Pure red cell aplasia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia

Overview

Acquired 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.

Causes

Pure red cell aplasia is regarded as an autoimmune disease. It may also be a manifestation of thymoma. It may also be as a result of viral infections such as HIV, herpes, parvovirus B19 (Fifth disease), or hepatitis. Association of pure red cell aplasia with T large granular lymphocyte leukemia is also well recognized, especially in China (http://jcp.bmj.com/cgi/content/abstract/51/9/672). Many cases of PRCA are considered idiopathic in that there is no discernable cause detected.

It can be associated with the administration of erythropoietin.

Drug Side Effect

• Mycophenolate

Treatment

PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin. It has also been also been shown to respond to treatments with Rituxan.

Contraindicated medications

Pure red cell aplasia is considered an absolute contraindication to the use of the following medications:

• Darbepoetin Alfa
• Methoxy polyethylene glycol-epoetin beta

See also

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

External links

• Aplastic Anemia & MDS International Foundation
• NEJM