| Pure red cell aplasia | |
| Classification and external resources | |
| ICD-10 | D60. |
|---|---|
| ICD-9 | 284.8 |
| DiseasesDB | 29063 |
| eMedicine | med/1967 |
| MeSH | D012010 |
Pure red cell aplasia (or PRCA) 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.
Contents |
Causes
Pure red cell aplasia is regarded as an autoimmune disease.
It may also be a manifestation of thymoma. [1]
It may also be as a result of viral infections such as HIV, herpes, parvovirus B19 (Fifth disease),[2] or hepatitis. Association of pure red cell aplasia with T large granular lymphocyte leukemia is also well recognized, especially in China.[3] Many cases of PRCA are considered idiopathic in that there is no discernible cause detected. [4]
It can be associated with the administration of erythropoietin.[5]]
Types: 1-Acute pure red cell aplasia.
2-Chronic pure red cell aplasia-Congenital.
3-Chronic pure red cell aplasia-Acquired.
Treatment
PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin in many patients,[6] though this approach is not without risk.[7]
It has also been shown to respond to treatments with Rituxan.
See also
- Diamond-Blackfan anemia (genetic red cell aplasia)
- aplastic anemia (aplasia affecting other bone marrow cells as well)
References
- ^ 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:. PMID 18166782. http://www.haematologica.org/cgi/pmidlookup?view=long&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". Clin Transplant 14 (6): 586–91. PMID 11127313. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0902-0063&date=2000&volume=14&issue=6&spage=586.
- ^ Kwong YL, Wong KF (1998). "Association of pure red cell aplasia with T large granular lymphocyte leukaemia". J. Clin. Pathol. 51 (9): 672–5. PMID 9930071. PMC: 500904. http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=9930071.
- ^ Three episodes of acquired pure red cell aplasia restricted to pregnancy. Miller AC, Rashid RM. J Perinat Med. 2008;36(3):270-1.
- ^ Bennett CL, Luminari S, Nissenson AR, et al. (2004). "Pure red-cell aplasia and epoetin therapy". N. Engl. J. Med. 351 (14): 1403–8. doi:. PMID 15459301. http://content.nejm.org/cgi/pmidlookup?view=short&pmid=15459301&promo=ONFLNS19.
- ^ 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. PMID 17640861. http://www.haematologica.org/cgi/pmidlookup?view=long&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:. PMID 18510682. PMC: 2592349. http://dx.doi.org/10.1111/j.1365-2141.2008.07216.x.
External links
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