噬血细胞综合征的病因和诊断
Hemophagocytic lymphohistiocytosis:pathogenesis and diagnosis
吴婷婷 1唐亮 1胡豫1
作者信息
- 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)
- 折叠
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.
Abstract
Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening hyperinflammatory syn-drome characterized by the overactivation of cytotoxic T cells,natural killer cells,and macrophages arising in cy-tokine 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 in-flammasome activation,etc.)and the latter being triggered by a variety of factors(infections,malignancy,iatro-genic 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Key words
hemophagocytic lymphohistiocytosis/hemophagocytic syndrome/macrophage activation syn-drome/immune hyperactivation引用本文复制引用
出版年
2024
NETL
NSTL
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