147例噬血细胞综合征病因及预后分析  

Causes and prognosis analysis of 147patients with hemophagocytic syndrome

作　　者：李硕[1] 王晶石[1] 王旖旎[1] 张嘉[1] 汤然[1] 王昭[1] 

机构地区：[1]首都医科大学附属北京友谊医院血液科,北京100050

出　　处：《临床血液学杂志》2014年第4期559-563,569,共6页Journal of Clinical Hematology

基　　金：国家自然科学基金(No:81270653);首都医学发展科研基金(No:2009-1032);北京自然科学基金(No:7132087);北京市科技计划首都临床特色应用研究(No:D101100050010005);中华医学会关于临床医学科研专项资金(No:11010020246)

摘　　要：目的:探讨噬血细胞综合征(HLH)病因及预后的相关特点。方法:回顾性分析我院147例HLH患者的病因及预后,计算各种病因HLH的发生率及生存率。结果:147例HLH病因以淋巴瘤最为多见(34.01%);其次为感染(30.61%),其中EB病毒感染高达20.41%;自身免疫性疾病占12.93%;原发性HLH占9.52%;其余为其他病因或原因不明。147例经治HLH患者1、3、6、12个月的生存率分别为69%、52%、45%、39%,各病因组生存曲线不同,自身免疫性疾病组生存率明显高于其他3组。结论:HLH可由多种病因所致,不同病因的HLH发生率及生存率各不相同。早期明确病因对HLH预后具有重要意义。Objective：To explore the etiology and prognosis characteristics of hemophagocytic lymphohistiocytosis（HLH）.Method：We retrospectively analyzed the etiology and prognosis of 147patients with HLH in our hospital,and calculated the incidence and survival of various etiologies in HLH patients.Result：Lymphoma（34.01%）was the most common cause in 147cases of HLH,while infection（30.61%）was the next.Epstein-Barr virus infection accounted for about 20.41%,rheumatic diseases accounted for 12.93%,primary hemophagocytic syndrome accounted for 9.52%,and the remaining for other causes or reasons unknown.Overall survival at 1,3,6,12months was 69%,52%,45%and 39%respectively for all the patients.The survival curves of different groups were various,and the survival of rheumatoid group was markedly higher than those in the other three groups.Conclusion：HLH can be caused by a variety of pathogenesis,and the HLH with different pathogenesis have different incidence and survival.Early identify of the HLH pathogenesis is critical to prognosis of this disease.

关 键 词：噬血细胞综合征 病因 预后 

分 类 号：R552[医药卫生—血液循环系统疾病]