2021 Volume 58 Issue 3 Pages 240-244
Autoimmune hemolytic anemia (AIHA) is the most common type of acquired hemolytic anemia. Moreover, AIHA is an autoimmune disease in which the autoantibodies produced react with antigens on the erythrocyte surface membrane. The antigen–antibody reaction causes hemolysis, resulting in a markedly shortened erythrocyte lifespan and consequently, anemia. Primary immunodeficiency is a general term for diseases caused by congenital abnormalities of immunocompetent cells or immunocompetent molecules in the immune system, and it includes more than 430 individual diseases. Most of the diseases are caused by a single genetic abnormality. These diseases have been termed as inborn errors of immunity (IEI) in recent years. Interestingly, AIHA has been noted as the first IEI symptom. The representative IEIs that complicate AIHA are cytotoxic T lymphocyte antigen-4 (CTLA-4) deficiency and lipopolysaccharide-responsive beige-like anchor protein (LRBA) deficiency. Thus, in this paper, we review the IEIs associated with AIHA, focusing on the CTLA-4 and LRBA deficiencies.
