Autoimmune hemolytic anemia (AIHA) is a rare type of anemia that makes less than 5% of all anemias. It is an acquired hemolytic anemia characterized by antibody mediated destruction of red blood cells as a primary process, or secondary due to autoimmune or lymphoproliferative disorders. AIHA is classified as warm, cold, mixed, or druginduced. Laboratory findings include increased reticulocyte count, elevated bilirubin, LDH and decreased haptoglobin. AIHA is diagnosed by a direct antiglobulin (Coombs) test. The treatment is directed by the cause of disease and the type of antibody. Warm AIHA is mediated by IgG antibodies and treated primarily by corticosteroids. Cold AIHA, i.e. cold agglutinin disease, is mostly mediated by IgM antibodies, and supportive treatment (warming) may suffice. Rituximab is an option in both entities, with addition of chemotherapy if a lymphoproliferative disease is present. Drug induced AIHA is caused by different mechanisms and generally withdraws with the drug discontinuation. In contrast to other anemias, administration of transfusion is exceptional and is reserved only for severe symptomatic AIHA.

Key words:
anemia, hemolytic, autoimmune; autoantibodies; Coombs test; glucocorticoids-therapeutic use; rituximab