A rare paraneoplastic condition in Hodgkin lymphoma: Evans syndrome and literature review.

Abstract

Evans syndrome (ES) is a spectrum of diseases in which the combination of autoimmune hemolytic anemia and immune thrombocytopenia or sometimes neutropenia. ES has been accepted usually as an idiopathic condition, but it may be secondary. The coexistence of autoimmune cytopenias and Hodgkin lymphoma (HL) is rarely observed and the rate of ES in HL patients is not clear. Here we describe a 56-year-old male patient who presented with ES and was diagnosed with HL. After corticosteroids, intravenous immunoglobulin (IVIG) and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment, immune cytopenias were completely resolved. The literature is also reviewed and we found 16 cases in which HL and ES coexist. Although AIHA and immune thrombocytopenia usually develop simultaneously, they rarely occur at different times. Many aspects of the pathogenesis are unknown, but it is thought to be a complex immunological background. Corticosteroids and/or IVIG are the most commonly used first-choice drugs in the initial treatment of ES. Response rates to treatment are variable and response to treatment may be poor, particularly with underlying conditions. If detected, the underlying lymphoma should be treated.