{"id":1045,"date":"2012-07-01T00:00:03","date_gmt":"2012-07-01T07:00:03","guid":{"rendered":"http:\/\/rhochistj.org\/RhoChiPost\/?p=1045"},"modified":"2014-02-17T22:13:17","modified_gmt":"2014-02-18T05:13:17","slug":"pathology-pure-red-cell-aplasia","status":"publish","type":"post","link":"https:\/\/rhochistj.org\/RhoChiPost\/pathology-pure-red-cell-aplasia\/","title":{"rendered":"The Pathology of Pure Red Cell Aplasia"},"content":{"rendered":"

By: Neal Shah, Co Editor-In-Chief<\/span><\/p>\n

–<\/p>\n

Pure Red Cell Aplasia (PRCA), also known as erythroblastopenia, is characterized by a suppression of erythrocytes in the bone marrow. \u00a0It is a peculiar oddity that the bone marrow\u2019s progenitor cells still differentiate into white blood cells and platelets.1<\/sup> \u00a0PRCA has idiopathic, viral, auto-immune, and genetic etiologies. \u00a0Diamond-Blackfan syndrome features PRCA due to defective red blood cell (RBC) production1<\/sup> resulting from genetic abnormalities in ribosomal protein production.2<\/sup> \u00a0Infection with the parvovirus B19 is linked to PRCA in immunocompromised patients,3<\/sup> and thymomas often present in up to 15% of PRCA cases.4<\/sup><\/p>\n

Drug induced PRCA is often seen with administration of erythropoiesis stimulating agents (ESA) such as Aranasp\u00ae (darbepoeitin) and Procrit\u00ae (epoeitin).5<\/sup> \u00a0The administration of these exogenous peptides results in antibody formation and subsequent destruction of erythrocyte precursors. \u00a0PRCA occurs most frequently when ESAs are administered subcutaneously.6<\/sup> \u00a0Discontinuation of the ESA usually does not stop PRCA from occurring, but immunosuppressive agents are shown to slow progression.7<\/sup> \u00a0In March 2012, the FDA approved the drug Omontys\u00ae (peginesatide).8<\/sup> \u00a0This drug does not resemble typical ESA because it is not a direct erythropoietin mimetic; instead it is a pegylated dimer that directly activates erythropoesis. \u00a0It is currently indicated for the treatment of anemia due to chronic kidney disease for patients on dialysis.10<\/sup><\/p>\n

Treatment of PRCA involves discontinuation of all ESA agents and a course of corticosteroids to suppress the immune system.11 <\/sup>\u00a0Some immunosuppresants including cyclophosphamide and cyclosporine are also successful in mitigating PRCA.12,13<\/sup> \u00a0Rituximab is also shown to be effective in treating PRCA.14<\/sup><\/p>\n

SOURCES:<\/strong><\/p>\n

    \n
  1. Raphael Rubin, David Strayer. Rubin\u2019s Pathology, 5th<\/sup> edition. Lippincott Williams & Wilkins, Wolters Kluwer. 2008;867.<\/li>\n
  2. Diamond-Blackfan anemia. Available at: http:\/\/ghr.nlm.nih.gov\/condition\/diamond-blackfan-anemia<\/a>. Accessed June 24, 2012.<\/li>\n
  3. Gilsanz F, Garcia Vela J, Vargas JA et al. Acquired pure red cell aplasia: a study of six cases. Ann Hematol<\/i>. 1995;71(4):181\u20143.<\/li>\n
  4. Pure Red Cell Aplasia. Available at: http:\/\/emedicine.medscape.com\/article\/205695-overview#a0104asd<\/a>. Accessed June 24, 2012.<\/li>\n
  5. 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<\/a>. Accessed June 24, 2012.<\/li>\n
  6. 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<\/a>. Accessed June 24, 2012.<\/li>\n
  7. Rossert J, Macdougall I, Casadevall N. Antibody-mediated pure red cell aplasia (PRCA) treatment and re-treatment: multiple options. Nephrol Dial Transplant<\/i>. 2005;20 Suppl 4:iv23\u20142<\/li>\n
  8. 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<\/i>. 2012;3:25\u201431. Epub 2012 May 23.<\/li>\n
  9. Green JM, Leu K, Worth A et al. Peginesatide and erythropoietin stimulate similar erythropoietin receptor-mediated signal transduction and gene induction events. Exp Hematol<\/i>. 2012;40(7):575\u201487. Epub 2012 Mar 6.<\/li>\n
  10. Omontys [package insert]. Palo Alto, CA: Affymax, Inc; 2012.<\/li>\n
  11. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol<\/i>. 2008;142(4):505\u201414.<\/li>\n
  12. 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<\/i>. 2011;20(14):1547\u20141550.<\/li>\n
  13. Bj\u00f6rkholm M. Intravenous immunoglobulin treatment in cytopenic haematological disorders. J Intern Med<\/i>. 1993;234(2):119\u201426.<\/li>\n
  14. 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<\/i>. 2009;82(3):235\u20149.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"

    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. \u00a0It is a peculiar oddity that the bone marrow\u2019s progenitor cells still differentiate into white blood cells and platelets.1 \u00a0PRCA has idiopathic, viral, auto-immune, and genetic etiologies. \u00a0Diamond-Blackfan syndrome…<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[7],"tags":[1825,871,343,53,22,120,1739,66,15,60,13,2229,2227,29,2232,314,62,1646,96,2235,86,363,1499,1500,671,220,21,149,453,16,1061],"class_list":["post-1045","post","type-post","status-publish","format-standard","hentry","category-clinical","tag-agents","tag-alfa","tag-and","tag-anemia","tag-blood","tag-condition","tag-cyclosporine","tag-dialysis","tag-disease","tag-disorder","tag-drug","tag-drugs","tag-ebola-virus-disease","tag-editor","tag-fda","tag-for","tag-gene","tag-intravenous","tag-june","tag-kidney","tag-lupus","tag-of","tag-omontys","tag-peginesatide","tag-r","tag-review","tag-study","tag-syndrome","tag-system","tag-treatment","tag-with"],"views":1128,"_links":{"self":[{"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/posts\/1045","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/comments?post=1045"}],"version-history":[{"count":0,"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/posts\/1045\/revisions"}],"wp:attachment":[{"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/media?parent=1045"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/categories?post=1045"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/rhochistj.org\/RhoChiPost\/wp-json\/wp\/v2\/tags?post=1045"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}