Evans Syndrome - The Diagnosis of Exclusion

Evans Syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. We present a case of woman aged 65 years presenting with Evans Syndrome. This is an uncommon condition where patient presents with combination (either simultaneously or sequentially) of immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) with the etiology remaining unknown. This condition generally runs a chronic course and needs life long followup and vigilance. It is characterised by frequent exacerbations and remissions. No sex predilection is known and Evans Syndrome has been described in all ethnic groups and at all ages. Due to hemolytic anemia and thrombocytopenia with recurrence there is more morbidity. The mortality results in severe cases especially in elderly individuals due to heart failure. First-line therapy is usually corticosteroids.