Pure red cell aplasia
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(Redirected from Constitutional red blood cell aplasia)
Pure red cell aplasia | |
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Classification and external resources | |
Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
ICD-10 | D60 |
ICD-9-CM | 284.8 |
DiseasesDB | 29063 |
eMedicine | med/1967 |
Patient UK | Pure red cell aplasia |
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:
- 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 such as mycophenolic acid[6] or erythropoietin.[citation needed]
- Congenital. The term "hereditary pure red cell aplasia" has been used to refer to Diamond-Blackfan anemia.[7]
Treatment
PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as cyclosporine in many patients,[8] though this approach is not without risk.[9]
It has also been shown to respond to treatments with Rituxan and Tacrolimus.
See also
- Diamond-Blackfan anemia (genetic red cell aplasia)
- Aplastic anemia (aplasia affecting other bone marrow cells as well)
References
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- ↑ Petrochko C (2009). "FDA Strengthens Warning on Transplant Drug." Medpage Today. 14 August 2009. Accessed on 19 August 2009.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 105650
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