Autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) encompasses a group of diseases mostly mediated by autoantibodies against erythrocyte surface antigens. This chapter will address the individual subtypes of AIHA with respect to epidemiology, pathogenesis, diagnostic procedure, clinical features, and therapy. In warm forms, autoantibody-mediated hemolysis is mainly extravascular in the spleen. The complement system is involved in pathogenesis in about 50% of cases of warm-antibody mediated AIHA and probably in all cases of cold agglutinin disease (CAD). We will highlight the importance of diagnosing the subtype of AIHA and identifying any underlying or associated disease. Corticosteroids remain the backbone of first-line therapy in warm-AIHA, while rituximab is now the most important approach in the second line. In contrast, CAD is treated by targeting the underlying B-cell bone marrow lymphoproliferative disorder or the classical complement pathway. The other, still rarer subtypes of AIHA will also be addressed, and future perspectives will be briefly discussed.