Antibody-mediated pure red-cell aplasia (PRCA): the Spanish experience

J Nephrol. 2005 Jul-Aug;18(4):382-7.

Abstract

Background: The incidence of antibody (Ab)-mediated pure red-cell aplasia (PRCA) in patients with chronic kidney disease (CKD) has increased between 1998 and 2002. After initially responding to treatment with recombinant human erythropoietic agents for CKD-associated anemia, patients became treatment-refractory and severely anemic. Although most PRCA cases have occurred in Europe, the varying epidemiologies among individual countries have not been well characterized.

Methods: We investigated Ab-mediated PRCA in 12 Spanish patients treated with epoetin alfa alone or prior to treatment with epoetin beta (n=1) or darbepoetin alfa (n=1). Serum Abs against epoetin alfa were detected by radioimmunoprecipitation (RIP) assay or bioassay. Following diagnosis of PRCA, erythropoietic treatment was stopped and patients received immunosuppressive therapy alone (n=11) or in combination with renal transplant (n=1).

Results: Treatments were administered for 16 months (average) before diagnosis of PRCA in bone marrow aspirates (n=8) or biopsies (n=4). At diagnosis, patients had an average of 0.68% blood reticulocytes and blood hemoglobin (Hb) level of 7.13 g/dL. Eight patients had anti-epoetin Abs detected by RIP, and 5 had neutralizing Abs measured in the bioassay. As of December 2003, 4 patients had died, 3 had no recovery, and 5 had recovered from anemia (blood Hb level, 9.9 g/dL). All 5 recovering patients received corticosteroid therapy alone, and 1 received a renal transplant as well as corticosteroids.

Conclusions: Sudden onset of treatment-refractory anemia in CKD patients suggests a course of treatment cessation followed by diagnostic procedures for Ab-mediated PRCA, and immunosuppressive therapy. This study may serve as a model for a centralized global PRCA registry.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies / immunology*
  • Biopsy
  • Bone Marrow / pathology
  • Darbepoetin alfa
  • Drug Therapy, Combination
  • Epoetin Alfa
  • Erythropoietin / analogs & derivatives
  • Erythropoietin / immunology*
  • Erythropoietin / therapeutic use
  • Female
  • Follow-Up Studies
  • Hematinics / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Male
  • Middle Aged
  • Radioimmunoprecipitation Assay
  • Recombinant Proteins
  • Red-Cell Aplasia, Pure / drug therapy
  • Red-Cell Aplasia, Pure / epidemiology
  • Red-Cell Aplasia, Pure / immunology*
  • Retrospective Studies
  • Spain / epidemiology

Substances

  • Antibodies
  • Hematinics
  • Immunosuppressive Agents
  • Recombinant Proteins
  • epoetin beta
  • Erythropoietin
  • Darbepoetin alfa
  • Epoetin Alfa