Hereditary Aspects of Autoimmune Hemolytic Anemia; a Retrospective Analysis

Summary. A family with three siblings involved in autoimmune hemolytic anemia is presented. One sibling had an idiopathic variety of the Evans' syndrome type of autoimmune hemolytic anemia. The other two siblings had secondary forms of autoimmune hemolytic anemia, with plasma cell myeloma present in one and systemic lupus erythematosus in the other. A retrospective analysis of the family histories of forty‐four patients with idiopathic autoimmune hemolytic anemia revealed that 20 % had family members involved in clinically detectable autoimmune disease. The hypothesis is presented that autoimmune hemolytic anemia results from a fundamental aberration of the immune apparatus which prevents the establishment of a normal immune homeostatic mechanism. This abnormality appears to be transmitted on a familial basis, and is not primarily related to erythrocyte immune homeostasis. As such, the concept of ‘idiopathic’ autoimmune hemolytic anemia may be illusory and simply represent a partial diagnosis of a more generalized medical disorder.