By: Neal Shah, Co Editor-In-Chief
Pure Red Cell Aplasia (PRCA), also known as erythroblastopenia, is characterized by a suppression of erythrocytes in the bone marrow. It is a peculiar oddity that the bone marrow’s progenitor cells still differentiate into white blood cells and platelets.1 PRCA has idiopathic, viral, auto-immune, and genetic etiologies. Diamond-Blackfan syndrome features PRCA due to defective red blood cell (RBC) production1 resulting from genetic abnormalities in ribosomal protein production.2 Infection with the parvovirus B19 is linked to PRCA in immunocompromised patients,3 and thymomas often present in up to 15% of PRCA cases.4
Drug induced PRCA is often seen with administration of erythropoiesis stimulating agents (ESA) such as Aranasp® (darbepoeitin) and Procrit® (epoeitin).5 The administration of these exogenous peptides results in antibody formation and subsequent destruction of erythrocyte precursors. PRCA occurs most frequently when ESAs are administered subcutaneously.6 Discontinuation of the ESA usually does not stop PRCA from occurring, but immunosuppressive agents are shown to slow progression.7 In March 2012, the FDA approved the drug Omontys® (peginesatide).8 This drug does not resemble typical ESA because it is not a direct erythropoietin mimetic; instead it is a pegylated dimer that directly activates erythropoesis. It is currently indicated for the treatment of anemia due to chronic kidney disease for patients on dialysis.10
Treatment of PRCA involves discontinuation of all ESA agents and a course of corticosteroids to suppress the immune system.11 Some immunosuppresants including cyclophosphamide and cyclosporine are also successful in mitigating PRCA.12,13 Rituximab is also shown to be effective in treating PRCA.14
- Raphael Rubin, David Strayer. Rubin’s Pathology, 5th edition. Lippincott Williams & Wilkins, Wolters Kluwer. 2008;867.
- Diamond-Blackfan anemia. Available at: http://ghr.nlm.nih.gov/condition/diamond-blackfan-anemia. Accessed June 24, 2012.
- Gilsanz F, Garcia Vela J, Vargas JA et al. Acquired pure red cell aplasia: a study of six cases. Ann Hematol. 1995;71(4):181—3.
- Pure Red Cell Aplasia. Available at: http://emedicine.medscape.com/article/205695-overview#a0104asd. Accessed June 24, 2012.
- Information on Erythropoiesis-Stimulating Agents (ESA) Epoetin alfa (marketed as Procrit, Epogen), Darbepoetin alfa (marketed as Aranesp). Available at: http://www.fda.gov/drugs/drugsafety/postmarketdrugsafetyinformationforpatientsandproviders/ucm109375.htm. Accessed June 24, 2012.
- Erythropoiesis-stimulating agents and antibody-mediated pure red-cell aplasia: here are we now and where do we go from here? Available at: http://ndt.oxfordjournals.org/content/19/2/288.full. Accessed June 24, 2012.
- Rossert J, Macdougall I, Casadevall N. Antibody-mediated pure red cell aplasia (PRCA) treatment and re-treatment: multiple options. Nephrol Dial Transplant. 2005;20 Suppl 4:iv23—2
- Mikhail A. Profile of peginesatide and its potential for the treatment of anemia in adults with chronic kidney disease who are on dialysis. J Blood Med. 2012;3:25—31. Epub 2012 May 23.
- Green JM, Leu K, Worth A et al. Peginesatide and erythropoietin stimulate similar erythropoietin receptor-mediated signal transduction and gene induction events. Exp Hematol. 2012;40(7):575—87. Epub 2012 Mar 6.
- Omontys [package insert]. Palo Alto, CA: Affymax, Inc; 2012.
- Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142(4):505—14.
- Gupta R, Ezeonyeji A, Thomas A, Scully M, Ehrenstein M, Isenberg D. A case of pure red cell aplasia and immune thrombocytopenia complicating systemic lupus erythematosus: Responseto rituximab and cyclophosphamide. Lupus. 2011;20(14):1547—1550.
- Björkholm M. Intravenous immunoglobulin treatment in cytopenic haematological disorders. J Intern Med. 1993;234(2):119—26.
- D’Arena G, Vigliotti ML, Dell’Olio M, Villa MR, Mantuano S, Scalzulli PR, et al. Rituximab to treat chronic lymphoproliferative disorder-associated pure red cell aplasia. Eur J Haematol. 2009;82(3):235—9.