Pure red cell aplasia

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Pure red cell aplasia
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Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells. There are multiple etiologies that can cause PRCA. The condition has been first described by Paul Kaznelson in 1922. [1]

Contents

Signs and symptoms

Signs and symptoms may include:

Causes

Causes of PRCA include:

Treatment

PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as cyclosporin in many patients, [9] though this approach is not without risk. [10]

It has also been shown to respond to treatments with rituximab and tacrolimus.[ citation needed ]

See also

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References

  1. Kaznelson P (1922). "Zur Entstehung der Blutplättchen". Verh Dtsch Ges Inn Med. 34: 557–8.
  2. Hirokawa M, Sawada K, Fujishima N, et al. (January 2008). "Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group". Haematologica. 93 (1): 27–33. doi: 10.3324/haematol.11655 . PMID   18166782.
  3. Geetha D, Zachary JB, Baldado HM, Kronz JD, Kraus ES (December 2000). "Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature". Clinical Transplantation. 14 (6): 586–91. doi:10.1034/j.1399-0012.2000.140612.x. PMID   11127313.
  4. Kwong YL, Wong KF (1998). "Association of pure red cell aplasia with T large granular lymphocyte leukaemia". J. Clin. Pathol. 51 (9): 672–5. doi:10.1136/jcp.51.9.672. PMC   500904 . PMID   9930071.
  5. Miller AC, Rashid RM (2008). "Three episodes of acquired pure red cell aplasia restricted to pregnancy". Journal of Perinatal Medicine. 36 (3): 270–1. doi:10.1515/JPM.2008.041. PMID   18576941.
  6. Petrochko C (2009). "FDA Strengthens Warning on Transplant Drug." Medpage Today. 14 August 2009. Accessed 19 August 2009.
  7. Macdougall, IC (November 2007). "Epoetin-induced pure red cell aplasia: diagnosis and treatment". Current Opinion in Nephrology and Hypertension. 16 (6): 585–8. doi:10.1097/MNH.0b013e3282f0c4bf. PMID   18089975.
  8. Online Mendelian Inheritance in Man (OMIM): 105650
  9. Sawada K, Hirokawa M, Fujishima N, et al. (August 2007). "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. 92 (8): 1021–8. doi: 10.3324/haematol.11192 . PMID   17640861.
  10. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC   2592349 . PMID   18510682.
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