Acquired pure red cell aplasia: updated review of treatment

Br J Haematol. 2008 Aug;142(4):505-14. doi: 10.1111/j.1365-2141.2008.07216.x. Epub 2008 May 28.


Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Alemtuzumab
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived
  • Antibodies, Neoplasm / therapeutic use
  • Antilymphocyte Serum / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Red-Cell Aplasia, Pure / diagnosis
  • Red-Cell Aplasia, Pure / drug therapy*
  • Red-Cell Aplasia, Pure / surgery
  • Rituximab
  • Thymectomy


  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived
  • Antibodies, Neoplasm
  • Antilymphocyte Serum
  • Immunosuppressive Agents
  • Alemtuzumab
  • Rituximab
  • Cyclosporine
  • Cyclophosphamide