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Pure Red Cell Aplasia (pure + red_cell_aplasia)

Distribution by Scientific Domains

Medical Sciences	100%

Distribution within Medical Sciences

Hematology	57%
Transplantation	23%

Selected Abstracts

Erythropoietin-induced, antibody-mediated pure red cell aplasia,

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 2005
The Pure Red Cell Aplasia Global Scientific Advisory Board (GSAB)
Abstract Pure red cell aplasia (PRCA) is a rare haematological condition that is characterized by severe aregenerative anaemia due to an almost complete cessation of red blood cell production. While antibody-mediated PRCA was extremely rare before 1998, the incidence of this disorder increased sharply after 1998 in patients receiving subcutaneous epoetin alfa produced by Ortho-Biotech and marketed outside the USA. The diagnosis of antibody-mediated PRCA relies mostly on the results of bone marrow biopsy or aspirate, which shows an absence of erythroid precursors and/or red cell maturation arrest while counts of white cell and platelet precursors are normal, and on the identification of circulating anti-erythropoietin antibodies. Retrospective analysis of PRCA cases has shown that immunosuppressive therapy can induce a disappearance of anti-erythropoietin antibodies in most patients. [source]

Acquired pure red cell aplasia associated with malignant lymphomas: A nationwide cohort study in Japan for the PRCA Collaborative Study Group

AMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2009
Makoto Hirokawa
Pure red cell aplasia (PRCA) has been reported in association with lymphoma as one of the autoimmune diseases seen during the course of lymphoid malignancies. However, the relation of PRCA with the underlying lymphomas remains unclear. The aim of this study was to clarify the histologic subtypes of lymphomas, the chronological sequence of anemia and lymphoma, and the response to treatment. We conducted a nationwide survey in Japan. From a cohort of 185 PRCA patients, 8 patients with lymphoma were evaluated. Histologic subtypes varied and the lymphoma was of the B-cell type in four cases and of the T-cell type in four. Four patients simultaneously developed PRCA and lymphoma. Three patients developed PRCA following lymphoma, two of whom developed anemia during remission of lymphoma. PRCA preceded lymphoma in one patient. Effective chemotherapy was associated with remission of anemia in concurrent lymphoma and PRCA. Overall, anemia responded to chemotherapy and/or immunosuppressive therapy in seven patients. In four responding patients, PRCA remained in durable remission without maintenance immunosuppressive therapy, which is different from a recurrent feature of idiopathic PRCA. We suggest that the mechanism of lymphoma-associated PRCA is heterogeneous and that durable maintenance-free remission of anemia can be obtained in some patients. Am. J. Hematol., 2009. © 2008 Wiley-Liss, Inc. [source]

Anemia in children after transplantation: etiology and the effect of immunosuppressive therapy on erythropoiesis

PEDIATRIC TRANSPLANTATION, Issue 4 2003
Amira Al-Uzri
Abstract: Anemia in children after renal transplantation is more common than previously appreciated. Multiple factors appear to play roles in the development of post-transplant anemia, the most common of which is absolute and/or functional iron deficiency anemia. Most experts recommend that iron limited anemias in transplant patients should be diagnosed using the same criteria as for chronic renal failure patients. Serum erythropoietin (EPO) levels are expected to normalize after a successful renal transplantation with a normal kidney function, yet both EPO deficiency and resistance have been reported. While no large controlled trials comparing the effect of different immunosuppressive agents on erythropoiesis after transplantation have been performed, generalized bone marrow suppression attributable to azathioprine (AZA), mycophenolate mofetil (MMF), tacrolimus, antithymocyte preparations has been reported. Pure red cell aplasia (PRCA) occurs rarely after transplantation and is characterized by the selective suppression of erythroid cells in the bone marrow. PRCA has been reported with the use of AZA, MMF, tacrolimus, angiotensin converting enzyme inhibitors (ACEI), but not with cyclosporine (CSA) use. Post-transplant hemolytic uremic syndrome has been reported with orthoclone anti T-cell antibody (OKT3), CSA and tacrolimus therapy. Viral infections including cytomegalovirus, Epstein,Barr virus and human parvovirus B19 have been reported to cause generalized marrow suppression. Management of severe anemia associated with immunosuppressive drugs generally requires lowering the dose, drug substitution or, when possible, discontinuation of the drug. Because this topic has been incompletely studied, our recommendation as to the best immunosuppressive protocol after renal transplantation remains largely dependent on the clinical response of the individual patient. [source]

Pure red cell aplasia in patients with refractory anaemia treated with two different recombinant erythropoietins

BRITISH JOURNAL OF HAEMATOLOGY, Issue 6 2004
Laurent Quint
No abstract is available for this article. [source]

Changes of isoagglutinin titres after ABO-incompatible allogeneic stem cell transplantation

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2003
Je-Hwan Lee
Summary. We investigated the changes in isoagglutinin titres in 62 patients who underwent ABO-incompatible allogeneic stem cell transplantation. After major [and/or (±) minor] ABO-incompatible transplantation, recipient-derived isoagglutinins against donor-type red blood cells (RBCs) disappeared more rapidly in unrelated recipients (P = 0·006) and in patients with acute graft-versus-host disease (GVHD, P = 0·025) than in sibling recipients and in patients without acute GVHD respectively. Pure red cell aplasia (PRCA) developed in 10 out of 35 evaluable patients who underwent major (± minor) ABO-incompatible transplantation, and the post-transplant increase of isoagglutinin titres was a significant predictor for the occurrence of PRCA. In five out of 36 patients who underwent minor (and/or (±) major) ABO-incompatible transplantation, donor-derived isoagglutinins against recipient RBCs were detectable without clinically overt haemolysis. Isoagglutinin titres against ABO antigens absent both on recipient and donor RBCs decreased during the early post-transplant period then rose subsequently in 24 out of 29 patients at (median) d 59 post transplant. Our study showed that changes in isoagglutinin titres might have clinical implications in the occurrence of immunohaematological complications such as PRCA or immune-mediated haemolysis, and might reflect immunohaematological reconstitution after transplantation. Furthermore, our data regarding time to disappearance of recipient-derived isoagglutinins against donor-type RBCs after major ABO-incompatible transplantation suggest the presence of a graft-versus-plasma cell effect. [source]

Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature

CLINICAL TRANSPLANTATION, Issue 6 2000
Duvuru Geetha
Human Parvovirus B19 (PV B19) is one of the several recently described ,emerging viruses' and has been identified as the etiological agent of ,fifth disease' in childhood. Human PV B19, which is the etiological agent of transient erythroblastopenia in hemolytic anemia, is also a recognized rare cause of red cell aplasia in immunocompromised patients, including transplant recipients. To date, 26 cases of PV B19-induced red cell aplasia have been reported in solid organ transplant recipients. Twelve patients had cyclosporine-based immunosuppression and 14 had tacrolimus-based immunosuppression. Sixteen of these patients required treatment with commercial intravenous immunoglobulin alone, 1 required treatment with intravenous immunoglobulin and plasmapheresis, 4 required intravenous immunoglobulin and erythropoietin, 1 required treatment with intravenous immunoglobulin and conversion of tacrolimus to cyclosporine, 1 had improvement in hematocrit with erythropoietin alone and in 3 patients the disease was self-limiting. Herein, we report a case of pure red cell aplasia caused by acute PV B19 infection in a renal transplant recipient in whom the immunosuppressive regimen included prednisone, mycophenolate mofetil and tacrolimus and the red cell aplasia resolved with discontinuation of mycophenolate mofetil. [source]

Erythropoietin-induced, antibody-mediated pure red cell aplasia,

A Retrospective Study of the Incidence and the Classification of Bone Marrow Disorders in the Dog at a Veterinary Teaching Hospital (1996,2004)

JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 4 2006
Douglas J. Weiss
Background: An 8-year retrospective study was conducted to evaluate the prevalence and the classification of canine bone marrow disorders in a clinical pathology service at a university referral hospital. Animals: Dogs evaluated for bone marrow disorders at a veterinary teaching hospital. Hypothesis: A better understanding of the spectrum and the prevalence of canine bone marrow disorders can be achieved with a multiyear retrospective study. Methods: Bone marrow aspirate smears, core biopsy specimens, and case records from 717 dogs were reviewed. Results: Bone marrow specimens were first categorized based on the presence or the absence of a primary bone marrow disorder. Nondysplastic and nonmalignant pathologic changes were placed into 14 subcategories. Frequently observed pathologic disorders included nonregenerative immune-mediated anemia, pure red cell aplasia, bone marrow necrosis, myelofibrosis, and hemophagocytic syndrome. Dysmyelopoiesis (n = 61) was subcategorized into myelodysplastic syndromes (n = 27), and congenital (n = 1) and secondary (n = 33) dysmyelopoiesis. One hundred twenty-six cases of neoplasia were divided into acute leukemia (n = 46), chronic leukemia (n = 7), stage 5 malignant lymphoma (n = 28), multiple myeloma (n = 25), malignant histiocytosis (n = 11), metastatic mast-cell tumor (n = 3), sarcoma (n = 5), and carcinoma (n = 1). Conclusions and Clinical Importance: This study provides a general indication of the spectrum and the prevalence of canine bone marrow disorders at a referral center in North America. [source]

Anti-erythropoietin antibody,mediated pure red cell aplasia in a living donor liver transplant recipient treated for hepatitis C virus

LIVER TRANSPLANTATION, Issue 11 2007
Jordan M. Schecter
After liver transplantation, reinfection of the newly engrafted liver with hepatitis C virus is essentially universal in patients who are viremic at the time of transplantation. Treatment with interferon preparations with or without ribavirin is recommended in patients with marked histologic injury; however, hematologic toxicity associated with therapy has been reported, which is usually treated with growth factor support, including erythropoietin analogues. We present the first reported case of anti-erythropoietin antibody,mediated pure red cell aplasia arising in the setting of hepatitis C virus therapy in a patient who underwent living donor liver transplantation. Liver Transpt 13: 1589,1592. 2007. © 2007 AASLD. [source]

Hypersensitivity reactions to the polysorbate contained in recombinant erythropoietin and darbepoietin (Case Report)

NEPHROLOGY, Issue 3 2005
RICHARD H STEELE
SUMMARY: The following case reports are of two patients who have developed hypersensitivity reactions to the red cell growth hormones, darbepoietin and erythropoietin. The subsequent skin testing and clinical course suggested that the cause of these reactions was due to the excipient polysorbate 80. This finding might have implications in the recent increase in the incidence of pure red cell aplasia. [source]

ZAP-70 expression is associated with increased risk of autoimmune cytopenias in CLL patients,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2010
Roberta Zanotti
Autoimmune cytopenias (AIC) are frequent in chronic lymphocytic leukemia (CLL) patients, but risk factors and prognostic relevance of these events are controversial. Data about the influence on AIC of biological prognostic markers, as ZAP-70, are scanty. We retrospectively evaluated AIC in 290 CLL patients tested for ZAP-70 expression by immunohistochemistry on bone marrow biopsy at presentation. They were 185 men, median age 63 years, 77.9% Binet stage A, 17.6% B and 4.5% C. AIC occurred in 46 patients (16%): 31 autoimmune hemolytic anemias, 10 autoimmune thrombocytopenias, four Evans syndromes, and one pure red cell aplasia. Of the 46 cases of AIC, 37 (80%) occurred in ZAP-70 positive patients and nine (20%) in ZAP-70 negatives. ZAP-70 expression [Hazard Ratio (HR) = 7.42; 95% confidence interval (CI): 2.49,22.05] and age >65 years (HR = 5.41; 95% CI: 1.67,17.49) resulted independent risk factors for AIC. Among the 136 patients evaluated both for ZAP-70 expression and IGHV status, the occurrence of AIC was higher in ZAP-70 positive/IGHV unmutated cases (35%) than in patients ZAP-70 negative/IGHV mutated (6%) or discordant for the two parameters (4%; P < 0.0001). In ZAP-70 positive patients, occurrence of AIC negatively influenced survival (HR = 1.75; 95% CI: 1.06,2.86). The high risk of developing AIC in ZAP-70 positive CLL, particularly when IGHV unmutated, should be considered in the clinical management. Am. J. Hematol. 2010. © 2010 Wiley-Liss, Inc. [source]

Acquired pure red cell aplasia associated with malignant lymphomas: A nationwide cohort study in Japan for the PRCA Collaborative Study Group

Combined pure red cell aplasia and autoimmune hemolytic anemia in systemic lupus erythematosus with anti-erythropoietin autoantibodies,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2008
Akinori Hara
A 42-year-old woman with systemic lupus erythematosus was admitted to our hospital because of severe anemia. Her bone marrow was almost normocellular and erythroblasts were nearly absent. Laboratory data showed elevated levels of lactate dehydrogenase and positive findings on Coombs' tests. On the basis of these findings, her anemia was diagnosed as the overlap of pure red cell aplasia with autoimmune hemolytic anemia. Radioimmunoprecipitation assay revealed that her serum was positive for anti-erythropoietin antibodies before therapy. Furthermore, the autoantibodies inhibited proliferation of an erythropoietin-dependent cell line in a dose-dependent manner. Immunosuppressive treatment improved the anemia accompanied with disappearance of the autoantibodies. © 2008 Wiley-Liss, Inc. Am. J. Hematol., 2008. [source]

Refractory idiopathic pure red cell aplasia complicated by immune thrombocytopenia successfully treated with subcutaneous alemtuzumab

AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2008
Dat C. Pham
No abstract is available for this article. [source]

Successful treatment of pure red cell aplasia with autologous stem cell transplantation

AMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2007
Jae H. Park
We report a case of 64-year-old patient with pure red cell aplasia (PRCA) who was intolerant of conventional immunosuppressive therapies but achieved a complete long-term remission following autologous hematologic stem cell transplant (HSCT). The patient was initially treated with high-dose prednisone, cyclophosphamide, cyclosporine, antithymocyte globulin, and then rituximab. With the exception of rituximab, all of the above regimens achieved a transient response. However, because of the persistent requirement for red blood cell transfusions and intolerance to the multiple immunosuppressive therapies, autologous HSCT was eventually performed. The patient remains in complete remission and on no other therapy for 36 months following the autologous HSCT. Am. J. Hematol., 2007. © 2007 Wiley-Liss, Inc. [source]

Valproate-induced pure red cell aplasia and megakaryocyte dysplasia

BRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2008
Sandip Bartakke
No abstract is available for this article. [source]

The effect of treatment with Campath-1H in patients with autoimmune cytopenias

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2001
F. Willis
We describe 21 patients with severe and life-threatening autoimmune cytopenias resistant to standard immunosuppression who were treated with the monoclonal antibody Campath-1H. Four patients had autoimmune neutropenia, four had autoimmune haemolytic anaemia, four had pure red cell aplasia, one had immune thrombocytopenia purpura (ITP), three had autoimmune haemolytic anaemia and ITP (Evan's syndrome), three had autoimmune pancytopenia (ITP, autoimmune neutropenia and autoimmune haemolytic anaemia), one had ITP (associated with acquired Glanzmann's disease) and autoimmune neutropenia, and one had ITP and red cell aplasia. Campath-1H was administered at a dose of 10 mg/d as an intravenous infusion for 10 d. Responses were seen in 15 patients, which were sustained in six. Relapse occurred in eight patients after Campath-1H treatment. Patients entering the study later, received cyclosporine after Campath-1H in an attempt to reduce the incidence of relapse. Three patients received a second course of Campath-1H; all responded but later relapsed. Fourteen patients are alive at a median of 12 months (range 4,61) after Campath-1H. Campath-1H represents an alternative therapeutic option for severe, refractory autoimmune cytopenias. [source]