免疫治疗相关噬血细胞综合征诊疗进展

Progress in the diagnosis and treatment of immunotherapy-related hemophagocytic lymphohistiocytosis

噬血细胞综合征又称噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH),是一种罕见的、危及生命的临床综合征。由于淋巴细胞及巨噬细胞失控性增殖并分泌大量细胞因子,导致多器官、多系统受累。免疫治疗是肿瘤和自身免疫性疾病治疗的重要组成部分,改变了传统治疗模式,提高了患者的生存率。随着免疫治疗的广泛应用,免疫治疗相关HLH因其病情凶险、进展迅速引起研究者关注。目前,这一类HLH多以病例报告形式出现,缺乏统一的诊疗标准。本文将从免疫治疗相关HLH的发病机制、诊断及治疗进展展开综述,以提高医务工作者对此类疾病的认知。

Hemophagocytic syndromes also known as hemophagocytic lymphohistiocytosis(HLH),is a rare and life-threatening clinical syndrome.HLH is caused by the proliferation and activation of lymphocytes and macrophages,which secrete cytokines.The excessive secretion of cytokines leads to damage of multiple organs and systems.Immunotherapy is an important part of the treatment of tumors and autoimmune diseases,which has changed the traditional treatment mode and improved the survival rate of patients.Immunotherapy-related HLH has attracted the attention of researchers due to its severity and rapid progression.At present,this type of HLH was usually reported as case,lacking uniform diagnosis and treatment standards.This paper will summarize the pathogenesis,diagnosis and treatment progress of immunotherapy-related HLH in order to improve the knowledge of medical practitioners about this disease.