61例EBV相关噬血细胞性淋巴组织细胞增多症患者的疗效及预后分析  

Treatment outcomes and prognostic analysis of 61 Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

作　　者：曾祥宗[1] 魏娜[1] 王旖旎[1] 王晶石[1] 张嘉[1] 吴林[1] 黄文秋[1] 高卓[1] 裴瑞君 陈建行[1] 金志丽[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,100050

出　　处：《中华血液学杂志》2015年第6期507-510,共4页Chinese Journal of Hematology

基　　金：国家自然科学基金（81270653）;国家自然科学基金青年科学基金（81401627）;北京自然科学基金（7132087）;北京市科技计划首都市民健康项目培育（Z131100006813041）;北京市优秀人才资助项目

摘　　要：目的 通过对EBV相关噬血细胞性淋巴组织细胞增多症（EBV-HLH）患者的疗效及生存状况的分析,探讨影响EBV-HLH患者预后的危险因素.方法 对2008年1月至2014年7月诊治的61例EBV-HLH患者的临床资料进行回顾性分析,建立COX模型对可能影响患者预后的相关危险因素进行单因素和多因素分析.结果 同期诊治的HLH患者共246例,伴EBV感染者102例,其中EBV-HLH 61例,淋巴瘤相关HLH 36例,原发性HLH 5例.61例EBV-HLH患者中,男40例,女21例,男、女比例为1.9∶1,中位发病年龄28（12～78）岁,中位随访时间3（0.5～28）个月,患者l、3、6、12个月的总体生存（OS）率分别为65.6％、47.5％、32.4％、25.0％.确诊HLH4周内未使用依托泊苷治疗者12例,HLH-94方案治疗者33例,HLH-2004方案治疗者16例,三组患者的治疗有效率分别为33.3％、51.5％、43.8％,差异无统计学意义（P=0.401）;未使用依托泊苷治疗组和HLH-94/HLH-2004方案治疗组患者的OS率差异有统计学意义（P=0.033）.血清白蛋白水平（P=0.033）及EBV是否转阴（P=0.010）是影响患者预后的独立危险因素.结论 EBV-HLH患者病情凶险,预后差,及早应用以依托泊苷为基础的免疫化疗可以改善患者的生存情况.血清白蛋白水平、EBV是否转阴是影响EBV-HLH患者预后的独立危险因素.Objective To investigate the outcomes,survival status,and the prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis （EBV-HLH） patients.Methods A retrospective study was carried out to analyze the clinical data of 61 EBV-HLH cases,from January 2008 to July 2014.Prognostic factors were analyzed through COX model （single factor and multiple factors）.Results A total of 246 patients with HLH were diagnosed,among which 102 cases were with EBV infection （including 61 EBV-HLH,36 lymphoma associated HLH,5 primary HLH）.Among the 61 cases,40 were male and 21 were female,with a ration of 1.9：1.The median age was 28 years （range,12-78）.1,3,6 and 12-month overall survival rates of 61 EBV-HLH were 65.6％,47.5％,32.4％,and 25.0％,respectively.The median follow-up time was 3 （0.5-28） months.12 patients didn＇t use etoposide within 4 weeks after diagnosis,while HLH-94 protocol was used in 33 patients and HLH-2004 protocol was used in 16 patients.Response rates of theses three groups were 33.3％,51.5％,and 43.8％,respectively （P=0.401）.There was statistically difference between the group without etoposide and the HLH-94/HLH-2004 group in the overall survival rate （P=0.033）.Serum albumin level （P=0.033）and whether EBV could became negative （P=0.010） were independent predictors for EBV-HLH.Conclusions EBV-HLH patients have severe clinical feature and poor prognosis.Early application of immune chemotherapy based on etoposide can improve survival.Serum albumin level and whether EBV can become negative are independent prognostic factors for survival.

关 键 词：疱疹病毒4型 人 淋巴组织细胞增多症 嗜血细胞性 治疗结果 预后 

分 类 号：R55[医药卫生—血液循环系统疾病]