Medscape is available in 5 Language Editions – Choose your Edition here.


Pure Red Cell Aplasia Workup

  • Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD  more...
Updated: Feb 25, 2014

Laboratory Studies

CBC count, RBC indices, a reticulocyte count, and a differential analysis of WBCs should be obtained.

Iron studies, especially iron saturation and serum ferritin levels, are used to diagnose hemosiderosis. This possibility should be considered in patients who have received multiple transfusions.

Serum vitamin B-12 and folate levels might be indicated in patients with macrocytosis.

Lactate dehydrogenase (LDH), indirect bilirubin, and serum haptoglobin levels are used to detect hemolysis.

Hemoglobin A2 and hemoglobin F are used to rule out thalassemia.

Flow cytometry is used to diagnose hematological malignancies and T-cell disorders.

Tests to identify parvovirus B19 infection,[26] hepatitis, infectious mononucleosis, and other infections are indicated.

Tests to detect autoimmune disorders should include an antinuclear antibody test, a C-reactive protein (CRP) level, an erythrocyte sedimentation rate (ESR), a quantitative immunoglobulin analysis, and a direct Coombs test to detect an autoimmune hemolytic anemia. Tests for thyroiditis, diabetes, rheumatoid arthritis, Sjögren syndrome, and SLE might be indicated.

Hemoglobin F, “I” on the surface of erythrocytes, and adenosine deaminase determinations are helpful in diagnosing Diamond-Blackfan syndrome.

Bone marrow histology

Peripheral smears demonstrate a normocytic anemia in most cases of PRCA. However, macrocytic anemia occurs in Diamond-Blackfan and Good syndromes and in HIV infections. Peripheral smears can be used to screen for infectious mononucleosis, megaloblastosis, and hematological malignancies.

Bone marrow aspiration smears in pure red cell aplasia (PRCA) usually reveal a normocellular marrow. An absence of erythroblasts is noted, whereas more immature erythrocyte progenitors are present (maturation arrest). WBCs and platelet maturation are normal. Bone marrow can be used to evaluate iron stores and help diagnose megaloblastosis and hematological malignancies.



Positron emission tomography (PET) and CT scans are used to detect thymomas.

Spleen size can be determined by ultrasound imaging.

Appropriate imaging studies are used to help diagnose and evaluate hematological malignancies.

Duel-energy x-ray absorptiometry scans are used to assess osteopenia and osteoporosis due to corticosteroid therapy.



Procedures can include (1) bone marrow aspiration and biopsy and (2) appropriate biopsies to diagnose hematological malignancies.

Contributor Information and Disclosures

Paul Schick, MD Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

  1. Hoffman R, Benz EJ, Furie B, Shattil SJ. Hematology: Basic Principles and Practice. Philadelphia, Pa: Churchill Livingstone; 2009.

  2. Kaushansky, Kenneth, Lichtman Marshall A, Beutler, Ernest , et al. Williams Hematology. Eighth Edition. McGraw-Hill Professional; 2010.

  3. Van Hook JW, Gill P, Cyr D, Kapur RP. Diamond-Blackfan anemia as an unusual cause of nonimmune hydrops fetalis: a case report. Reprod Med. 40:850-854. [Medline].

  4. Hirokawa M. RPS19 mutations in patients with Diamond-Blackfan anemia. Br J Haematol. 2008. 142:911-20.

  5. Morimoto K, Lin S, Sakamoto K. The functions of RPS19 and their relationship to Diamond-Blackfan anemia: a review. Mol Genet Metab. 2007 Apr. 90(4):358-62. [Medline].

  6. Boultwood J, Yip BH, Vuppusetty C, Pellagatti A, Wainscoat JS. Activation of the mTOR pathway by the amino acid (L)-leucine in the 5q- syndrome and other ribosomopathies. Adv Biol Regul. 2013 Jan. 53(1):8-17. [Medline].

  7. Boultwood J, Pellagatti A, Wainscoat JS. Haploinsufficiency of ribosomal proteins and p53 activation in anemia: Diamond-Blackfan anemia and the 5q- syndrome. Adv Biol Regul. 2012 Jan. 52(1):196-203. [Medline].

  8. Ellis SR, Gleizes PE. Diamond Blackfan anemia: ribosomal proteins going rogue. Semin Hematol. 2011 Apr. 48(2):89-96. [Medline].

  9. Garçon L, Ge J, Manjunath SH, Mills JA, Apicella M, Parikh S. Ribosomal and hematopoietic defects in induced pluripotent stem cells derived from Diamond Blackfan anemia patients. Blood. 2013 Aug 8. 122(6):912-21. [Medline].

  10. Cherrick I, Karayalcin G, Lanzkowsky P. Transient erythroblastopenia of childhood. Prospective study of fifty patients. Am J Pediatr Hematol Oncol. 1994 Nov. 16(4):320-4. [Medline].

  11. Baker RI, Manoharan A, de Luca E, Begley CG. Pure red cell aplasia of pregnancy: a distinct clinical entity. Br J Haematol. 1993 Nov. 85(3):619-22. [Medline].

  12. Bierman PJ, Warkentin P, Hutchins MR, Klassen LW. Pure red cell aplasia following ABO mismatched marrow transplantation for chronic lymphocytic leukemia: response to antithymocyte globulin. Leuk Lymphoma. 1993 Jan. 9(1-2):169-71. [Medline].

  13. Bennett CL, Cournoyer D, Carson KR, Rossert J, Luminari S, Evens AM, et al. Long-term outcome of individuals with pure red cell aplasia and antierythropoietin antibodies in patients treated with recombinant epoetin: a follow-up report from the Research on Adverse Drug Events and Reports (RADAR) Project. Blood. 2005 Nov 15. 106(10):3343-7. [Medline]. [Full Text].

  14. Casadevall N. Pure red cell aplasia and anti-erythropoietin antibodies in patients treated with epoetin. Nephrol Dial Transplant. 2003 Nov. 18 Suppl 8:viii37-41. [Medline].

  15. Liu Z, Stoll VS, Devries PJ, Jakob CG, Xie N, Simmer RL, et al. A potent erythropoietin-mimicking human antibody interacts through a novel binding site. Blood. 2007 Oct 1. 110(7):2408-13. [Medline].

  16. al-Awami Y, Sears DA, Carrum G, Udden MM, Alter BP, Conlon CL. Pure red cell aplasia associated with hepatitis C infection. Am J Med Sci. 1997 Aug. 314(2):113-7. [Medline].

  17. Tomida S, Matsuzaki Y, Nishi M, Ikegami T, Chiba T, Abei M, et al. Severe acute hepatitis A associated with acute pure red cell aplasia. J Gastroenterol. 1996 Aug. 31(4):612-7. [Medline].

  18. Lee TH, Oh SJ, Hong S, Lee KB, Park H, Woo HY. Pure red cell aplasia caused by acute hepatitis a. Chonnam Med J. 2011 Apr. 47(1):51-3. [Medline]. [Full Text].

  19. Herbert KE, Prince HM, Westerman DA. Pure red-cell aplasia due to parvovirus B19 infection in a patient treated with alemtuzumab. Blood. 2003. 101:1654.

  20. Ahsan N, Holman MJ, Gocke CD, Groff JA, Yang HC. Pure red cell aplasia due to parvovirus B19 infection in solid organ transplantation. Clin Transplant. 1997 Aug. 11(4):265-70. [Medline].

  21. Smalling R, Foote M, Molineux G, Swanson SJ, Elliott S. Drug-induced and antibody-mediated pure red cell aplasia: a review of literature and current knowledge. Biotechnol Annu Rev. 2004. 10:237-50. [Medline].

  22. Thompson DF, Gales MA. Drug-induced pure red cell aplasia. Pharmacotherapy. 1996 Nov-Dec. 16(6):1002-8. [Medline].

  23. Li G, Li ZQ, Yang QY, Yang JD. Acquired pure red cell aplasia due to treatment with clopidogrel: first case report. J Thromb Thrombolysis. 2013 Dec 3. [Medline].

  24. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008 Aug. 142(4):505-14. [Medline]. [Full Text].

  25. 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. [Medline].

  26. Wong S, Brown KE. Development of an improved method of detection of infectious parvovirus B19. J Clin Virol. 2006 Apr. 35(4):407-13. [Medline].

  27. Björkholm M. Intravenous immunoglobulin treatment in cytopenic haematological disorders. J Intern Med. 1993 Aug. 234(2):119-26. [Medline].

  28. Dhodapkar MV, Lust JA, Phyliky RL. T-cell large granular lymphocytic leukemia and pure red cell aplasia in a patient with type I autoimmune polyendocrinopathy: response to immunosuppressive therapy. Mayo Clin Proc. 1994 Nov. 69(11):1085-8. [Medline].

  29. Helbig G, Stella-Holowiecka B, Krawczyk-Kulis M, Wojnar J, Markiewicz M, Wojciechowska-Sadus M. Successful treatment of pure red cell aplasia with repeated, low doses of rituximab in two patients after ABO-incompatible allogeneic haematopoietic stem cell transplantation for acute myeloid leukaemia. Haematologica. 2005 Nov. 90 Suppl:ECR33. [Medline].

  30. 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 Mar. 82(3):235-9. [Medline].

  31. Viviano KR, Webb JL. Clinical use of cyclosporine as an adjunctive therapy in the management of feline idiopathic pure red cell aplasia. J Feline Med Surg. 2011 Dec. 13(12):885-95. [Medline].

  32. Passweg JR, Rabusin M, Musso M, Beguin Y, Cesaro S, Ehninger G, et al. Haematopoetic stem cell transplantation for refractory autoimmune cytopenia. Br J Haematol. 2004 Jun. 125(6):749-55. [Medline].

  33. Musso M, Porretto F, Crescimanno A, Polizzi V, Scalone R. Donor lymphocyte infusions for refractory pure red cell aplasia relapsing after both autologous and nonmyeloablative allogeneic peripheral stem cell transplantation. Bone Marrow Transplant. 2004 Apr. 33(7):769-71. [Medline].

All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.