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Pure red cell aplasia

May 05, 2024

Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of aplastic anemia affecting the precursors to red blood cells but usually 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]

Pure red cell aplasia
Scanning electron micrograph of blood cells. From left to right: human red blood cell, thrombocyte (platelet), leukocyte. Pure red cell aplasia affects the red blood cells in particular.
SpecialtyHematology 

Signs and symptoms edit

Signs and symptoms may include:

  • Pale appearance
  • Rapid heart rate
  • Fatigue

Causes edit

Causes of PRCA include:

Diagnosis edit

Treatment edit

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]

For cases related to B19 parvovirus, administration of commercial immunoglobulin can treat or cure parvovirus by replacing neutralizing antibodies.[11]

See also edit

References edit

  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. S2CID 39011566.
  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. S2CID 24613046.
  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.
  11. ^ National Organization for Rare Disorders. (2003). Nord Guide to Rare Disorders. Lippincott Williams & Wilkins.

External links edit

May 05, 2024
pure, cell, aplasia, prca, erythroblastopenia, refers, type, aplastic, anemia, affecting, precursors, blood, cells, usually, white, blood, cells, prca, bone, marrow, ceases, produce, blood, cells, there, multiple, etiologies, that, cause, prca, condition, been. Pure red cell aplasia PRCA or erythroblastopenia refers to a type of aplastic anemia affecting the precursors to red blood cells but usually 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 Pure red cell aplasiaScanning electron micrograph of blood cells From left to right human red blood cell thrombocyte platelet leukocyte Pure red cell aplasia affects the red blood cells in particular SpecialtyHematology Contents 1 Signs and symptoms 2 Causes 3 Diagnosis 4 Treatment 5 See also 6 References 7 External linksSigns and symptoms editSigns and symptoms may include Pale appearance Rapid heart rate FatigueCauses editCauses of PRCA include Autoimmune disease Thymoma 2 Viral infections such as HIV herpes parvovirus B19 Fifth disease 3 or hepatitis citation needed Lymphoproliferative Association of pure red cell aplasia with T cell large granular lymphocyte leukemia is well recognized especially in China 4 Idiopathic Many cases of PRCA are considered idiopathic in that there is no discernible cause detected 5 Drugs such as mycophenolic acid 6 or erythropoietin 7 citation needed Congenital The term hereditary pure red cell aplasia has been used to refer to Diamond Blackfan anemia 8 Diagnosis editThis section is empty You can help by adding to it February 2024 Treatment editPRCA 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 For cases related to B19 parvovirus administration of commercial immunoglobulin can treat or cure parvovirus by replacing neutralizing antibodies 11 See also editDiamond Blackfan anemia genetic red cell aplasia Aplastic anemia aplasia affecting other bone marrow cells as well References edit Kaznelson P 1922 Zur Entstehung der Blutplattchen Verh Dtsch Ges Inn Med 34 557 8 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 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 S2CID 39011566 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 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 Petrochko C 2009 FDA Strengthens Warning on Transplant Drug Medpage Today 14 August 2009 Accessed 19 August 2009 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 S2CID 24613046 Online Mendelian Inheritance in Man OMIM 105650 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 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 National Organization for Rare Disorders 2003 Nord Guide to Rare Disorders Lippincott Williams amp Wilkins External links edit Retrieved from https en wikipedia org w index php title Pure red cell aplasia amp oldid 1210938812, wikipedia, wiki, book, books, library,

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