PURPOSE: The outcome of autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES) in adults is not well-known and their management is mainly based on empirical data. CURRENT KNOWLEDGE AND KEY POINTS: The main characteristics of both AIHA and ES are reviewed in this article, based on the data available in the literature and on our experience. Although controlled studies and evidence-based data are lacking, some recommendations regarding the minimal workup useful to search for an underlying disease can be made as well as proposals for a better therapeutic strategy of both disorders. FUTURE PROSPECTS AND PROJECTS: A better knowledge of the mechanisms triggering AIHA and ES as well as therapeutic trials are needed in order to better stratify treatment taking into account the promising efficacy of rituximab.
