Pure Red Cell Aplasia

Overview

Journal Hematology Am Soc Hematol Educ Program

Specialty Hematology

Date 2016 Dec 4

PMID 27913462

Citations 26

Authors

Robert T Means Jr

Affiliations

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Abstract

Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.

Citing Articles

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Early bone marrow alterations in patients with adenosine deaminase 2 deficiency across disease phenotypes and severities.

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A Case of Erythropoietin (EPO)-Induced Pure Red Cell Aplasia and Its Treatment Efficacy With Desidustat.

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Unusual Presentation of B-Cell Chronic Lymphocytic Leukemia Accompanied by Pure Red Cell Aplasia: Case Report.

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