噬血细胞性淋巴组织细胞增生症诊断和治疗的进展  

Progress in diagnosis and treatment of hemophagocytic lymphohistiocytosis

作　　者：吴聪 郭涛[1] WU Cong;GUO Tao(Department of Hematology,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血液科,武汉430022

出　　处：《临床血液学杂志》2025年第1期41-46,共6页Journal of Clinical Hematology

摘　　要：噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH),又称噬血细胞综合征(hemophagocytic syndrome,HPS),是一种以免疫系统过度激活为特征的免疫失调综合征,由于T淋巴细胞、巨噬细胞和自然杀伤(NK)细胞无法控制的过度激活从而产生大量的细胞因子,进而引发多器官功能损伤、衰竭甚至死亡。目前,多部国内外指南或共识对HLH的诊断治疗和监测提出了原则性和针对性的指导意见。本文将结合目前国内外的指南、专家共识及现有的研究证据,对HLH诊断和治疗及其进展作一总结。Hemophagocytic lymphohistiocytosis(HLH),also known as hemophagocytic syndrome(HPS),is an immunological disorder characterized by excessive immune activation.This condition arises from uncontrolled overactivation of T lymphocytes,macrophages,and natural killer(NK)cells,leading to a massive production of cytokines and subsequent multi-organ functional damage,failure,and even death.Currently,several domestic and international guidelines and consensus statements have provided principled and targeted guidance on the diagnosis,treatment,and monitoring of HLH.This article will summarize the progress in diagnosis and treatment of HLH by integrating current guidelines,expert consensus,and existing research evidence from both domestic and international sources.

关 键 词：噬血细胞性淋巴组织细胞增生症 诊断 治疗 进展 

分 类 号：R55[医药卫生—血液循环系统疾病]