儿童重症噬血性淋巴组织细胞增多症免疫抑制治疗的方法与选择  

Approaches and options for immunosuppressive treatment of severe hemophagocytic lymphohistiocytosis in children

作　　者：周益平 王春霞 张育才 Zhou Yiping;Wang Chunxia;Zhang Yucai(Department of Critical Care Medicine,Shanghai Children's Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200062,China)

机构地区：[1]上海市儿童医院、上海交通大学医学院附属儿童医院重症医学科,200062

出　　处：《中国小儿急救医学》2024年第11期806-811,共6页Chinese Pediatric Emergency Medicine

基　　金：国家重点研发计划(2021YFC2701704)。

摘　　要：噬血性淋巴组织细胞增多症(HLH)/巨噬细胞活化综合征(MAS)是一种以儿童发病为主、并不少见的高炎症反应疾病。其主要的病理生理机制为T细胞和NK细胞功能降低或缺乏,导致淋巴细胞及巨噬细胞异常增殖和活化,产生细胞因子风暴和器官功能损害。主要表现为高热、血细胞减少、肝脾肿大、铁蛋白升高、肝衰竭和凝血功能障碍,病死率可超过50%。近年来,HLH/MAS在传统激素与免疫抑制剂治疗的基础上,发展出了新型免疫治疗方法。本文聚焦HLH/MAS新型免疫治疗方法的临床研究及其治疗策略。Hemophagocytic lymphohistiocytosis(HLH)/macrophage activation syndrome(MAS)are hyperinflammatory diseases that primarily affect children.The main pathophysiological mechanism involves the reduction or lack of T lymphocyte and natural killer cell function,leading to abnormal proliferation and activation of lymphocytes and macrophages,resulting in cytokine storm and organ dysfunction.The main manifestations are high fever,cytopenia,hepatosplenomegaly,elevated ferritin,liver failure,and coagulation dysfunction,with a mortality rate that can exceed 50%.In recent years,HLH/MAS has developed new immunotherapy methods based on the treatment of traditional immunosuppressive agents.This article focused on the clinical research and treatment of new immunotherapy methods.

关 键 词：噬血性淋巴组织细胞增多症 巨噬细胞活化综合征 免疫治疗 儿童 

分 类 号：R725.9[医药卫生—儿科]