噬血细胞综合征21例临床分析

目的探讨噬血细胞综合征（HPS）的临床特点。方法回顾性分析21例噬血细胞综合征患者的病因、临床表现、实验室检查指标、治疗方案及治疗转归情况。结果21例HPS中感染12例（57．1％），其中EBV感染6例，血液系统恶性肿瘤3例（14．2％），经过HLH-2004方案为基础的治疗后，死亡9例（42．8％），其中早期死亡6例，3个月后死亡3例。结论HPS病因多样，预后差，需要早诊断，早治疗。

Clinical analysis of 21 patients hemophagocytic syndrome

Objective To investigate the clinical features of hemophagocytic syndrome （HPS）. Methods A retrospective study was carried out to analyze the causes, clinical features, laboratory findings, treatment and clinical out- comes of 21 patients with HPS. Results Among 21 patients with HPS, 12 （57. 1%） patients had definite infection, 6 patients had EB virus infection, 7 （17.5%） were malignancy-associated, after the combined treatment with HLH-2004 criteria, mortality was 42.8% （9/21）. Conclusion Hemophagocytic syndrome has complicated etiology and pathogene- sis, and poor prognosis. Doctors should grasp the clinical features of HPS, diagnose and treat it as early as possible.