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pure red cell aplasia; erythrocyte aplasia

Etiology: 1) T-cell autoimmune disease a) systemic lupus erythematosus b) lymphoproliferative disorders - large granular lymphocytosis - lymphocytic leukemia, lymphoma [2] c) thymoma d) pregnancy e) bone marrow disease & chronic hematologic disease - myleloproliferative disorders - myelodysplastic syndromes - Diamond-Blackfan syndrome f) solid tumors 2) viral infection a) parvovirus B19 (infects erythrocyte precursors) b) HIV1 c) hepatitis C [5] 3) ABO-incompatible stem cell transplantation [2] 4) pharmacologic causes: a) azathioprine b) carbamazepine c) chloramphenicol d) chlorpropamide e) erythropoietin (recombinant) - formation of erythropoietin Ab f) isoniazid g) mycophenolate h) phenytoin i) trimethoprim-sulfamethoxazole j) zidovudine Pathology: - T-cell autoimmune disease vs direct toxicity to erythrocyte precursors [2] Laboratory: 1) complete blood count (CBC) a) normocytic or macrocytic anemia b) platelets & leukocytes are unaffected 2) reticulocyte count < 25,000/uL or < 0.5% 3) bone marrow biopsy a) few erythroid precursors b) abnormalities restricted to erythroid progenitor cells [2] c) required for diagnosis [2] 4) normal BFU-E in stem cell culture generally predicts a good clinical response 5) parvovirus B19 testing a) parvovirus B19 IgM, parvovirus B19 IgG b) parvovirus B19 DNA 6) serum lactate dehydrogenase & serum haptoglobin normal [5] Radiology: - CT of chest to rule-out thymoma [2] Management: 1) stop offending drug 2) erythrocyte transfusions as needed 3) immunosuppressive therapy when cause is unknown [2] a) glucocorticoid alone or in combination with cyclosporine or cyclophosphamide b) improvement in 1-3 months c) antithymocyte globulin for thymoma [2] 4) thymectomy for thymoma [2]

Interactions

disease interactions

Related

erythrocyte; red blood cell (RBC) erythropoiesis

Specific

Diamond-Blackfan syndrome (congenital erythroid hypoplasia)

General

aplastic anemia

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2022 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Sawada K, Hirokawa M, Fujishima N. Diagnosis and management of acquired pure red cell aplasia. Hematol Oncol Clin North Am. 2009 Apr;23(2):249-59. PMID: 19327582
  4. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008 Aug;142(4):505-14 PMID: 18510682
  5. NEJM Knowledge+ Hematology
  6. Means RT JR Pure red cell aplasia. Blood. 2016 Nov 24;128(21):2504-2509 PMID: 27881371 Free article