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自身免疫性溶血性贫血是由各种原因导致机体免疫功能紊乱，产生可与红细胞自身抗原反应的自身抗体和(或)补体，导致红细胞破坏增多并超过骨髓造血的代偿能力。儿童多急性起病，临床表现与发病机制相关。糖皮质激素作为该病的一线治疗，多数患儿对其反应良好。部分患儿因存在激素依赖，激素耐药，或因抗体类型不同导致病程反复，常需采取二线治疗方案，如脾切除、免疫抑制剂等。该文就近年来儿童自身免疫性溶血性贫血的治疗进展做一综述。“,”Autoimmune hemolytic anemia is a disorder of immune function caused by various reasons, by produceing autoantibody or complement which can react with erythrocyte autoantigen, increasing the destruction of red blood cell and beyond the compensatory ability of bone marrow hematopoiesis.Children usually have acute onset and the clinical manifestations are related to the pathogenesis.As the first-line treatment of glucocorticoids, Most children respond well to glucocorticoids.Some children suffer from steroid dependence and resistance; or recurrence due to different types of antibodies, often requiring second-line treatment, such as splenectomy, immunosuppressive, etc.This article reviews the recent progress in the treatment of children with autoimmune hemolytic anemia.