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Pure Red Cell Aplasia

  • Robert T. MeansJr.Email author
Chapter

Abstract

The term “pure red cell aplasia” (PRCA) describes normocytic normochromic anemia associated with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. PRCA is not a single disorder but includes congenital diseases caused by mutations in genes regulating ribosomal or mitochondrial proteins (Diamond-Blackfan anemia and Pearson syndrome), acquired myelodysplastic syndromes with the morphologic features of PRCA, as well as immune-mediated syndromes. Primary acquired PRCA, the best-known member of the class of PRCA disorders, is an autoimmune disorder, frequently antibody-mediated. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders; lymphoproliferative disorders; infections, particularly B19 parvovirus; thymoma and other solid tumors; or treatment with drugs. The therapeutic approach in most cases of primary or secondary acquired PRCA is immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. The single most effective immunosuppressive agent appears to be cyclosporine A, alone or with concurrent corticosteroids.

Keywords

Pure red cell aplasia Anemia Parvovirus Bone marrow morphology Thymoma Immunosuppression 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.James H. Quillen College of Medicine, East Tennessee State UniversityJohnson CityUSA

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