[Chinese expert consensus on the diagnosis and treatment of Evans syndrome (2024)].

Abstract

Evans syndrome (ES) is a rare autoimmune disorder characterized by the presence of at least two autoimmune cytopenias (AIC), including immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), and autoimmune neutropenia (AIN). Secondary ES accounts for 21%-50% of cases. ES is characterized by recurrent relapses, serious complications such as thrombosis and infection, and high mortality. The management of ES is highly heterogeneous, necessitating treatment for AIC, the primary disease, as well as associated complications. However, there remains a lack of prospective evidence, randomized clinical trials for ES. To standardize the diagnosis and management of ES in China effectively, this consensus was developed by the Red Blood Cell Diseases (Anemia) Group under the Chinese Society of Hematology within the Chinese Medical Association. It aims to provide valuable guidance for diagnosing and managing ES in clinical practice based on international consensus and comprehensive review of both domestic and international literature.