Evan's Syndrome - A Case Series

The Evans syndrome is a rare autoimmune disease and occurs when an individual's antibodies attack the blood and platelets in the body. It is characterized by the simultaneous or sequential development of Immune Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) with a positive Direct Antiglobulin Test (DAT). The type of antibody determines whether AIHA is cold, warm, or mixed. Approximately 80% of AIHA cases are warm AIHA. Warm AIHA is mostly caused by IgG antibody which reacts at 37C. Typically, warm AIHA occurs with Evans syndrome. Evans syndrome can be associated with leucocytosis in some variants. Three cases of Evans syndrome are presented here, which can be classified as 1. classical Evans syndrome, 2. Evans syndrome with leucocytosis and 3. Evans syndrome associated with neuropathic pain. In this case series, pathophysiology, workup done at blood centre and blood component transfusion support in patients with EVANS syndrome has been discussed.