噬血细胞综合征21例临床分析

摘要

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 outcomes 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.%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.