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Evans syndrome (ES) is a rare disorder in which the immune system produces antibodies that accidentally destroy red blood cells and platelets. The diagnosis of ES is made by the simultaneous presence of autoimmune hemolytic anemia and immune thrombocytopenia. However, the diagnosis of ES associated with hematologic malignancies necessitates a comprehensive evaluation of clinical course, clinical findings, and laboratory test results. Hematological malignancies are the most significant underlying diseases factors impacting ES and the prognosis, but there is no established standard therapy for autoimmune cytopenia associated with hematological malignancies. In ES associated with hematologic malignancies, achieving remission of the underlying disease seems to be the key to long-term remission.