儿童噬血细胞综合征的分层治疗  

The stratified therapy for hemophagocytic lymphohistiocytosis in children

作　　者：李琴 杨明华 Li Qin;Yang Minghua(Department of Pediatrics,The Third Xiangya Hospital,Central South University,Hunan Clinical Research Center of Pediatric Cancer,Changsha 410013,China)

机构地区：[1]中南大学湘雅三医院儿科、湖南省儿童肿瘤性疾病临床医学研究中心,长沙410013

出　　处：《中国小儿急救医学》2024年第11期812-817,共6页Chinese Pediatric Emergency Medicine

基　　金：国家临床重点专科重大科研专项(Z2023023);湖南省科技创新领军人才项目(2022RC3077);2024年度湖南省卫生科研课题(重点课题)(20232811);2023年湖南省卫生健康高层次人才(学科带头人)。

摘　　要：噬血细胞综合征,即噬血细胞性淋巴组织细胞增多症(HLH),是一种遗传性或获得性免疫调节功能异常导致的严重炎症反应综合征。儿童HLH的病因复杂,异质性强,治疗反应及预后差异大,需要采用分层治疗。目前,HLH的分层治疗体系主要包括根据序贯器官衰竭评分分层、细胞因子分层、芦可替尼治疗反应分层等。本文总结分析上述国内外儿童HLH分层治疗的现状,希望能够更好地帮助相关专科医生规范和提升HLH的诊疗。Hemophagocytic syndrome,also known as hemophagocytic lymphohistiocytosis(HLH),represents a severe syndrome of heightened inflammatory response caused by genetic or acquired abnormalities in immune regulation.The etiology of HLH in children is complex,highly heterogeneous,and there are significant differences in treatment response and prognosis.Therefore,stratified treatment is necessary.Currently,the stratified treatment system for HLH mainly includes sequential organ failure assessment stratification,cytokine stratification,and treatment response stratification with ruxolitinib.This review summarized and analyzed the current status of stratified treatment for HLH in children both domestically and internationally,with the hope of better assisting relevant specialists in standardizing and improving the diagnosis and treatment of HLH.

关 键 词：儿童 噬血细胞综合征 分层治疗 

分 类 号：R725.9[医药卫生—儿科]