噬血细胞综合征30例临床分析  

A clinical analysis of 30 patients with hemophagocytic syndrome

作　　者：李国辉[1] 范玉贞[2] 郝淼旺[1] 黄斯勇[1] 刘利[1] 杨健[1] 梁英民[1] 

机构地区：[1]第四军医大学唐都医院血液科,西安710038 [2]第四军医大学唐都医院中医科,西安710038

出　　处：《临床血液学杂志》2011年第3期276-278,共3页Journal of Clinical Hematology

摘　　要：目的:探讨噬血细胞综合征(HPS)的临床特点、诊断、治疗以及预后的危险因素。方法:对我院30例HPS患者的病因、临床表现、实验室检查指标、治疗方案及临床转归进行回顾性分析。结果:30例HPS病因以感染最多见(30%),其中EB病毒感染达20%,然而病因不明者也高达56.7%。HPS临床表现为持续高热(100%)、脾肿大(93.3%)、全血细胞减少(83.3%)、乳酸脱氢酶(100%)及血清铁蛋白(100%)升高。肝功能损害(90%)及心肌酶谱(60%)升高也较为常见。30例经治HPS患者30d、100d、1年的生存率分别为36.7%、23.3%、10.0%。其中7例给予包含VP16的化疗方案,30d、100d、1年的生存率分别为85.1%、71.4%、42.9%。结论:HPS可由多种病因所致,EB病毒最为常见,临床表现多样。发热、血清铁蛋白、乳酸脱氢酶升高在诊断中的灵敏度较高。包含VP16的化疗方案是有效的治疗方案。Objective：To investigate the clinical features,diagnosis and treatment of hemophagocytic syndrome（HPS） and possible factors affecting prognosis.Method：A retrospective study was carried out to analyze the causes,clinical features,laboratory findings,treatment and clinical outcomes of 30 patients with HPS.Result：Infection was the most common underlying disease（30%）,in which EB virus-associated HPS was predominant,accounting for 20%.However,the cause of HPS was unknown in 56.7% patients.HPS was clinically characterized by prolonged fever（100%）,splenomegaly（93.3%）,Cytopenias（83.3%） and other features including elevated Serum ferritin（100%） and lactate dehydrogenase（100%）.Liver dysfunction（90%） and elevated cardiac enzyme levels（60%） were also frequently observed.Overall survival at 30 days,100 days and 1 years was 36.7%,23.3%,10% respectively for all the patients,while the rate was 85.1%,71.4%,42.9% for those using chemotherapy regimens containing etoposide.Conclusion：There are various underlying diseases and clinical manifestations for HPS.Prolonged fever,elevated Serum ferritin and lactate dehydrogenase are sensitive diagnostic indicators of HPS.We conclude that early administration of etoposide is effective in treating patients with HPS.

关 键 词：噬血细胞综合征 治疗 依托泊苷 预后 

分 类 号：R551[医药卫生—血液循环系统疾病]