Pure red cell aplasia

Blood. 2016 Nov 24;128(21):2504-2509. doi: 10.1182/blood-2016-05-717140.


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.

Publication types

  • Review

MeSH terms

  • Anemia, Diamond-Blackfan* / immunology
  • Anemia, Diamond-Blackfan* / pathology
  • Anemia, Diamond-Blackfan* / therapy
  • Autoimmune Diseases* / immunology
  • Autoimmune Diseases* / pathology
  • Autoimmune Diseases* / therapy
  • Erythema Infectiosum / immunology
  • Erythema Infectiosum / pathology
  • Erythema Infectiosum / therapy
  • Humans
  • Leukemia, Large Granular Lymphocytic / immunology
  • Leukemia, Large Granular Lymphocytic / pathology
  • Leukemia, Large Granular Lymphocytic / therapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / immunology
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology
  • Leukemia, Lymphocytic, Chronic, B-Cell / therapy
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Erythematosus, Systemic / pathology
  • Lupus Erythematosus, Systemic / therapy
  • Myelodysplastic Syndromes / immunology
  • Myelodysplastic Syndromes / pathology
  • Myelodysplastic Syndromes / therapy
  • Parvovirus B19, Human / immunology