Pure red cell aplasia

Pure Red Cell Aplasia (English: aplasia of the red blood cells PRCA) or isolated aplastic anemia and pure red cell aplasia is anemia, which is caused by the destruction or lack of formation of erythroblasts in the bone marrow. It can be congenital or acquired. Today the congenital PRCA is mostly known as Diamond Blackfan syndrome ( qv) called during the abbreviation PRCA is mostly used for the acquired form.

Causes

Acquired PRCA is considered by many clinicians as an autoimmune disease. They often coexist with other autoimmune diseases such as lupus erythematosus, or thymoma. PRCA addition may occur as a result of viral infection, such as Parvovirus B19 (especially in children), HIV, herpes simplex virus as well as viral hepatitis. PRCA also occurs frequently in connection with immune-mediated bone marrow disorders, such as aplastic anemia and T- cell leukemia LGL. Accordingly, autoantibodies, autoimmune T cells or drugs (eg, erythropoietin, or mycophenolate mofetil ) suggests, in turn, often lead to the formation of autoantibodies as causes.

PRCA may also occur as a complication of immunohematological graft-versus- host disease after stem cell transplantation. As with many blood diseases, the cause is in a number of cases, however, unknown ( idiopathic).

Treatment

PRCA patients respond well to treatment with immunosuppressive drugs ( eg cyclosporin or rituximab), which confirms the hypothesis that this is an autoimmune disease.

Complications

The complications occurring in the course of the disease are bleeding and often the transition in aplastic anemia. It is believed that PRCA occurs as a symptom of (possibly not yet detected ) aplastic anemia.

History

The first description of acquired PRCA was made by Paul Kaznelson in 1922. The first description of congenital PRCA goes back to Louis and Kenneth Blackfan Diamond.