摘要
噬血细胞性淋巴组织细胞增生症(HLH)也称为噬血细胞综合征(HPS),是一种异质性综合征,可以导致严重的对生命有威胁的过度炎性反应。从病原学来看,HLH可以分为原发和继发两种类型。前者可以由基因和免疫缺陷引起,后者可以由感染、自身免疫性疾病或者恶性肿瘤等许多因素诱发。其最常见的临床症状为持续性高热、脾大、外周血细胞减少,一些严重患者会同时伴随中枢神经系统的累及。HLH预后很差,如果不给予合理的治疗通常会导致死亡。本文通过对HLH的分类、病因、临床表现、诊断以及实验室检测结果进行全面综述,以期对该病的诊断治疗提供帮助。
Hemoph-agocytic lymphohistiocytosis（HLH）,also known as hemophagocytic syndrome（HPS）,is a heterogenic syndrome,which can lead to an acute,life-threatening,excessive inflammatory reaction. Depending on the etiology,HLH can be divided into primary and secondary forms. Among the first forms,the former can be caused by genes and immunodeficiency. The latter can be triggered by infections, autoimmune diseases, or malignancies. The most common symptoms of HLH are unremitting fever,splenomegaly,and peripheral blood cytopenia,some severely ill patients present with central nervous system involvement. Prognosis in HLH is very serious which often leads to death without appropriate treatment. In this paper,the classification,etiology,clinical manifestation,diagnosis and laboratory results of HLH were given a comprehensive review,in order to help the diagnosis and treatment of the disease.
出处
《中国城乡企业卫生》
2017年第9期19-22,共4页
Chinese Journal of Urban and Rural Enterprise Hygiene
关键词
噬血细胞综合征
病因
检测结果
研究进展
Hemoph-agocytic lymphohistiocytosis
The etiology
Laboratory results
Research progress
