Autoimmune Hemolytic Anemia:Focusing on Therapy According to Classification

Abstract

AutoImmune Hemolytic Anemia (AIHA) is a rare disease characterized by premature destruction of the red blood cells due to autoantibodies produced against erythrocyte antigens. Early destruction shortens the lifespan of the erythrocytes and causes anemia, the hallmark of the disease. It is a heterogeneous disease so the clinical presentation is diverse from a mild asymptomatic anemia to a refractory disease associated with mortality and morbidity. The different classifications are constituted to determine the prognosis and appropriate treatment strategy. It can be classified according the absence or presence of an underlying cause as primary or secondary. Another classification in AIHA is according to the type and thermal activity of the antibody as Cold Agglutinin Disease (CAD) or warm AIHA. In CAD, rituximab monotherapy or combinations is the treatment of choice. In the warm AIHA, the steroid should be preferred as a first line therapy. In the steroid refractory or relapsed patients, rituximab or splenectomy can be chosen according to the patient’s characteristics and the experience of the doctor. Since treatment mainly depends on the subtype of the disease differential diagnosis between the subtypes becomes important. This review will be focused on the classification and the treatment of AIHAs according to subtypes.