Evan’s Syndrome: Case Series with Review of Literature

Abstract

Evans syndrome is a rare autoimmune disease in which an individual’s antibodies attack the body’s own red blood cells and platelets. It is characterised by simultaneous or sequential development of Immune Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) with positive Direct Antiglobulin test (DAT). AIHA can be cold, warm or mixed type depending on the type of antibody. 80% of total AIHA cases are warm AIHA. Warm AIHA is mostly caused by IgG antibody which reacts at 370 C. Warm AIHA is associated with classical case of Evan’s syndrome. Some variants of Evan’s syndrome are associated with leucocytosis. The current article discusses 3 cases of Evan’s syndrome, all presenting differently - Classical Evan’s syndrome, Evan’s syndrome associated with leucocytosis and Evan’s syndrome associated with neuropathic pain. In this case series, pathophysiology, workup done at blood centre and blood component transfusion support in patients with Evan’s syndrome has been discussed. Keywords: Evan’s syndrome, Anemia, Autoimmune