儿童噬血细胞综合征及血液净化技术治疗的研究进展  

Research progress on blood purification technology in children with hemophagocytic syndrome

作　　者：罗南都 杜作晨(综述) 陈艳(审校) Luo Nandu;Du Zuochen;Chen Yan(Department of Pediatric Medicine,Affiliated Hospital of Zunyi Medical University,Guizhou Province,Zunyi 563000,China)

机构地区：[1]遵义医科大学附属医院小儿内科,贵州遵义563000

出　　处：《疑难病杂志》2023年第12期1336-1339,共4页Chinese Journal of Difficult and Complicated Cases

基　　金：贵州省科技计划项目(黔科合基础-ZK[2022]一般641)。

摘　　要：噬血细胞综合征是以免疫细胞异常活化产生高细胞因子血症和免疫介导的多器官系统损伤综合征。基于化学免疫疗法的应用有效降低了总体病死率,但早期合并多脏器障碍的患者死亡率高。近年来血液净化的早期应用不仅可快速降低免疫炎性介质,而且有效保护和恢复器官功能,对改善患者的预后具有重要临床意义。未来需要更多大样本血液净化治疗的临床研究形成标准化治疗方案,使患者最大程度受益。文章从噬血细胞淋巴组织细胞增生症(HLH)病因、发病机制及近年来血液净化技术应用进行综述,以期为临床诊治HLH提供科学参考依据。Hemophagocytic lymphohistiocytosis is a syndrome of multiple organ system injury induced by hypercytokinemia and immune-mediated abnormal activation of immune cells.The application of chemoimmunotherapy has effectively reduced the overall mortality rate,but the mortality rate of patients with early multiple organ dysfunction is still high.In recent years,the early application of blood purification has not only rapidly reduced immune inflammatory mediators,but also effectively protected and restored organ function,which has important clinical significance for improving the prognosis of patients.In the future,more clinical studies on large-scale blood purification therapy are needed to form standardized treatment plans,in order to maximize the benefits for patients.This article reviews the etiology,pathogenesis,and application of blood purification technology in recent years of HLH,in order to provide scientific reference for clinical diagnosis and treatment of HLH.

关 键 词：噬血细胞淋巴组织细胞增生症 发病机制 血液净化 化学免疫疗法 

分 类 号：R552[医药卫生—血液循环系统疾病]