噬血细胞综合征研究进展  

Research advances in hemophagocytic syndrome

作　　者：吴小艳[1] 王琳[1] 陈鸿博 Wu Xiaoyan;Wang Lin;Chen Hongbo(Department of Pediatrics,Union Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,China)

机构地区：[1]华中科技大学同济医学院附属协和医院儿科,武汉430022

出　　处：《中华实用儿科临床杂志》2020年第15期1125-1129,共5页Chinese Journal of Applied Clinical Pediatrics

摘　　要：噬血细胞综合征(HPS)起病急,病情进展迅速,是一种免疫介导的可危及生命的疾病。该病多见于儿童,病因、临床表现复杂多样,治疗难度大,病死率高。近年来,随着HPS诊断及治疗水平的提高,此类患儿的预后已得到很大改善,但仍存在不少难治性病例。现就HPS的分类、发病机制、诊断及治疗的研究进展进行综述,以期提高对该病的认识。Hemophagocytic syndrome(HPS)is a serious rapid—developing disease with high mortality,which is more commonly seen in children.The etiology and clinical manifestation of HPS are heterogenous.For the past few years,the understanding of HPS is greatly improved with advances in diagnosis and treatment.However,there are still a lot of refractory cases.This article describes the etiology,pathogenesis,diagnosis,treatment of HPS,in order to enhance understanding of this disease.

关 键 词：噬血细胞综合征 分类 发病机制 诊断 治疗 

分 类 号：R725.5[医药卫生—儿科]