez; Ballas, Samir K

    2015-01-01

    This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV) 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH) levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr) > 1.0 mg/dL was lower than the group with Cr ≤ 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.

  12. Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

    Directory of Open Access Journals (Sweden)

    Clarisse Lopes de Castro Lobo

    Full Text Available This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr > 1.0 mg/dL was lower than the group with Cr ≤ 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.

  13. A case of severe glutathione synthetase deficiency with novel GSS mutations

    Science.gov (United States)

    Xia, H.; Ye, J.; Wang, L.; Zhu, J.; He, Z.

    2018-01-01

    Glutathione synthetase deficiency (GSSD) is a rare inborn error of glutathione metabolism with autosomal recessive inheritance. The severe form of the disease is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. A case of a male newborn infant who had severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia is reported. A high level of 5-oxoproline was detected in his urine and a diagnosis of generalized GSSD was made. DNA sequence analysis revealed the infant to be compound heterozygous with two mutations, c.738dupG in exon 8 of GSS gene resulting in p.S247fs and a repetitive sequence in exon 3 of GSS gene. Treatment after diagnosis of GSSD included supplementation with antioxidants and oral sodium hydrogen bicarbonate. However, he maintained a variable degree of metabolic acidosis and succumbed shortly after his parents requested discontinuation of therapy because of dismal prognosis and medical futility when he was 18 days old. PMID:29340523

  14. A case of severe glutathione synthetase deficiency with novel GSS mutations

    Directory of Open Access Journals (Sweden)

    H. Xia

    2018-01-01

    Full Text Available Glutathione synthetase deficiency (GSSD is a rare inborn error of glutathione metabolism with autosomal recessive inheritance. The severe form of the disease is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. A case of a male newborn infant who had severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia is reported. A high level of 5-oxoproline was detected in his urine and a diagnosis of generalized GSSD was made. DNA sequence analysis revealed the infant to be compound heterozygous with two mutations, c.738dupG in exon 8 of GSS gene resulting in p.S247fs and a repetitive sequence in exon 3 of GSS gene. Treatment after diagnosis of GSSD included supplementation with antioxidants and oral sodium hydrogen bicarbonate. However, he maintained a variable degree of metabolic acidosis and succumbed shortly after his parents requested discontinuation of therapy because of dismal prognosis and medical futility when he was 18 days old.

  15. Iron-Deficiency Anemia

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    Full Text Available ... heart failure . Increased risk of infections Motor or cognitive development delays in children Pregnancy complications, such as ... for iron-deficiency anemia. Learn about exciting research areas that NHLBI is exploring about iron-deficiency anemia. ...

  16. Iron-Deficiency Anemia

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    Full Text Available ... Hemophilia Pernicious Anemia Restless Legs Syndrome Von Willebrand Disease Other Resources NHLBI resources Your Guide to Anemia [PDF, 1.54MB] Cardiovascular Health Study Recipient Epidemiology Donor Studies (REDS) program ...

  17. Iron-Deficiency Anemia

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    Full Text Available ... hemoglobin levels. This was associated with a greater risk of death even with mild anemia. Now, anemia in older adults is recognized as an important condition. NHLBI Small Business Program. Through the NHLBI Small Business Program , we ...

  18. Iron-Deficiency Anemia

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    Full Text Available ... to improve health through research and scientific discovery. Improving health with current research Learn about the following ... deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in premature ...

  19. Iron-Deficiency Anemia

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    Full Text Available ... with the Eunice Kennedy Shriver National Institute of Child Health and Human Development, we are investigating how ... Anemia in Chronic Kidney Disease (National Institute of Diabetes and Digestive and Kidney Diseases) Avoiding Anemia (National ...

  20. Iron-Deficiency Anemia

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    Full Text Available ... deficiency anemia can cause serious complications, including heart failure and development delays in children. Explore this Health ... lead to iron-deficiency anemia include: End-stage kidney failure, where there is blood loss during dialysis. ...

  1. Iron-Deficiency Anemia

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    Full Text Available ... iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in ... Visit Children and Clinical Studies to hear experts, parents, and children talk about their experiences with clinical ...

  2. Iron-Deficiency Anemia

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    Full Text Available ... may be diagnosed with iron-deficiency anemia if you have low iron or ferritin levels in your blood. More testing may be needed to rule out other types of anemia. Tests for gastrointestinal ...

  3. Iron-Deficiency Anemia

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    Full Text Available ... for iron-deficiency anemia. Lifestyle habits Certain lifestyle habits may increase your risk for iron-deficiency anemia, including: Vegetarian or vegan eating patterns. Not eating enough iron-rich foods, such ...

  4. Iron-Deficiency Anemia

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    Full Text Available ... deficiency anemia can cause serious complications, including heart failure and development delays in children. Explore this Health ... to iron-deficiency anemia include: End-stage kidney failure, where there is blood loss during dialysis. People ...

  5. Iron-Deficiency Anemia

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    Full Text Available ... mg and women need 18 mg. After age 51, both men and women need 8 mg. Pregnant ... for iron-deficiency anemia. Learn about exciting research areas that NHLBI is exploring about iron-deficiency anemia. ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron-deficiency anemia. These conditions include: Intestinal and digestive conditions, such as celiac disease; inflammatory bowel diseases, ... iron-deficiency anemia , such as bleeding in the digestive or urinary tract or heavy menstrual bleeding, your ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... less than 12 g/dl for women is diagnostic of anemia. In iron-deficiency anemia, red blood ... both full-term and preterm infants. Look for Diagnosis will explain tests and procedures that your doctor ...

  8. Iron-Deficiency Anemia

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    Full Text Available ... less than 12 g/dl for women is diagnostic of anemia. In iron-deficiency anemia, red blood ... physical exam, or order blood tests or other diagnostic tests. Physical exam Your doctor may ask about ...

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron-fortified foods that have iron added. Vegetarian diets can provide enough iron if you choose nonmeat ... Anemia in Chronic Kidney Disease (National Institute of Diabetes and Digestive and Kidney Diseases) Avoiding Anemia (National ...

  10. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... conditions that can cause iron-deficiency anemia. Blood tests to screen for iron-deficiency anemia To screen ... the size of your liver and spleen. Blood tests Based on results from blood tests to screen ...

  11. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... from developing iron-deficiency anemia. Foods that are good sources of iron include dried beans, dried fruits, eggs, lean red meat, ... signs of iron-deficiency anemia include: Brittle nails ...

  12. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... your doctor may recommend you eat heart-healthy foods or control other conditions that can cause iron-deficiency anemia. Blood tests to screen for iron-deficiency anemia To screen ...

  13. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... learning how having iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. ... iron-deficiency anemia in blood donors affects the quality of donated red blood cells, such as how ...

  14. Vitamin Deficiency Anemia

    Science.gov (United States)

    ... are unique to specific vitamin deficiencies. Folate-deficiency anemia risk factors include: Undergoing hemodialysis for kidney failure. ... the metabolism of folate. Vitamin B-12 deficiency anemia risk factors include: Lack of intrinsic factor. Most ...

  15. Sickle cell anemia

    Science.gov (United States)

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

  16. Side Effects: Anemia

    Science.gov (United States)

    Anemia is a side effect of cancer treatments, including chemotherapy and radiation therapy. It can make women and men feel fatigued, dizzy, and short of breath. Learn how to manage fatigue caused by anemia during cancer treatment.

  17. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... endoscopy or colonoscopy, to stop bleeding. Healthy lifestyle changes To help you meet your daily recommended iron ... iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in ...

  18. Iron-Deficiency Anemia

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    Full Text Available ... and pregnancy. Good sources of iron are meat, poultry, fish, and iron-fortified foods that have iron ... Anemia Restless Legs Syndrome Von Willebrand Disease Other Resources NHLBI resources Your Guide to Anemia [PDF, 1. ...

  19. Sickle cell anemia.

    OpenAIRE

    ŘÍHOVÁ, Tereza

    2013-01-01

    This thesis is about the disease called sickle cell anemia, or drepanocytosis. In this thesis is described the history of the disease, pathophysiology, laboratory features, various clinical features, diferencial diagnosis, quality of life in sickle cell anemia and therapy.

  20. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... leaving cells where it is stored or from being absorbed in the duodenum, the first part of ... treatments for iron-deficiency anemia. Living With After being diagnosed with iron-deficiency anemia, it is important ...

  1. Iron-Deficiency Anemia

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    Full Text Available ... exploring about iron-deficiency anemia. Read more New treatments for disorders that lead to iron-deficiency anemia. We are ... and other pathways. This could help develop new therapies for conditions that ... behavior, thinking, and mood during adolescence. Treating anemia in ...

  2. Iron-Deficiency Anemia

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    Full Text Available ... anemia if you have certain risk factors , including pregnancy. To prevent iron-deficiency anemia, your doctor may recommend you eat heart-healthy foods or control other conditions that can cause iron-deficiency anemia. Blood tests to screen for ...

  3. Iron-Deficiency Anemia

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    Full Text Available ... Home / < Back To Health Topics / Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer en español ... bleeding Consuming less than recommended daily amounts of iron Iron-deficiency anemia can be caused by getting ...

  4. Iron-Deficiency Anemia

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    Full Text Available ... Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer en español Iron-deficiency anemia is a ... address the cause of your iron deficiency, such as any underlying bleeding. If undiagnosed or untreated, iron- ...

  5. Iron-Deficiency Anemia

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    Full Text Available ... Topics section only, or the News and Resources section. NHLBI Entire Site NHLBI Entire Site Health ... español Iron-deficiency anemia is a common type of anemia that occurs if you do not have enough iron in your body. People with mild or moderate iron-deficiency anemia ...

  6. Iron-Deficiency Anemia

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    Full Text Available ... Topics News & Resources Intramural Research Home / < Back To Health Topics / Iron-Deficiency Anemia Iron-Deficiency Anemia Also known as Leer ... and symptoms as well as complications from iron-deficiency anemia. Research for Your Health The NHLBI is part of the U.S. Department ...

  7. Iron-Deficiency Anemia

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    Full Text Available ... for iron-deficiency anemia if you have certain risk factors , including pregnancy. To prevent iron-deficiency anemia, your doctor may recommend you eat heart-healthy foods or control other conditions that can cause iron-deficiency anemia. ...

  8. Iron deficiency anemia

    Science.gov (United States)

    Anemia - iron deficiency ... iron from old red blood cells. Iron deficiency anemia develops when your body's iron stores run low. ... You may have no symptoms if the anemia is mild. Most of the time, ... slowly. Symptoms may include: Feeling weak or tired more often ...

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Medicine (TOPMed) Program Non-NHLBI resources Anemia (National Library of Medicine, MedlinePlus) Anemia in Chronic Kidney Disease ( ... Supplement Fact Sheet (NIH) Iron-Deficiency Anemia (National Library of Medicine, MedlinePlus) Building 31 31 Center Drive ...

  10. Contribution of hly homologs to the hemolytic activity of Prevotella intermedia.

    Science.gov (United States)

    Suzuki, Naoko; Fukamachi, Haruka; Arimoto, Takafumi; Yamamoto, Matsuo; Igarashi, Takeshi

    2012-06-01

    Prevotella intermedia is a periodontal pathogen that requires iron for its growth. Although this organism has hemolytic activity, the precise nature of its hemolytic substances and their associated hemolytic actions are yet to be fully determined. In the present study, we identified and characterized several putative hly genes in P. intermedia ATCC25611 which appear to encode hemolysins. Six hly genes (hlyA, B, C, D, E, and hlyI) of P. intermedia were identified by comparing their nucleotide sequences to those of known hly genes of Bacteroides fragilis NCTC9343. The hlyA-E, and hlyI genes were overexpressed individually in the non-hemolytic Escherichia coli strain JW5181 and examined its contribution to the hemolytic activity on sheep blood agar plates. E. coli cells expressing the hlyA and hlyI genes exhibited hemolytic activity under anaerobic conditions. On the other hand, only E. coli cells stably expressing the hlyA gene were able to lyse the red blood cells when cultured under aerobic conditions. In addition, expression of the hlyA and hlyI genes was significantly upregulated in the presence of red blood cells. Furthermore, we found that the growth of P. intermedia was similar in an iron-limited medium supplemented with either red blood cells or heme. Taken together, our results indicate that the hlyA and hlyI genes of P. intermedia encode putative hemolysins that appear to be involved in the lysis of red blood cells, and suggest that these hemolysins might play important roles in the iron-dependent growth of this organism. Copyright © 2012 Elsevier Ltd. All rights reserved.

  11. Anemia: An approach to evaluation, 2014

    Directory of Open Access Journals (Sweden)

    Philip Kuriakose

    2015-01-01

    Full Text Available Anemia is very commonly encountered in general clinical practice among all age groups. The more commonly used way to classify anemia has been to categorize it as being microcytic (mean corpuscular volume [MCV] 100 fL, which in turn allows for a more practical way to attempt to come up with a cause for any decrease in hemoglobin. Microcytic anemias are usually due to iron deficiency (in turn, a result of a number of different etiologies ranging from decreased intake, malabsorption, or blood loss, hemoglobinopathies (thalassemic syndromes, and some cases of severe anemia resulting from chronic disease. Normocytic anemia is often a result of anemia of chronic disease, hemolysis, or secondary to bone marrow failure. Macrocytic anemias are frequently caused by deficiencies of folic acid and/or Vitamin B12, exposure to toxic agents like drugs that interfere with DNA metabolism and alcohol, as also bone marrow failure states, such as from myelodysplastic syndrome. A comprehensive history, physical examination, and directed laboratory evaluation will help to identify a specific cause for anemia.

  12. C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction

    Directory of Open Access Journals (Sweden)

    Ravneet Bajwa

    2018-02-01

    Full Text Available The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS, renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement. In this report, we present 2 cases of alternative pathway dysfunction. The 60-year-old female patient had biopsy-proven C3 glomerulopathy, while the 32-year-old female patient was diagnosed with aHUS based on renal dysfunction, thrombocytopenia, anemia, and normal ADAMTS-13 level. The aHUS patient was successfully treated with the monoclonal antibody (eculizumab for complement blockade. The patient with C3 glomerulopathy did not receive the monoclonal antibody. In this patient, management focused on blood pressure and proteinuria control with an angiotensin-converting enzyme inhibitor. This article focuses on the clinical differences, pathophysiology, and treatment of aHUS and C3 glomerulopathy.

  13. Magnitude of Anemia at Discharge Increases 30-Day Hospital Readmissions.

    Science.gov (United States)

    Koch, Colleen G; Li, Liang; Sun, Zhiyuan; Hixson, Eric D; Tang, Anne; Chagin, Kevin; Kattan, Michael; Phillips, Shannon C; Blackstone, Eugene H; Henderson, J Michael

    2017-12-01

    Anemia during hospitalization is associated with poor health outcomes. Does anemia at discharge place patients at risk for hospital readmission within 30 days of discharge? Our objectives were to examine the prevalence and magnitude of anemia at hospital discharge and determine whether anemia at discharge was associated with 30-day readmissions among a cohort of hospitalizations in a single health care system. From January 1, 2009, to August 31, 2011, there were 152,757 eligible hospitalizations within a single health care system. The endpoint was any hospitalization within 30 days of discharge. The University HealthSystem Consortium's clinical database was used for demographics and comorbidities; hemoglobin values are from the hospitals' electronic medical records, and readmission status was obtained from the University HealthSystem Consortium administrative data systems. Mild anemia was defined as hemoglobin of greater than 11 to less than 12 g/dl in women and greater than 11 to less than 13 g/dl in men; moderate, greater than 9 to less than or equal to 11 g/dl; and severe, less than or equal to 9 g/dl. Logistic regression was used to assess the association of anemia and 30-day readmissions adjusted for demographics, comorbidity, and hospitalization type. Among 152,757 hospitalizations, 72% of patients were discharged with anemia: 31,903 (21%), mild; 52,971 (35%), moderate; and 25,522 (17%), severe. Discharge anemia was associated with severity-dependent increased odds for 30-day hospital readmission compared with those without anemia: for mild anemia, 1.74 (1.65-1.82); moderate anemia, 2.76 (2.64-2.89); and severe anemia, 3.47 (3.30-3.65), P < 0.001. Anemia at discharge is associated with a severity-dependent increased risk for 30-day readmission. A strategy focusing on anemia treatment care paths during index hospitalization offers an opportunity to influence subsequent readmissions.

  14. From Bad to Worse: Anemia on Admission and Hospital-Acquired Anemia.

    Science.gov (United States)

    Koch, Colleen G; Li, Liang; Sun, Zhiyuan; Hixson, Eric D; Tang, Anne S; Phillips, Shannon C; Blackstone, Eugene H; Henderson, J Michael

    2017-12-01

    Anemia at hospitalization is often treated as an accompaniment to an underlying illness, without active investigation, despite its association with morbidity. Development of hospital-acquired anemia (HAA) has also been associated with increased risk for poor outcomes. Together, they may further heighten morbidity risk from bad to worse. The aims of this study were to (1) examine mortality, length of stay, and total charges in patients with present-on-admission (POA) anemia and (2) determine whether these are exacerbated by development of HAA. In this cohort investigation, from January 1, 2009, to August 31, 2011, a total of 44,483 patients with POA anemia were admitted to a single health system compared with a reference group of 48,640 without POA anemia or HAA. Data sources included the University HealthSystem Consortium database and electronic medical records. Risk-adjustment methods included logistic and linear regression models for mortality, length of stay, and total charges. Present-on-admission anemia was defined by administrative coding. Hospital-acquired anemia was determined by changes in hemoglobin values from the electronic medical record. Approximately one-half of the patients experienced worsening of anemia with development of HAA. Risk for death and resource use increased with increasing severity of HAA. Those who developed severe HAA had 2-fold greater odds for death; that is, mild POA anemia with development of severe HAA resulted in greater mortality (odds ratio, 2.57; 95% confidence interval, 2.08-3.18; P < 0.001), increased length of stay (2.23; 2.16-2.31; P < 0.001), and higher charges (2.09; 2.03-2.15; P < 0.001). Present-on-admission anemia is associated with increased mortality and resource use. This risk is further increased from bad to worse when patients develop HAA. Efforts to address POA anemia and HAA deserve attention.

  15. The effects of cinacalcet treatment on bone mineral metabolism, anemia parameters, left ventricular mass index and parathyroid gland volume in hemodialysis patients with severe secondary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Dilek Torun

    2016-01-01

    Full Text Available The aim of this study was to investigate the effects of cinacalcet therapy on anemia parameters, bone mineral metabolism, left ventricular mass index (LVMI and parathyroid gland volume in hemodialysis (HD patients with secondary hyperparathyroidism. Twenty-five HD patients (M/F: 11/14, mean age: 45.2 ± 17.9 years, mean HD duration: 96.4 ± 32.7 months were included in this prospective pilot study. The indication to start calcimimetic therapy was persistent serum levels of parathyroid hormone (PTH >1000 pg/mL, refractory to intravenous (i.v. vitamin D and phosphate-binding therapy. The initial and one-year results of adjusted serum calcium (Ca +2 , phosphate (P, Ca × P product, PTH, hemoglobin (Hb and ferritin levels, transferrin saturation index (TSAT, median weekly erythropoietin (EPO dose, LVMI, and parathyroid volume by parathyroid ultrasonography were determined. There were no differences between pre- and post-treatment levels of serum Ca +2 (P = 0.853, P (P = 0.447, Ca × P product (P = 0.587, PTH (P = 0.273, ferritin (P = 0.153 and TSAT (P = 0.104. After 1 year of calcimimetic therapy, the Hb levels were significantly higher than the initial levels (P = 0.048. The weekly dose of EPO decreased with no statistical significance. The dose of cinacalcet was increased from 32.4 ± 12.0 to 60.0 ± 24.4 mg/day (P = 0.01. There were no differences between the pre- and post-treatment results regarding weekly vitamin D dose, parenteral iron dose, LVMI and parathyroid volume. The results of our study suggest that cinacalcet therapy might have an additional benefit in the control anemia in HD patients.

  16. ABO incompatibility hemolytic disease following exchange transfusion 96 newborn

    OpenAIRE

    Khatami S.F; Behjati SH.

    2007-01-01

    Background: ABO incompatibility hemolytic disease of the newborn is a common cause of clinical jaundice and causes two-thirds of the hemolytic disease in newborns. This study was undertaken to determine the frequency of ABO incompatibility hemolytic disease and its complications in newborns undergoing exchange transfusion.Methods: This prospective and descriptive study was performed in jaundiced newborn infants during a three-year period. Inclusion criteria were: maternal blood type O, newbor...

  17. Protrusio acetabuli in sickle-cell anemia

    International Nuclear Information System (INIS)

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

    1984-01-01

    Of 155 adults with sickle-cell anemia (SS, SC), radiographs of the pelvis or hip demonstrated protrusio acetabuli on at least one side in 14 (3 men and 11 women), as indicated by projection of the acetabular line medial to the ilio-ischial line. All 14 patients had bone changes attributable to sickle-cell anemia, including marrow hyperplasia and osteonecrosis; however, the severity of femoral or acetabular osteonecrosis did not appear directly related to the protrusion. The authors conclude that sickle-cell anemia can predispose to development of protrusio acetabuli

  18. Anemia in Chronic Kidney Disease

    Science.gov (United States)

    ... artérielle Heart Disease Mineral & Bone Disorder Anemia in Chronic Kidney Disease What is anemia? Anemia is a condition in ... as they should. How is anemia related to chronic kidney disease? Anemia commonly occurs in people with chronic kidney ...

  19. Anemia in Chronic Kidney Disease

    Science.gov (United States)

    ... Cysts Solitary Kidney Your Kidneys & How They Work Anemia in Chronic Kidney Disease What is anemia? Anemia is a condition in which the body ... function as well as they should. How is anemia related to chronic kidney disease? Anemia commonly occurs ...

  20. Susceptibility to β-lactams in β-hemolytic streptococci.

    Science.gov (United States)

    Bonofiglio, Laura; Gagetti, Paula; García Gabarrot, Gabriela; Kaufman, Sara; Mollerach, Marta; Toresani, Inés; Vigliarolo, Laura; von Specht, Martha; Lopardo, Horacio A

    2018-03-13

    Group A (GAS), B (GBS), C (GCS) and G (GGS) β-hemolytic streptococci are important human pathogens. They cause infections of different severity and frequency. Nowadays, after 70 years of use, penicillin is still universally active against GAS, GCS and GGS. However, therapeutic failures have been recorded in 2-28% of pharyngitis cases (median: 12%) attributable to different causes. By contrast, some GBS with reduced susceptibility to penicillin have been described, especially in Japan. In this group of bacteria, it is important to highlight that confirmation by reference methods is mandatory when decreased susceptibility to penicillin is suspected as well as checked for the detection of the mechanisms involved. Copyright © 2017 Asociación Argentina de Microbiología. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. Atypical hemolytic uremic syndrome: Laboratory characteristics, complement-amplifying conditions, renal biopsy, and genetic mutations

    Directory of Open Access Journals (Sweden)

    Mohammad A Hossain

    2018-01-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS. First, because of the lack of a single specific laboratory test for aHUS, other forms of thrombotic microangiopathies such as thrombotic thrombocytopenic purpura and Shiga toxin-associated HUS must be excluded to successfully establish the diagnosis of aHUS. Second, application of the knowledge of complement-amplifying conditions is critically important in making an accurate diagnosis. Third, when available, a renal biopsy can reveal changes consistent with thrombotic microangiopathy. Fourth, genetic mutations are increasingly clarifying the underlying complement dysfunction and gaining importance in the diagnosis and management of patients with aHUS. This review concentrates on the four aspects of aHUS and calls for heightened awareness in making an accurate diagnosis of aHUS.

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

    Directory of Open Access Journals (Sweden)

    Fengxiao Bu

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Han-Mou Tsai

    2014-01-01

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

  4. Delayed hemolytic transfusion reaction/hyperhemolysis syndrome in children with sickle cell disease.

    Science.gov (United States)

    Talano, Julie-An M; Hillery, Cheryl A; Gottschall, Jerome L; Baylerian, Diane M; Scott, J Paul

    2003-06-01

    Alloimmunization in patients with sickle cell disease (SCD) has a reported incidence of 5% to 36%. One complication of alloimmunization is delayed hemolytic transfusion reaction/hyperhemolysis (DHTR/H) syndrome, which has a reported incidence of 11%. In patients with SCD, clinical findings in DHTR/H syndrome occur approximately 1 week after the red blood cell (RBC) transfusion and include the onset of increased hemolysis associated with pain and profound anemia. The hemoglobin (Hb) often drops below pretransfusion levels. In many reported adult cases, the direct antiglobulin test (DAT) remains negative and no new alloantibody is detected as the cause for these transfusion reactions. To date, few pediatric cases have been reported with this phenomenon. The objective of this study was to describe the clinical and laboratory findings of a case series in children who had SCD and experienced a DHTR/H syndrome at our institution. An 11-year retrospective chart review of patients with discharge diagnosis of SCD and transfusion reaction was performed. DHTR/H syndrome was defined as the abrupt onset of signs and symptoms of accelerated hemolysis evidenced by an unexplained fall in Hb, elevated lactic dehydrogenase, elevated bilirubin above baseline, and hemoglobinuria, all occurring between 4 and 10 days after an RBC transfusion. Patient characteristics, time from transfusion, symptoms, reported DAT, new autoantibody or alloantibody formation, laboratory abnormalities, and complications were recorded. Patients with acute transfusion reactions were excluded. We encountered 7 patients who developed 9 episodes of DHTR/H syndrome occurring 6 to 10 days after RBC transfusion. Each presented with fever and hemoglobinuria. All but 1 patient experienced pain initially ascribed to vaso-occlusive crisis. The DAT was positive in only 2 of the 9 episodes. The presenting Hb was lower than pretransfusion levels in 8 of the 9 events. Severe complications were observed after the onset of

  5. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... grams per deciliter (g/dl) for men and less than 12 g/dl for women is diagnostic of anemia. In iron-deficiency anemia, ... blood levels of iron will be low, or less than 10 micromoles per liter (mmol/L) for both men and women. Normal levels are 10 to 30 mmol/L. ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... loss and lead to iron-deficiency anemia. Common causes of blood loss that lead to iron-deficiency anemia include: Bleeding in your GI tract, from an ulcer, colon cancer, or regular use of medicines such as aspirin ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron supplements work best to treat iron-deficiency anemia in children who do not consume the daily recommended amount ... and Clinical Studies to hear experts, parents, and children talk about their experiences with clinical ... Anemia Arrhythmia Blood Donation Blood Tests Blood ...

  8. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron-deficiency anemia. Search the NIH Research Portfolio Online Reporting Tools (RePORT) to learn about research that ... iron-deficiency anemia in blood donors affects the quality of donated red blood cells, such as how ...

  9. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... how having iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in premature or very small newborns . In collaboration with the Eunice Kennedy Shriver National Institute of Child Health and Human Development, we are investigating how ...

  10. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... blocks the intestine from taking up iron. Other medical conditions Other medical conditions that may lead to iron-deficiency anemia ... daily amount of iron. If you have other medical conditions that cause iron-deficiency anemia , such as ...

  11. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... if you are diagnosed with iron-deficiency anemia. Risk Factors You may have an increased risk for iron-deficiency anemia because of your age, ... or sex. Age You may be at increased risk for iron deficiency at certain ages: Infants between ...

  12. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Are you curious about how inflammation from chronic diseases can cause iron-deficiency anemia? Read more When there is ... DBDR) is a leader in research on the causes, prevention, and treatment of blood diseases, including iron-deficiency anemia. Search the NIH Research ...

  13. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... stores are developed during the third trimester of pregnancy. Children between ages 1 and 2, especially if they drink a lot ... Resources NHLBI resources Your Guide to Anemia [PDF, ... (National Institute of Diabetes and Digestive and Kidney Diseases) Avoiding Anemia (National ...

  14. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... anemia, including: Vegetarian or vegan eating patterns. Not eating enough iron-rich foods, such as meat and fish, may result in ... deficiency anemia, your doctor may recommend heart-healthy eating and choosing iron-rich foods, especially during certain stages of life when more ...

  15. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in premature or very small newborns . In collaboration with the Eunice Kennedy Shriver National Institute of Child Health and Human Development, we are investigating how best to treat ...

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... for iron-deficiency anemia. Lifestyle habits Certain lifestyle habits may increase your risk for iron-deficiency anemia, including: Vegetarian or vegan eating patterns. Not eating enough iron-rich foods, such as meat and fish, may result in ...

  17. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... other conditions that can cause iron-deficiency anemia. Blood tests to screen for iron-deficiency anemia To screen ... check the size of your liver and spleen. Blood tests Based on results from blood tests to screen ...

  18. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... also are hoping to determine which iron supplements work best to treat iron-deficiency anemia in children who do not consume the daily recommended amount of iron. Read less Participate in NHLBI Clinical Trials We lead or sponsor many studies related to iron-deficiency anemia. See if you ...

  19. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... iron-deficiency anemia early in life affects later behavior, thinking, and mood during adolescence. Treating anemia in premature or very small newborns . In collaboration with the Eunice Kennedy Shriver National Institute of Child Health and Human Development, we are investigating how ...

  20. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Precision Medicine Activities Obesity, Nutrition, and Physical Activity Population and Epidemiology Studies Women’s Health All Science A- ... to help your body absorb iron. Avoid drinking black tea, which reduces iron ... was associated with a greater risk of death even with mild anemia. Now, anemia in older ...

  1. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... anemia. Return to Signs, Symptoms, and Complications to review signs and symptoms as well as complications from iron-deficiency ... NIH]) Heavy Menstrual Bleeding (Centers for Disease Control and ... Dietary Supplement Fact Sheet (NIH) Iron-Deficiency Anemia (National Library ...

  2. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... be at risk for iron-deficiency anemia. Lifestyle habits Certain lifestyle habits may increase your risk for iron-deficiency anemia, ... you are experiencing side effects such as a bad metallic taste, vomiting, diarrhea, constipation, or upset stomach. ...

  3. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... over 65 years of age had low hemoglobin levels. This was associated with a greater risk of death even with mild anemia. Now, anemia in older adults is recognized as an important condition. NHLBI Small Business Program. Through the NHLBI Small Business Program , we ...

  4. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... even with mild anemia. Now, anemia in older adults is recognized as an important condition. NHLBI Small Business Program. Through the NHLBI Small Business Program , we fund research and development for domestic small businesses that have strong potential ...

  5. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... how we are using current research and advancing research to prevent iron-deficiency anemia. Participate in NHLBI Clinical Trials will explain our ongoing clinical studies that are investigating prevention strategies for iron-deficiency anemia. Signs, Symptoms, and Complications ...

  6. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  7. Predictors of anemia in women of reproductive age: Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project.

    Science.gov (United States)

    Wirth, James P; Woodruff, Bradley A; Engle-Stone, Reina; Namaste, Sorrel Ml; Temple, Victor J; Petry, Nicolai; Macdonald, Barbara; Suchdev, Parminder S; Rohner, Fabian; Aaron, Grant J

    2017-07-01

    Background: Anemia in women of reproductive age (WRA) (age range: 15-49 y) remains a public health problem globally, and reducing anemia in women by 50% by 2025 is a goal of the World Health Assembly. Objective: We assessed the associations between anemia and multiple proximal risk factors (e.g., iron and vitamin A deficiencies, inflammation, malaria, and body mass index) and distal risk factors (e.g., education status, household sanitation and hygiene, and urban or rural residence) in nonpregnant WRA. Design: Cross-sectional, nationally representative data from 10 surveys ( n = 27,018) from the Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project were analyzed individually and pooled by the infection burden and risk in the country. We examined the severity of anemia and measured the bivariate associations between anemia and factors at the country level and by infection burden, which we classified with the use of the national prevalences of malaria, HIV, schistosomiasis, sanitation, and water-quality indicators. Pooled multivariate logistic regression models were constructed for each infection-burden category to identify independent determinants of anemia (hemoglobin concertation <120 g/L). Results: Anemia prevalence was ∼40% in countries with a high infection burden and 12% and 7% in countries with moderate and low infection burdens, respectively. Iron deficiency was consistently associated with anemia in multivariate models, but the proportion of anemic women who were iron deficient was considerably lower in the high-infection group (35%) than in the moderate- and low-infection groups (65% and 71%, respectively). In the multivariate analysis, inflammation, vitamin A insufficiency, socioeconomic status, and age were also significantly associated with anemia, but malaria and vitamin B-12 and folate deficiencies were not. Conclusions: The contribution of iron deficiency to anemia varies according to a country's infection

  8. Predictors of anemia in women of reproductive age: Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project

    Science.gov (United States)

    Woodruff, Bradley A; Petry, Nicolai; Macdonald, Barbara; Aaron, Grant J

    2017-01-01

    Background: Anemia in women of reproductive age (WRA) (age range: 15–49 y) remains a public health problem globally, and reducing anemia in women by 50% by 2025 is a goal of the World Health Assembly. Objective: We assessed the associations between anemia and multiple proximal risk factors (e.g., iron and vitamin A deficiencies, inflammation, malaria, and body mass index) and distal risk factors (e.g., education status, household sanitation and hygiene, and urban or rural residence) in nonpregnant WRA. Design: Cross-sectional, nationally representative data from 10 surveys (n = 27,018) from the Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia (BRINDA) project were analyzed individually and pooled by the infection burden and risk in the country. We examined the severity of anemia and measured the bivariate associations between anemia and factors at the country level and by infection burden, which we classified with the use of the national prevalences of malaria, HIV, schistosomiasis, sanitation, and water-quality indicators. Pooled multivariate logistic regression models were constructed for each infection-burden category to identify independent determinants of anemia (hemoglobin concertation Anemia prevalence was ∼40% in countries with a high infection burden and 12% and 7% in countries with moderate and low infection burdens, respectively. Iron deficiency was consistently associated with anemia in multivariate models, but the proportion of anemic women who were iron deficient was considerably lower in the high-infection group (35%) than in the moderate- and low-infection groups (65% and 71%, respectively). In the multivariate analysis, inflammation, vitamin A insufficiency, socioeconomic status, and age were also significantly associated with anemia, but malaria and vitamin B-12 and folate deficiencies were not. Conclusions: The contribution of iron deficiency to anemia varies according to a country’s infection burden. Anemia

  9. Prevalence of Anemia in Latin America and the Caribbean.

    Science.gov (United States)

    Mujica-Coopman, María F; Brito, Alex; López de Romaña, Daniel; Ríos-Castillo, Israel; Coris, Héctor; Olivares, Manuel

    2015-06-01

    In Latin America and the Caribbean, anemia has been a public health problem that affects mainly women of childbearing age and children under 6 years of age. However, the current prevalence of anemia in this region is unknown. To examine the latest available prevalence data on anemia in Latin America and the Caribbean. A systematic review was conducted in 2011 and updated in 2014. Studies determining the prevalence of anemia conducted in apparently healthy populations with national or regional representativeness were included in the review. The lowest prevalence rates of anemia among children under 6 years of age were found in Chile (4.0%), Costa Rica (4.0%), Argentina (7.6%), and Mexico (19.9%). In Nicaragua, Brazil, Ecuador, El Panama, and Honduras, anemia was a moderate public health problem, with prevalence ranging Salvador, Cuba, Colombia, the Dominican Republic, Peru, from 20.1% to 37.3%. Anemia was a severe public health problem in Guatemala, Haiti, and Bolivia. The prevalence of anemia among women of childbearing age was lowest in Chile (5.1%). In Colombia, El Salvador, Costa Rica, Nicaragua, Ecuador, Mexico, Peru, Honduras, and Argentina, anemia was a mild public health problem, with prevalence ranging from 7.6% to 18.7%. In Guatemala, Brazil, the Dominican Republic, and Bolivia, anemia was a moderate public health problem, with prevalence ranging from 21.4% to 38.3%. Panama and Haiti had the highest reported prevalence rates (40.0% and 45.5%, respectively), and anemia was considered a severe public health problem in those countries. Anemia remains a public health problem in children under 6 years of age and women of childbearing age in most Latin America and Caribbean countries for which data are available.

  10. Successful medical management of a neonate with spontaneous splenic rupture and severe hemophilia A.

    Science.gov (United States)

    Badawy, Sherif M; Rossoff, Jenna; Yallapragada, Sushmita; Liem, Robert I; Sharathkumar, Anjali A

    2017-03-01

    Splenic rupture in neonates is a rare event, usually occurring in the setting of underlying predisposing conditions. Here, we present the case of a term neonate who presented with worsening anemia in the setting of known hemolytic disease during the newborn period and was later found to have a spontaneous splenic rupture. He was subsequently diagnosed with severe hemophilia A, and was managed medically with recombinant factor VIII replacement therapy without any surgical intervention. This is the first reported case of a neonate who had spontaneous splenic rupture and severe hemophilia A, and underwent successful medical treatment without any surgical intervention. Copyright © 2016 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.

  11. Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia

    International Nuclear Information System (INIS)

    Schuster, F R; Hubner, B; Führer, M; Eckermann, O; Gombert, M; Dornmair, K; Binder, V; Reuther, S; Krell, P; Keller, T; Borkhardt, A

    2011-01-01

    One of the major obstacles of immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA) comes from the often months-long unpredictability of bone-marrow (BM) recovery. In this prospective study in children with newly diagnosed very severe AA (n=10), who were enrolled in the therapy study SAA-BFM 94, we found a dramatically reduced diversity of both CD4+ and CD8+ BM cells, as scored by comprehensive V-beta chain T-cell receptor (TCR) analysis. Strongly skewed TCR V-beta pattern was highly predictive for good or at least partial treatment response (n=6, CD8+ complexity scoring median 35.5, range 24–73). In contrast, IST in patients with rather moderate reduction of TCR V-beta diversity (n=4, CD8+ complexity scoring median 109.5, range 82–124) always failed (P=0.0095). If confirmed in a larger series of patients, TCR V-beta repertoire in BM may help to assign children with SAA up-front either to IST or to allogeneic stem-cell transplantation

  12. Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Schuster, F R; Hubner, B [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Führer, M [Department of Pediatric Oncology and Hematology, Dr von Haunersches Children' s Hospital, University of Munich, Munich (Germany); Eckermann, O; Gombert, M [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Dornmair, K [Department for Clinical Neuroimmunology, University of Munich, Munich (Germany); Binder, V; Reuther, S; Krell, P [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Keller, T [Acomed, statistical analysis GmbH, Leipzig (Germany); Borkhardt, A [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany)

    2011-03-01

    One of the major obstacles of immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA) comes from the often months-long unpredictability of bone-marrow (BM) recovery. In this prospective study in children with newly diagnosed very severe AA (n=10), who were enrolled in the therapy study SAA-BFM 94, we found a dramatically reduced diversity of both CD4+ and CD8+ BM cells, as scored by comprehensive V-beta chain T-cell receptor (TCR) analysis. Strongly skewed TCR V-beta pattern was highly predictive for good or at least partial treatment response (n=6, CD8+ complexity scoring median 35.5, range 24–73). In contrast, IST in patients with rather moderate reduction of TCR V-beta diversity (n=4, CD8+ complexity scoring median 109.5, range 82–124) always failed (P=0.0095). If confirmed in a larger series of patients, TCR V-beta repertoire in BM may help to assign children with SAA up-front either to IST or to allogeneic stem-cell transplantation.

  13. Antimicrobial, hemolytic and thrombolytic activities of some new N ...

    African Journals Online (AJOL)

    . Hemolytic and thrombolytic activities were determined by measuring absorbance before and after incubation of blood cells with test compound. Results: Compound 9d strongly inhibited Bacillus subtilis and Escherichia coli with zone of ...

  14. Hemolytic activity of Trichomonas vaginalis and Tritrichomonas foetus

    Directory of Open Access Journals (Sweden)

    Geraldo A De Carli

    1996-02-01

    Full Text Available The hemolytic activity of live isolates and clones of Trichomonas vaginalis and Tritrichomonas foetus was investigated. The isolates were tested against human erythrocytes. No hemolytic activity was detected by the isolates of T. foetus. Whereas the isolates of T. vaginalis lysed erythrocytes from all human blood groups. No hemolysin released by the parasites could be detected. Our preliminary results suggest that hemolysis depend on the susceptibility of red cell membranes to destabilization and the intervention of cell surface receptors as a mechanism of the hemolytic activity. The mechanism could be subject to strain-species-genera specific variation of trichomonads. The hemolytic activity of T. vaginalis is not due to a hemolysin or to a product of its metabolism. Pretreatment of trichomonads with concanavalin A reduced levels of hemolysis by 40%.

  15. Prevalence and Severity of Malaria Parasitemia among Children ...

    African Journals Online (AJOL)

    induced anemia is much more common in younger children and may require blood transfusion with high mortality rates.[2,3]. In Sub-Saharan Africa (SSA), children admitted with severe anemia are more likely to die than those without anemia.[4,5]. Blood transfusion in severe malarial anemia can be important in preventing ...

  16. Anemia caused by low iron -- infants and toddlers

    Science.gov (United States)

    ... tongue Have headaches or dizziness With more severe anemia, your child may have: Blue-tinged or pale whites of ... and prevention of iron deficiency and iron-deficiency anemia in infants and young children (0-3 years of age). Pediatrics. 2010;126( ...

  17. ORIGINAL ARTICLE Anemia among HIV Infected Individuals Taking ...

    African Journals Online (AJOL)

    2018-01-01

    Jan 1, 2018 ... reactions (18). The magnitude and characteristics of ZDV associated anemia among Ethiopian patients has not been well documented. Thus, the aim of this study was to provide a comparative information about the magnitude, severity and characteristics of anemia among HIV/AIDS patients initiated with.

  18. Iron-Deficiency Anemia

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    Full Text Available ... blood loss during dialysis. People who have chronic kidney disease also often take other medicines—such as proton ... anemia or who have chronic conditions such as kidney disease or celiac disease may be more likely to ...

  19. Iron-Deficiency Anemia

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    Full Text Available ... fatigue or tiredness, shortness of breath, or chest pain. If your doctor diagnoses you with iron-deficiency ... Common symptoms of iron-deficiency anemia include: Chest pain Coldness in the hands and feet Difficulty concentrating ...

  20. Iron-Deficiency Anemia

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    Full Text Available ... body to absorb iron from the gastrointestinal tract (GI tract). Blood loss When you lose blood, you ... to iron-deficiency anemia include: Bleeding in your GI tract, from an ulcer, colon cancer, or regular ...

  1. Iron-Deficiency Anemia

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  2. Iron-Deficiency Anemia

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  5. Iron-Deficiency Anemia

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    Full Text Available ... your blood, such as vitamin B12 or folic acid. Visit our Pernicious Anemia Health Topic to learn ... recommend options such as taking your supplements with food, lowering the dose, trying a different type of ...

  6. Iron-Deficiency Anemia

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  9. Iron-Deficiency Anemia

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  10. Iron-Deficiency Anemia

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  11. Iron-Deficiency Anemia

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    Full Text Available ... red blood cells, called hemolysis . Hemolysis, in this case, is caused by strong muscle contractions and the ... to prevent iron-deficiency anemia. Participate in NHLBI Clinical Trials will explain our ongoing clinical studies that ...

  12. Iron-Deficiency Anemia

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    Full Text Available ... and dark green leafy vegetables. Foods rich in vitamin C, such as oranges, strawberries, and tomatoes, may ... of other nutrients in your blood, such as vitamin B12 or folic acid. Visit our Pernicious Anemia ...

  13. Iron-Deficiency Anemia

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  14. Iron-Deficiency Anemia

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    Full Text Available ... counts, hemoglobin or hematocrit levels, or mean corpuscular volume (MCV) that would suggest anemia. Different tests help ... complete blood count measures hemoglobin and mean corpuscular volume (MCV). Hemoglobin of less than 13 grams per ...

  15. Iron-Deficiency Anemia

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    Full Text Available ... interferes with the body’s ability to make hemoglobin. Family history and genetics Von Willebrand disease is an ... deficiency anemia. Return to Risk Factors to review family history, lifestyle, unhealthy environments, or other factors that ...

  16. Iron-Deficiency Anemia

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    Full Text Available ... from developing iron-deficiency anemia. Foods that are good sources of iron include dried beans, dried fruits, ... iron is needed, such as childhood and pregnancy. Good sources of iron are meat, poultry, fish, and ...

  17. Iron-Deficiency Anemia

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    Full Text Available ... A-Z Clinical Trials Publications and Resources Health Education and Awareness The Science Science Home Blood Disorders ... infancy has lasting effects. We are interested in learning how having iron-deficiency anemia early in life ...

  18. Iron-Deficiency Anemia

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    Full Text Available ... heart failure . Increased risk of infections Motor or cognitive development delays in children Pregnancy complications, such as ... iron-deficiency anemia may require intravenous (IV) iron therapy or a blood transfusion . Iron supplements Your doctor ...

  19. Iron-Deficiency Anemia

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    Full Text Available ... absorb iron and lead to iron-deficiency anemia. These conditions include: Intestinal and digestive conditions, such as ... tract. Inflammation from congestive heart failure or obesity . These chronic conditions can lead to inflammation that may ...

  20. Anemia of chronic disease

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    ... systemic lupus erythematosus , rheumatoid arthritis , and ulcerative colitis Cancer , including lymphoma and Hodgkin disease Long-term infections, such as bacterial endocarditis, osteomyelitis (bone infection), HIV/AIDS , lung abscess, hepatitis B or hepatitis C Symptoms Anemia of ...

  1. Iron-Deficiency Anemia

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  2. Iron-Deficiency Anemia

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    Full Text Available ... as a TMRPSS6 gene mutation that causes a person’s body to make too much of a hormone ... anemia from trauma, surgery, or heavy menstrual periods. Individuals with a gene for hemophilia, including symptomatic female ...

  3. Iron-Deficiency Anemia

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  4. Iron-Deficiency Anemia

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  5. Iron-Deficiency Anemia

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  6. Iron-Deficiency Anemia

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  9. Iron-Deficiency Anemia

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  10. Iron-Deficiency Anemia

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  12. Iron-Deficiency Anemia

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    Full Text Available ... lead in their blood from their environment or water. Lead interferes with the body’s ability to make ... iron-deficiency anemia in blood donors affects the quality of donated red blood cells, such as how ...

  13. Iron-Deficiency Anemia

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    Full Text Available ... also often take other medicines—such as proton pump inhibitors, anticoagulants, or blood thinners—that may cause iron-deficiency anemia. Proton pump inhibitors interfere with iron absorption, and blood thinners ...

  14. Iron-Deficiency Anemia

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    Full Text Available ... Look for Treatment will discuss medicines and eating pattern changes that your doctors may recommend if you ... iron-deficiency anemia, including: Vegetarian or vegan eating patterns. Not eating enough iron-rich foods, such as ...

  15. Iron-Deficiency Anemia

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  18. Iron-Deficiency Anemia

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    Full Text Available ... screen for iron-deficiency anemia, your doctor may order a blood test called a complete blood count ( ... your risk factors , do a physical exam, or order blood tests or other diagnostic tests. Physical exam ...

  19. Iron-Deficiency Anemia

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    Full Text Available ... Learn more about participating in a clinical trial . View all trials from ClinicalTrials.gov . Visit Children and Clinical ... Resources NHLBI resources Your Guide to Anemia [PDF, 1. ...

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    Full Text Available ... risk for iron-deficiency anemia, including: Vegetarian or vegan eating patterns. Not eating enough iron-rich foods, ... iron-fortified foods that have iron added. Vegetarian diets can provide enough iron if you choose nonmeat ...