58例成人噬血细胞综合征回顾性临床和预后分析  

Retrospective analysis of clinics and the prognosis of 58 adult patients with hemophagocytic syndrome in a single center

作　　者：李菲[1] 李蒲[1] 张荣艳[1] 纪德香[1] 胥倩[1] 杨赣萍[1] 黄先豹[1] 魏艳淋[1] 黄瑞滨[1] 陈国安[1] 

机构地区：[1]南昌大学第一附属医院血液科,南昌市330006

出　　处：《中国肿瘤临床》2014年第5期324-327,共4页Chinese Journal of Clinical Oncology

基　　金：江西省教育厅科学技术研究计划项目(编号:GJJ10061);江西省卫生厅课题计划项目(编号:20091047);南昌大学校基金项目(编号:2008)资助~~

摘　　要：目的:提高对成人噬血细胞综合征(hemophagocytic syndrome,HPS)的认识,减少误诊。方法:回顾性分析南昌大学第一附属医院58例成人HPS患者的临床特点和生存资料。结果:本组患者以发热(100%)和脾肿大(89.7%)最常见。实验室指标以血清铁蛋白≥500μg/L(100%)、两系以上血细胞降低(96.6%)最为常见。感染组和风湿免疫组患者预后较好(中位生存时间为未达到和190 d),肿瘤相关组HPS预后最差(中位生存时间仅为30 d)。单因素分析示Fbg<1.5 g/L、PLT<40×109/L和LDH≥2 000 U/L的患者预后明显更差(P均<0.001),多因素分析显示PLT<40×109/L是影响本组患者生存时间的独立不良因素(P=0.011)。结论:HPS临床表现错综复杂,病因多种多样;Fbg<1.5 g/L、PLT<40×109/L和LDH≥2 000 U/L患者预后不佳,需尽早行系统方案治疗。Objective: This study aimed to achieve the early diagnosis and active treatment of adult hemophagocytic syndrome (HPS) and investigate the clinical characteristics and prognostic factors of this syndrome. Methods: A single-center retrospective analysis was performed to analyze clinical characteristics, laboratory findings, and survival data. Results: In 58 patients, the most common clinical manifestations were fever (100%) and splenomegaly (89.7%). The most common laboratory parameters were serum ferritin 500 g/L (100%) and peripheral cytopenia in two or more lineages (96.6%). platelet count, fibrinogen, and lactate dehydrogenase in the death group were significantly lower than in the survival group (P=-0.000, 0.001, and 0.000). Survival analysis results showed that infections in the rheu- matological group exhibited good prognosis [the overall survival (OS) time was not reached in 190 d]. Patients with unexplained causes had moderate prognosis (OS time was 60 d); tumor-associated lIPS patients had poor prognosis (the OS time was only 30 d). Univariate analysis results showed that patients with Fbg〈l.5 g/L, PLT〈40×10 9/L, and LDH ≥ 2000 U/L also exhibited poor prognosis (P=-0.000). Multivariate analysis results showed that PLT〈40 ≥ 10 9/L was an independent adverse factor (HR=6.472, 95% CI: 1.526-26.065, P=-0.011). Conclusion: HPS exhibits complex clinical manifestations and varied etiology. Patients with infection and rheumatism-related HPS had good prognosiss compared with those manifesting tumor-associated HPS. Fbg〈1.5 g/L, PLT〈40 × 109/L, and LDH≥2 000 U/L were the univariate factors that affected the survival time of patients. PLT〈40× 10 9/L is an independent adverse factor. These patients need systemic treatments as early as possible.

关 键 词：噬血细胞综合征 临床表现 实验室检查预后 

分 类 号：R55[医药卫生—血液循环系统疾病]