噬血细胞综合征的病因和诊断  

Hemophagocytic lymphohistiocytosis:pathogenesis and diagnosis

作　　者：吴婷婷 唐亮 胡豫[1] WU Tingting;TANG Liang;HU Yu(Institute 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病学研究所,武汉430022

出　　处：《临床血液学杂志》2024年第9期677-682,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)即噬血细胞综合征,是一种罕见的危及生命的免疫失调综合征,以细胞毒性T细胞、自然杀伤细胞和巨噬细胞过度活化为特征,引起细胞因子风暴,导致一系列的临床症状、器官损伤甚至死亡。HLH可分为原发性和继发性,前者存在致病基因(淋巴细胞毒性缺陷、炎症小体异常活化等),后者可由多种因素触发(感染、肿瘤、自身免疫疾病、医源性因素等)。早期敏锐的识别和及时治疗对提高生存率至关重要,目前的诊断使用最广泛的是HLH-2004标准和Hscore积分。本文系统讨论和更新了HLH的病因、实验室相关检查以及诊断方法。Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening hyperinflammatory syndrome characterized by the overactivation of cytotoxic T cells,natural killer cells,and macrophages arising in cytokine storms,a range of clinical symptoms,organ damage,and even death.HLH can be divided into primary and secondary,with the former having susceptibility genes(lymphocyte cytotoxicity defects,abnormalities of inflammasome activation,etc.)and the latter being triggered by a variety of factors(infections,malignancy,iatrogenic factors,etc.).Prompt diagnosis and treatment are essential to improve survival.The HLH-2004 criteria and the Hscore are two widely used diagnostic tools in clinical practice.In this review,we discussed the etiology,laboratory tests,and diagnosis of HLH.

关 键 词：噬血细胞性淋巴组织细胞增生症 噬血细胞综合征 巨噬细胞活化综合征 免疫失调 

分 类 号：R55[医药卫生—血液循环系统疾病]