Adult Evans' Syndrome

Abstract

Evans syndrome (ES) is a rare immune disorder defined as the simultaneous or sequential occurrence in a single patient of immune thrombocytopenia (ITP) and warm autoimmune hemolytic anemia (wAIHA) ± autoimmune neutropenia (AIN). ES represents approximately 5% to 10% of all wAIHA and 2%-5% of all ITP cases in adults and its mortality rate is high. When ITP and wAIHA occurred concomitantly, other differential diagnoses must be ruled out. ES can be primary or secondary and isolated or associated with another underlying disorder and secondary ES. The management of ES is mostly empirical with a low level of evidence. This review reports some new insights on this rare disease and provides some practical tools for the diagnosis and management of adult ES.

Keywords: Autoimmune cytopenia; Autoimmune hemolytic anemia; Autoimmune neutropenia; Evans syndrome; Immune thrombocytopenic purpura; Primary immunodeficiencies.