Pure red cell aplasia

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 (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. The condition has been first described by Paul Kaznelson in 1922.^[1]

Causes

Causes include:

• Autoimmune disease.

• Thymoma^[2] or

• Viral infections such as HIV, herpes, parvovirus B19 (Fifth disease),^[3] or hepatitis.^{[citation needed]}

• Lymphoproliferative. Association of pure red cell aplasia with T 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, e.g., erythropoietin^[6] or mycophenolic acid.^[7]

• Congenital. The term "hereditary pure red cell aplasia" has been used to refer to Diamond-Blackfan anemia.^[8]

Treatment

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

It has also been shown to respond to treatments with Rituxan.

Popular culture

In an episode of House, an athlete is diagnosed with pure red cell aplasia as a manifestation of a thymoma.

See also

• Diamond-Blackfan anemia (genetic red cell aplasia)
• aplastic anemia (aplasia affecting other bone marrow cells as well)

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. http://www.haematologica.org/cgi/pmidlookup?view=long&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". Clin Transplant 14 (6): 586–91. doi:10.1034/j.1399-0012.2000.140612.x. 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. 
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. http://jcp.bmj.com/cgi/pmidlookup?view=long&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. ^ 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:10.1056/NEJMoa040528. PMID 15459301. http://content.nejm.org/cgi/pmidlookup?view=short&pmid=15459301&promo=ONFLNS19. 
7. ^ Petrochko C (2009). "FDA Strengthens Warning on Transplant Drug." Medpage Today. 14 August 2009. Accessed on 19 August 2009.
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. http://www.haematologica.org/cgi/pmidlookup?view=long&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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2592349. 

External links

• Aplastic Anemia & MDS International Foundation