History

Fact Explanation
An underlying predisposing condition Most of the times pure red cell aplasia is associated with autoimmune conditions, but there have been strong relationships to thymoma, chronic kidney disease, Parvovirus B19 infection, hematological malignancies and anti-erythropoietin antibodies. Out of the drugs (except erythropoietin, of course, since mentioned above), phenytoin, azathioprine, and isoniazid have been recognized as common drugs that cause pure red cell aplasia. [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14]
Features of severe anemia Severe exertional dyspnea, chest pain on exertion, fatigue and lethargy are major symptoms of severe anemia. The symptoms of leucocytopenia (i.e. frequent bacterial infections, poor wound healing ) or thrombocytopenia (i.e. mucosal bleeding, petichial hemorrhages) are not to be seen in pure red cell aplasia. [15] [16] [17] [18] [19]
Acutely presenting in children Acute acquired form is predominantly present in children. [1]
Chronically presenting in adults Chronic form is predominantly present in adults. [1]
Features of acute heart failure i.e. orthopnea, paroxysmal nocturnal dyspnea, exertional intolerance. Due to high output cardiac failure. [20] [21]
References
  1. SAWADA KENICHI, FUJISHIMA NAOHITO, HIROKAWA MAKOTO. Acquired pure red cell aplasia: updated review of treatment. [online] December, 142(4):505-514 [viewed 17 August 2014] Available from: doi:10.1111/j.1365-2141.2008.07216.x
  2. SAWADA KENICHI, HIROKAWA MAKOTO, FUJISHIMA NAOHITO. Diagnosis and Management of Acquired Pure Red Cell Aplasia. Hematology/Oncology Clinics of North America [online] 2009 April, 23(2):249-259 [viewed 17 August 2014] Available from: doi:10.1016/j.hoc.2009.01.009
  3. VERHELST DAVID, ROSSERT JéRôME, CASADEVALL NICOLE, KRüGER ANNE, ECKARDT KAI-UWE, MACDOUGALL IAIN C. Treatment of erythropoietin-induced pure red cell aplasia: a retrospective study. The Lancet [online] 2004 May, 363(9423):1768-1771 [viewed 17 August 2014] Available from: doi:10.1016/S0140-6736(04)16302-2
  4. DJALDETTI M, BLAY A, BERGMAN M, SALMAN H, BESSLER H. Pure red cell aplasia—a rare disease with multiple causes. Biomedicine & Pharmacotherapy [online] 2003 October, 57(8):326-332 [viewed 17 August 2014] Available from: doi:10.1016/j.biopha.2003.08.001
  5. GEETHA DUVURU, ZACHARY JAMES B, BALDADO HELEN M, KRONZ JOSEPH D, KRAUS EDWARD S. Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature. Clin Transplant [online] 2000 December, 14(6):586-591 [viewed 17 August 2014] Available from: doi:10.1034/j.1399-0012.2000.140612.x
  6. ECKARDT K.-U., CASADEVALL N.. Pure red-cell aplasia due to anti-erythropoietin antibodies. Nephrology Dialysis Transplantation [online] 2003 May, 18(5):865-869 [viewed 17 August 2014] Available from: doi:10.1093/ndt/gfg182
  7. BOVEN KATIA, STRYKER SCOTT, KNIGHT JOHN, THOMAS ADRIAN, VAN REGENMORTEL MARC, KEMENY DAVID M., POWER DAVID, ROSSERT JEROME, CASADEVALL NICOLE. The increased incidence of pure red cell aplasia with an Eprex formulation in uncoated rubber stopper syringes. Kidney Int [online] 2005 June, 67(6):2346-2353 [viewed 17 August 2014] Available from: doi:10.1111/j.1523-1755.2005.00340.x
  8. LOCATELLI F, VECCHIO L. D. and POZZONI P. Pure red-cell aplasia “epidemic”—mystery completely revealed? Perit Dial Int June [online] 2007 vol. 27 no. Supplement 2 S303-S307 [viewed 17 August 2014] Available from: http://www.pdiconnect.com/content/27/Supplement_2/S303.full
  9. THOMPSON DF, GALES MA. Drug-induced pure red cell aplasia. Pharmacotherapy [online] 1996 Nov-Dec, 16(6):1002-8 [viewed 17 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8947971, DOI: 10.1002/j.1875-9114.1996.tb03025.x
  10. LOULERGUE PIERRE, MIR OLIVIER, DHOTE ROBIN. Pure Red Blood Cell Aplasia and Isoniazid Use. Emerg. Infect. Dis. [online] 2007 September, 13(9):1427-1428 [viewed 17 August 2014] Available from: doi:10.3201/eid1309.061334
  11. MACDOUGALL IAIN C, ROGER SIMON D, DE FRANCISCO ANGEL, GOLDSMITH DAVID J A, SCHELLEKENS HUUB, EBBERS HANS, JELKMANN WOLFGANG, LONDON GéRARD, CASADEVALL NICOLE, HöRL WALTER H, KEMENY DAVID M, POLLOCK CAROL. Antibody-mediated pure red cell aplasia in chronic kidney disease patients receiving erythropoiesis-stimulating agents: new insights. Kidney Int [online] December, 81(8):727-732 [viewed 17 August 2014] Available from: doi:10.1038/ki.2011.500
  12. MACDOUGALL IAIN C, ROGER SIMON D, DE FRANCISCO ANGEL, GOLDSMITH DAVID J A, SCHELLEKENS HUUB, EBBERS HANS, JELKMANN WOLFGANG, LONDON GéRARD, CASADEVALL NICOLE, HöRL WALTER H, KEMENY DAVID M, POLLOCK CAROL. Antibody-mediated pure red cell aplasia in chronic kidney disease patients receiving erythropoiesis-stimulating agents: new insights. Kidney Int [online] December, 81(8):727-732 [viewed 17 August 2014] Available from: doi:10.1038/ki.2011.500
  13. POLLOCK C, JOHNSON DW, HöRL WH, ROSSERT J, CASADEVALL N, SCHELLEKENS H, DELAGE R, DE FRANCISCO A, MACDOUGALL I, THORPE R, TOFFELMIRE E. Pure red cell aplasia induced by erythropoiesis-stimulating agents. Clin J Am Soc Nephrol [online] 2008 Jan, 3(1):193-9 [viewed 17 August 2014] Available from: doi:10.2215/CJN.02440607
  14. BOVEN K.. Epoetin-associated pure red cell aplasia in patients with chronic kidney disease: solving the mystery. Nephrology Dialysis Transplantation [online] 2005 May, 20(suppl_3):iii33-iii40 [viewed 17 August 2014] Available from: doi:10.1093/ndt/gfh1072
  15. KALTER HD, BURNHAM G, KOLSTAD PR, HOSSAIN M, SCHILLINGER JA, KHAN NZ, SAHA S, DE WIT V, KENYA-MUGISHA N, SCHWARTZ B, BLACK RE. Evaluation of clinical signs to diagnose anaemia in Uganda and Bangladesh, in areas with and without malaria. Bull World Health Organ [online] 1997:103-11 [viewed 18 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9529723
  16. SHIH A, KASSANOFF RE, ALTRABULSI B. Severe anemia Proc (Bayl Univ Med Cent) [online] 2001 Jul, 14(3):289-293 [viewed 18 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1305832
  17. O'MARA N. B.. Anemia in Patients With Chronic Kidney Disease. Diabetes Spectrum [online] 2008 January, 21(1):12-19 [viewed 18 August 2014] Available from: doi:10.2337/diaspect.21.1.12
  18. BHATT V. and SALEEM A. Drug-Induced Neutropenia – Pathophysiology, Clinical Features, and Management. Ann Clin Lab Sci Spring [online] 2004 vol. 34 no. 2 131-137. [viewed 18 August 2014] Available from: http://www.annclinlabsci.org/content/34/2/131.full
  19. UHRYNOWSKA MALGORZATA, MASLANKA KRYSTYNA, ZUPANSKA BARBARA. Neonatal Thrombocytopenia: Incidence, Serological and Clinical Observations. Amer J Perinatol [online] December, 14(07):415-418 [viewed 19 August 2014] Available from: doi:10.1055/s-2007-994171
  20. CALDAS CEZAR AUGUSTO MUNIZ, CARVALHO JOZéLIO FREIRE. Pure red cell aplasia and primary antiphospholipid syndrome: a unique association. Rheumatol Int [online] December, 32(5):1363-1365 [viewed 19 August 2014] Available from: doi:10.1007/s00296-010-1387-4
  21. MEHTA P.A., DUBREY S.W.. High output heart failure. QJM [online] 2009 February, 102(4):235-241 [viewed 19 August 2014] Available from: doi:10.1093/qjmed/hcn147

Examination

Fact Explanation
Pallor Pallor in the mucus membranes, conjunctiva and palms/soles is a prominent feature of anemia. Absence of pallor does not rule out the presence of anemia. [1] [2] [3] [4] [5]
Signs of acute heart failure Ankle edema, bilateral fine crepitations of the lung, increased jugular venous pressure. Due to high output cardiac failure. [6] [7] [8] [9] [10]
References
  1. SHETH TN, BARTSSC, CHOUDHRY NK, BOWES M, DETSKY AS. The Relation of Conjunctival Pallor to the Presence of Anemia J Gen Intern Med [online] 1997 Feb, 12(2):102-106 [viewed 19 August 2014] Available from: doi:10.1046/j.1525-1497.1997.00014.x
  2. STOLTZFUS RJ, EDWARD-RAJ A, DREYFUSS ML, ALBONICO M, MONTRESOR A, DHOJ THAPA M, WEST KP JR, CHWAYA HM, SAVIOLI L, TIELSCH J. Clinical pallor is useful to detect severe anemia in populations where anemia is prevalent and severe. J Nutr [online] 1999 Sep, 129(9):1675-81 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10460203
  3. MISHRIKI YEHIA Y.. Conjunctival Pallor and the Presence of Anemia. J Gen Intern Med [online] 1997 July, 12(7):455-455 [viewed 19 August 2014] Available from: doi:10.1046/j.1525-1497.1997.012007455.x
  4. KALTER HD, BURNHAM G, KOLSTAD PR, HOSSAIN M, SCHILLINGER JA, KHAN NZ, SAHA S, DE WIT V, KENYA-MUGISHA N, SCHWARTZ B, BLACK RE. Evaluation of clinical signs to diagnose anaemia in Uganda and Bangladesh, in areas with and without malaria. Bull World Health Organ [online] 1997:103-11 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9529723
  5. WEBER MW, KELLINGRAY SD, PALMER A, JAFFAR S, MULHOLLAND EK, GREENWOOD BM. Pallor as a clinical sign of severe anaemia in children: an investigation in the Gambia. Bull World Health Organ [online] 1997:113-8 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/9529724
  6. MEHTA P.A., DUBREY S.W.. High output heart failure. QJM [online] 2009 February, 102(4):235-241 [viewed 19 August 2014] Available from: doi:10.1093/qjmed/hcn147
  7. CALDAS CEZAR AUGUSTO MUNIZ, CARVALHO JOZéLIO FREIRE. Pure red cell aplasia and primary antiphospholipid syndrome: a unique association. Rheumatol Int [online] December, 32(5):1363-1365 [viewed 19 August 2014] Available from: doi:10.1007/s00296-010-1387-4
  8. MARMONT A, PESCHLE C, SANGUINETI M, CONDORELLI M. Pure red cell aplasia (PRCA): Response of three patients of cyclophosphamide and/or antilymphocyte globulin (ALG) and demonstration of two types of serum IgG inhibitors to erythropoiesis. Blood [online] 1975 Feb, 45(2):247-61 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/1078982
  9. AOKI KAZUNARI, ONO YUICHIRO, TABATA SUMIE, MATSUSHITA AKIKO, ISHIKAWA TAKAYUKI. Successful treatment of anti-erythropoietin antibody-mediated pure red cell aplasia with low-dose prednisolone. Int J Hematol [online] December, 97(2):272-274 [viewed 19 August 2014] Available from: doi:10.1007/s12185-013-1258-3
  10. COATS A J S. Anaemia and heart failure. Heart [online] 2004 September, 90(9):977-979 [viewed 19 August 2014] Available from: doi:10.1136/hrt.2003.012997

Differential Diagnoses

Fact Explanation
Aplastic anemia Aplastic anemia is a life threatening condition where new blood cells are not produced from the bone marrow. Aplastic anemia also can be caused by autoimmune conditions (i.e. Lupus syndromes), drugs, pregnancy and infections (i.e. Parvovirus B19). It also can present with the same features of red cell aplasia. But here, all sell lineages are affected, so there will be features of leucopenia (i.e. recurrent infections, poor wound healing), and thrombocytopenia (i.e. petichial hemorrhages) also. A simple differentiating investigation is complete blood count, where you can see that there is a pancytopenia, not only anemia. [1] [2] [3] [4] [5] [6] [7] [8]
Iron deficiency anemia This is an important differential, especially in the tropical countries. Iron deficiency is a very common cause of anemia. In blood picture, hypochromic microcytic blood cells are seen, and serum iron studies (i.e. serum ferritin, serum iron) can be done if there is a doubt. [9] [10] [11]
Other causes of anemia Anemia is multifactorial. i.e. nutritional deficiencies (iron, folate, vitamin B12), hemorrhage, chronic disease, bone marrow failure, hemolysis. When the diagnosis is not clear, each of this entity should be evaluated separately. [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24]
Chronic heart failure The patients with severe anemia can present with heart failure. The patients with chronic heart failure can get anemia of chronic disease. So it is an important differential that should be assessed. [25] [26] [27] [28] [29] [30]
References
  1. YOUNG NEAL S, SCHEINBERG PHILLIP, CALADO RODRIGO T. Aplastic anemia. Current Opinion in Hematology [online] 2008 May, 15(3):162-168 [viewed 19 August 2014] Available from: doi:10.1097/MOH.0b013e3282fa7470
  2. GUINAN EVA C.. Acquired Aplastic Anemia in Childhood. Hematology/Oncology Clinics of North America [online] 2009 April, 23(2):171-191 [viewed 19 August 2014] Available from: doi:10.1016/j.hoc.2009.01.011
  3. YOUNG NS, CALADO RT, SCHEINBERG P. Current concepts in the pathophysiology and treatment of aplastic anemia. Blood [online] 2006 Oct 15, 108(8):2509-19 [viewed 19 August 2014] Available from: doi:10.1182/blood-2006-03-010777
  4. YOUNG NEAL S.. Acquired Aplastic Anemia. Ann Intern Med [online] 2002 April [viewed 19 August 2014] Available from: doi:10.7326/0003-4819-136-7-200204020-00011
  5. YOUNG NEAL S, SCHEINBERG PHILLIP, CALADO RODRIGO T. Aplastic anemia. Current Opinion in Hematology [online] 2008 May, 15(3):162-168 [viewed 19 August 2014] Available from: doi:10.1097/MOH.0b013e3282fa7470
  6. BRODSKY ROBERT A, JONES RICHARD J. Aplastic anaemia. The Lancet [online] 2005 May, 365(9471):1647-1656 [viewed 19 August 2014] Available from: doi:10.1016/S0140-6736(05)66515-4
  7. GUINAN E. C.. Diagnosis and Management of Aplastic Anemia. Hematology [online] December, 2011(1):76-81 [viewed 19 August 2014] Available from: doi:10.1182/asheducation-2011.1.76
  8. YOUNG N.S.. Aplastic anaemia. The Lancet [online] 1995 July, 346(8969):228-232 [viewed 19 August 2014] Available from: doi:10.1016/S0140-6736(95)91273-8
  9. CLARK S. F.. Iron Deficiency Anemia. Nutrition in Clinical Practice [online] 2008 April, 23(2):128-141 [viewed 19 August 2014] Available from: doi:10.1177/0884533608314536
  10. JOHNSON-WIMBLEY TD, GRAHAM DY. Diagnosis and management of iron deficiency anemia in the 21st century Therap Adv Gastroenterol [online] 2011 May, 4(3):177-184 [viewed 19 August 2014] Available from: doi:10.1177/1756283X11398736
  11. LIU K, KAFFES AJ. Iron deficiency anaemia: a review of diagnosis, investigation and management. Eur J Gastroenterol Hepatol [online] 2012 Feb, 24(2):109-16 [viewed 19 August 2014] Available from: doi:10.1097/MEG.0b013e32834f3140
  12. BAKER SJ, DEMAEYER EM. Nutritional anemia: its understanding and control with special reference to the work of the World Health Organization. Am J Clin Nutr [online] 1979 Feb, 32(2):368-417 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/369352
  13. CARMEL RALPH. Nutritional Anemias and the Elderly. Seminars in Hematology [online] 2008 October, 45(4):225-234 [viewed 19 August 2014] Available from: doi:10.1053/j.seminhematol.2008.07.009
  14. LINDENBAUM J, ROMAN MJ. Nutritional anemia in alcoholism. Am J Clin Nutr [online] 1980 Dec, 33(12):2727-35 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/7001890
  15. MAZZA JJ. Insights into Global Nutritional Anemia Clin Med Res [online] 2007 Dec, 5(4):209 [viewed 19 August 2014] Available from: doi:10.3121/cmr.2007.783
  16. KAFERLE J, STRZODA CE. Evaluation of macrocytosis. Am Fam Physician [online] 2009 Feb 1, 79(3):203-8 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19202968
  17. DHALIWAL G, CORNETT PA, TIERNEY LM JR. Hemolytic anemia. Am Fam Physician [online] 2004 Jun 1, 69(11):2599-606 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/15202694
  18. POYART CLAUDE, WAJCMAN HENRI. Chapter 2 Hemolytic anemias due to hemoglobinopathies. Molecular Aspects of Medicine [online] 1996 April, 17(2):129-142 [viewed 19 August 2014] Available from: doi:10.1016/0098-2997(96)88344-0
  19. GEHRS BRADLEY C., FRIEDBERG RICHARD C.. Autoimmune hemolytic anemia. Am. J. Hematol. [online] December, 69(4):258-271 [viewed 19 August 2014] Available from: doi:10.1002/ajh.10062
  20. ROSSE WF, HILLMEN P, SCHREIBER AD. Immune-mediated hemolytic anemia. Hematology Am Soc Hematol Educ Program [online] 2004:48-62 [viewed 19 August 2014] Available from: doi:10.1182/asheducation-2004.1.48
  21. EVANS ER. DIAGNOSIS OF THE HEMOLYTIC ANEMIAS Calif Med [online] 1951 Oct, 75(4):271-275 [viewed 19 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1520966
  22. ALEXANIAN R, ALFREY C. Erythropoiesis in the anemia of bone marrow failure J Clin Invest [online] 1970 Nov, 49(11):1986-1992 [viewed 19 August 2014] Available from: doi:10.1172/JCI106418
  23. CECCALDI R, PARMAR K, MOULY E, DELORD M, KIM JM, REGAIRAZ M, PLA M, VASQUEZ N, ZHANG QS, PONDARRE C, PEFFAULT DE LATOUR R, GLUCKMAN E, CAVAZZANA-CALVO M, LEBLANC T, LARGHERO J, GROMPE M, SOCIé G, D'ANDREA AD, SOULIER J. Bone marrow failure in Fanconi anemia is triggered by an exacerbated p53/p21 DNA damage response that impairs hematopoietic stem and progenitor cells. Cell Stem Cell [online] 2012 Jul 6, 11(1):36-49 [viewed 19 August 2014] Available from: doi:10.1016/j.stem.2012.05.013
  24. ALEXANIAN R, ALFREY C. Erythropoiesis in the anemia of bone marrow failure. J Clin Invest [online] 1970 Nov, 49(11):1986-92 [viewed 19 August 2014] Available from: doi:10.1172/JCI106418
  25. MEHTA P.A., DUBREY S.W.. High output heart failure. QJM [online] 2009 February, 102(4):235-241 [viewed 19 August 2014] Available from: doi:10.1093/qjmed/hcn147
  26. CALDAS CEZAR AUGUSTO MUNIZ, CARVALHO JOZéLIO FREIRE. Pure red cell aplasia and primary antiphospholipid syndrome: a unique association. Rheumatol Int [online] December, 32(5):1363-1365 [viewed 19 August 2014] Available from: doi:10.1007/s00296-010-1387-4
  27. COATS A J S. Anaemia and heart failure. Heart [online] 2004 September, 90(9):977-979 [viewed 19 August 2014] Available from: doi:10.1136/hrt.2003.012997
  28. DRAKOS SG, ANASTASIOU-NANA MI, MALLIARAS KG, NANAS JN. Anemia in chronic heart failure. Congest Heart Fail [online] 2009 Mar-Apr, 15(2):87-92 [viewed 19 August 2014] Available from: doi:10.1111/j.1751-7133.2009.00049.x
  29. TANG WH, YEO PS. Epidemiology of anemia in heart failure. Heart Fail Clin [online] 2010 Jul, 6(3):271-8 [viewed 19 August 2014] Available from: doi:10.1016/j.hfc.2010.03.007
  30. OKONKO DARLINGTON O., ANKER STEFAN D.. Anemia in chronic heart failure: pathogenetic mechanisms. Journal of Cardiac Failure [online] 2004 February, 10(1):S5-S9 [viewed 19 August 2014] Available from: doi:10.1016/j.cardfail.2004.01.004

Investigations - for Diagnosis

Fact Explanation
Complete blood count Hemoglobin and red blood cell count is low, but other cell lineages are not affected. The MCV and MCH are normal. [1] [2]
Blood picture There is a normocytic normochromic anemia. [1] [3] [4]
Bone marrow biopsy for histology Shows hypoplasia of the erythroid series of cells. [1] [5] [6] [7]
Echocardiography To exclude or quantify cardiac failure. [1] [9]
CT scan neck To exclude thymoma. [10] [11]
References
  1. SAWADA K, FUJISHIMA N, HIROKAWA M. Acquired pure red cell aplasia: updated review of treatment Br J Haematol [online] 2008 Aug, 142(4):505-514 [viewed 19 August 2014] Available from: doi:10.1111/j.1365-2141.2008.07216.x
  2. LOULERGUE P, MIR O, DHOTE R. Pure Red Blood Cell Aplasia and Isoniazid Use Emerg Infect Dis [online] 2007 Sep, 13(9):1427-1428 [viewed 19 August 2014] Available from: doi:10.3201/eid1309.061334
  3. BALAKRISHNAN A, VALSALAN R, SHESHADRI S, PANDIT VR, MEDEP V, AGRAWAL RK. Zidovudine-induced reversible pure red cell aplasia Indian J Pharmacol [online] 2010 Jun, 42(3):189-191 [viewed 19 August 2014] Available from: doi:10.4103/0253-7613.66845
  4. CHOI JH, OH YH, PARK IK. A Case of Pure Red Cell Aplasia Associated with Angioimmunoblastic T-cell Lymphoma Cancer Res Treat [online] 2010 Jun, 42(2):115-117 [viewed 19 August 2014] Available from: doi:10.4143/crt.2010.42.2.115
  5. SAHA M, RAY S, KUNDU S, CHAKRABARTI P. Pure red cell aplasia following autoimmune hemolytic anemia: An enigma. J Postgrad Med [serial online] 2013 [cited 2014 Aug 23];59:51-3. Available from: http://www.jpgmonline.com/text.asp?2013/59/1/51/109495
  6. VLACHAKI EFTHYMIA, DIAMANTIDIS MICHAEL D., KLONIZAKIS PHILIPPOS, HARALAMBIDOU-VRANITSA STYLIANI, IOANNIDOU-PAPAGIANNAKI ELIZABETH, KLONIZAKIS IOANNIS. Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association. The Scientific World Journal [online] 2012 December, 2012:1-7 [viewed 23 August 2014] Available from: doi:10.1100/2012/475313
  7. KATZ LJ, HOFFMAN R, RITCHEY AK, DAINIAK N. The proliferative capacity of pure red cell aplasia bone marrow cells. Yale J Biol Med [online] 1981, 54(2):89-94 [viewed 23 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2595869
  8. ROSU CRISTIAN, COHEN SANDRA, MEUNIER CAROLINE, OUELLETTE DENISE, BEAUCHAMP GILLES, RAKOVICH GEORGE. Pure red cell aplasia and associated thymoma. Clinics Pract [online] 2011 March [viewed 23 August 2014] Available from: doi:10.4081/cp.2011.e1
  9. KIM YOO RI, PYUN WOOK BUM, SHIN GIL JA. Relation of Anemia to Echocardiographically Estimated Left Ventricular Filling Pressure in Hypertensive Patients Over 50 Year-Old. J Cardiovasc Ultrasound [online] 2010 December [viewed 23 August 2014] Available from: doi:10.4250/jcu.2010.18.3.86
  10. ROSU CRISTIAN, COHEN SANDRA, MEUNIER CAROLINE, OUELLETTE DENISE, BEAUCHAMP GILLES, RAKOVICH GEORGE. Pure red cell aplasia and associated thymoma. Clinics Pract [online] 2011 March [viewed 23 August 2014] Available from: doi:10.4081/cp.2011.e1
  11. MIZOBUCHI S., YAMASHIRO T., NONAMI Y., YAMAMOTO A., KUME M., NAKAYA H., SAWADA T., TAGUCHI H., MORIKI T., OGOSHI S.. Pure Red Cell Aplasia and Myasthenia Gravis with Thymoma: A Case Report and Review of the Literature. Japanese Journal of Clinical Oncology [online] 1998 November, 28(11):696-701 [viewed 23 August 2014] Available from: doi:10.1093/jjco/28.11.696

Management - General Measures

Fact Explanation
Educate the patient Educate the patient about the nature and course of the disease, and the necessity of adherence to the treatments.
Treat the underlying disease There are so many associated disease and predisposing conditions, drugs studied in relation to pure red cell aplasia. their diagnosis and management should be done as indicated. i.e. surgical removal of thymomas, optimizing renal functions in chronic kidney disease. [1] [2] [3] [4] [5] [6] [7] [8] [9] [10]
Stop the offending drugs if any The known drugs that can be stopped should be stopped. i.e. erythropietin,phenytoin, azathioprin. Other drug classes should be considered instead. [11] [12] [13]
References
  1. SAWADA KENICHI, FUJISHIMA NAOHITO, HIROKAWA MAKOTO. Acquired pure red cell aplasia: updated review of treatment. [online] December, 142(4):505-514 [viewed 17 August 2014] Available from: doi:10.1111/j.1365-2141.2008.07216.x
  2. SAWADA KENICHI, HIROKAWA MAKOTO, FUJISHIMA NAOHITO. Diagnosis and Management of Acquired Pure Red Cell Aplasia. Hematology/Oncology Clinics of North America [online] 2009 April, 23(2):249-259 [viewed 17 August 2014] Available from: doi:10.1016/j.hoc.2009.01.009
  3. VERHELST DAVID, ROSSERT JéRôME, CASADEVALL NICOLE, KRüGER ANNE, ECKARDT KAI-UWE, MACDOUGALL IAIN C. Treatment of erythropoietin-induced pure red cell aplasia: a retrospective study. The Lancet [online] 2004 May, 363(9423):1768-1771 [viewed 17 August 2014] Available from: doi:10.1016/S0140-6736(04)16302-2
  4. DJALDETTI M, BLAY A, BERGMAN M, SALMAN H, BESSLER H. Pure red cell aplasia—a rare disease with multiple causes. Biomedicine & Pharmacotherapy [online] 2003 October, 57(8):326-332 [viewed 17 August 2014] Available from: doi:10.1016/j.biopha.2003.08.001
  5. GEETHA DUVURU, ZACHARY JAMES B, BALDADO HELEN M, KRONZ JOSEPH D, KRAUS EDWARD S. Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature. Clin Transplant [online] 2000 December, 14(6):586-591 [viewed 17 August 2014] Available from: doi:10.1034/j.1399-0012.2000.140612.x
  6. LOCATELLI F, VECCHIO L. D. and POZZONI P. Pure red-cell aplasia “epidemic”—mystery completely revealed? Perit Dial Int June [online] 2007 vol. 27 no. Supplement 2 S303-S307 [viewed 17 August 2014] Available from: http://www.pdiconnect.com/content/27/Supplement_2/S303.full
  7. LOULERGUE PIERRE, MIR OLIVIER, DHOTE ROBIN. Pure Red Blood Cell Aplasia and Isoniazid Use. Emerg. Infect. Dis. [online] 2007 September, 13(9):1427-1428 [viewed 17 August 2014] Available from: doi:10.3201/eid1309.061334
  8. MACDOUGALL IAIN C, ROGER SIMON D, DE FRANCISCO ANGEL, GOLDSMITH DAVID J A, SCHELLEKENS HUUB, EBBERS HANS, JELKMANN WOLFGANG, LONDON GéRARD, CASADEVALL NICOLE, HöRL WALTER H, KEMENY DAVID M, POLLOCK CAROL. Antibody-mediated pure red cell aplasia in chronic kidney disease patients receiving erythropoiesis-stimulating agents: new insights. Kidney Int [online] December, 81(8):727-732 [viewed 17 August 2014] Available from: doi:10.1038/ki.2011.500
  9. POLLOCK C, JOHNSON DW, HöRL WH, ROSSERT J, CASADEVALL N, SCHELLEKENS H, DELAGE R, DE FRANCISCO A, MACDOUGALL I, THORPE R, TOFFELMIRE E. Pure red cell aplasia induced by erythropoiesis-stimulating agents. Clin J Am Soc Nephrol [online] 2008 Jan, 3(1):193-9 [viewed 17 August 2014] Available from: doi:10.2215/CJN.02440607
  10. BOVEN K.. Epoetin-associated pure red cell aplasia in patients with chronic kidney disease: solving the mystery. Nephrology Dialysis Transplantation [online] 2005 May, 20(suppl_3):iii33-iii40 [viewed 17 August 2014] Available from: doi:10.1093/ndt/gfh1072
  11. ECKARDT K.-U., CASADEVALL N.. Pure red-cell aplasia due to anti-erythropoietin antibodies. Nephrology Dialysis Transplantation [online] 2003 May, 18(5):865-869 [viewed 17 August 2014] Available from: doi:10.1093/ndt/gfg182
  12. BOVEN KATIA, STRYKER SCOTT, KNIGHT JOHN, THOMAS ADRIAN, VAN REGENMORTEL MARC, KEMENY DAVID M., POWER DAVID, ROSSERT JEROME, CASADEVALL NICOLE. The increased incidence of pure red cell aplasia with an Eprex formulation in uncoated rubber stopper syringes. Kidney Int [online] 2005 June, 67(6):2346-2353 [viewed 17 August 2014] Available from: doi:10.1111/j.1523-1755.2005.00340.x
  13. THOMPSON DF, GALES MA. Drug-induced pure red cell aplasia. Pharmacotherapy [online] 1996 Nov-Dec, 16(6):1002-8 [viewed 17 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8947971, DOI: 10.1002/j.1875-9114.1996.tb03025.x

Management - Specific Treatments

Fact Explanation
Red cell concentrate transfusion To correct anemia rapidly. Some patients might exhibit transfusion dependence, which might resolve spontaneously or with danazol. [1] [2]
Danazol Maintains remission in refractory cases. [3] [4]
Corticosteroids First line treatment, relapses are common though. [2] [5] [6]
Other immunosuppressive drugs In some studies they have proven that up to 80% of the patients can relapse after corticosteroids. The other drugs that can be used are cyclosporine A, other cytotoxic immunosuppressive dugs alemtuzumab and rituximab. [2] [7] [8] [9] [10]
References
  1. STRAVITZ R. TODD, CHUNG HAROLD, STERLING RICHARD K., LUKETIC VELIMIR A., SANYAL ARUN J., PRICE ANGIE S., PURRINGTON AMY, SHIFFMAN MITCHELL L.. Antibody-Mediated Pure Red Cell Aplasia Due to Epoetin Alfa During Antiviral Therapy of Chronic Hepatitis C. Am J Gastroenterology [online] 2005 June, 100(6):1415-1419 [viewed 23 August 2014] Available from: doi:10.1111/j.1572-0241.2005.41910.x
  2. SAWADA K, FUJISHIMA N, HIROKAWA M. Acquired pure red cell aplasia: updated review of treatment Br J Haematol [online] 2008 Aug, 142(4):505-514 [viewed 23 August 2014] Available from: doi:10.1111/j.1365-2141.2008.07216.x
  3. CHANG JAE C., SLUTZKER BERKELEY, LINDSAY NAOMI. Remission of pure red cell aplasia following oxymetholone therapy. The American Journal of the Medical Sciences [online] 1978 May, 275(3):345-351 [viewed 23 August 2014] Available from: doi:10.1097/00000441-197805000-00012
  4. KWONG Y. L., WONG K. F., LIANG R. H. S., CHU Y. C., CHAN L. C., CHAN T. K.. Pure red cell aplasia: Clinical features and treatment results in 16 cases. Annals of Hematology [online] 1996 March, 72(3):137-140 [viewed 23 August 2014] Available from: doi:10.1007/s002770050151
  5. YANG MUH-HWA, HSU HUI-CHI. Pure red cell aplasia after ABO-incompatible allogeneic stem cell transplantation in severe aplastic anemia with response to steroids: a case report and literature review. Annals of Hematology [online] 2001 May, 80(5):299-301 [viewed 23 August 2014] Available from: doi:10.1007/s002770000275
  6. CLARK DA, DESSYPRIS EN, KRANTZ SB. Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients. Blood [online] 1984 Feb, 63(2):277-86 [viewed 23 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/6581839
  7. BURNSCOX C. EARLY DETECTION OF DIABETIC RETINOPATHY. The Lancet [online] 1984 September, 324(8404):693-694 [viewed 23 August 2014] Available from: doi:10.1016/S0140-6736(84)91246-7
  8. SAWADA K.-I., HIROKAWA M., FUJISHIMA N., TERAMURA M., BESSHO M., DAN K., TSURUMI H., NAKAO S., URABE A., OMINE M., OZAWA K.. Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group. Haematologica [online] 2007 August, 92(8):1021-1028 [viewed 23 August 2014] Available from: doi:10.3324/haematol.11192
  9. D’ARENA GIOVANNI, VIGLIOTTI MARIA LUIGIA, DELL’OLIO MATTEO, VILLA MARIA ROSARIA, MANTUANO SAVERIO, SCALZULLI POTITO ROSARIO, LA SALA ANTONIO, ABBADESSA ANTONIO, MASTRULLO LUCIA, CASCAVILLA NICOLA. Rituximab to treat chronic lymphoproliferative disorder-associated pure red cell aplasia. [online] 2009 March, 82(3):235-239 [viewed 23 August 2014] Available from: doi:10.1111/j.1600-0609.2008.01187.x
  10. GHAZAL H. Successful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia. Blood [online] 2002 Feb 1, 99(3):1092-4 [viewed 23 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/11807020