成年人淋巴瘤相关噬血细胞综合征临床预后多中心研究  

Clinical prognosis of lymphoma-associated hemophagocytic syndrome in adults:a multicenter study

作　　者：沈子园 贺晨露 王颖 刘沁华[3] 张颢[4] 苗雨青 顾伟英[6] 王春玲[7] 王玲[8] 叶静静[9] 金英良 桑威 朱太岗[11] 淮海淋巴瘤协作组 Shen Ziyuan;He Chenlu;Wang Ying;Liu Qinhua;Zhang Hao;Miao Yuqing;Gu Weiying;Wang Chunling;Wang Ling;Ye Jingjing;Jin Yingliang;Sang Wei;Zhu Taigang;Huaihai Lymphoma Working Group(Department of Epidemiology and Health Statistics,School of Public Health,Xuzhou Medical University,Xuzhou 221004,China;Department of Personnel,Suqian First People's Hospital in Jiangsu Province,Suqian 223800,China;Department of Hematology,the First Affiliated Hospital of Anhui Medical University,Hefei 230022,China;Department of Hematology,the Affiliated Hospital of Jining Medical University,Jining 272000,China;Department of Hematology,Yancheng First People's Hospital in Jiangsu Province,Yancheng 224001,China;Department of Hematology,the First People's Hospital of Changzhou in Jiangsu Province,Changzhou 213003,China;Department of Hematology,the First People's Hospital of Huaian in Jiangsu Province,Huaian 223000,China;Department of Hematology,Taian Central Hospital in Shandong Province,Taian 271000,China;Department of Hematology,Qilu Hospital of Shandong University,Jinan 250012,China;Department of Hematology,Affiliated Hospital of Xuzhou Medical University,Xuzhou 221002,China;Department of Hematology,the General Hospital of Wanbei Coal-Electric Group,Suzhou 234000,China;不详)

机构地区：[1]徐州医科大学公共卫生学院流行病与卫生统计学系,江苏徐州221004 [2]江苏省宿迁市第一人民医院人事处,223800 [3]安徽医科大学附属第一医院血液科,合肥230022 [4]济宁医学院附属医院血液科,山东济宁272000 [5]江苏省盐城市第一人民医院血液科,224001 [6]江苏省常州市第一人民医院血液科,213003 [7]江苏省淮安市第一人民医院血液科,223000 [8]山东省泰安市中心医院血液科,271000 [9]山东大学齐鲁医院血液科,济南250012 [10]徐州医科大学附属医院血液科,江苏徐州221002 [11]皖北煤电集团总医院血液科,安徽宿州234000 [12]不详

出　　处：《白血病．淋巴瘤》2021年第9期542-546,共5页Journal of Leukemia & Lymphoma

基　　金：江苏省自然科学基金(BK20171181);江苏省科技厅社会发展重点项目(BE2019638);江苏省青年医学重点人才(QNRC2016791)。

摘　　要：目的基于多中心数据探讨成年人淋巴瘤相关噬血细胞综合征(LAHS)预后的影响因素。方法回顾性分析2015年1月至2020年8月淮海淋巴瘤协作组中9家医疗中心确诊的86例LAHS患者的临床资料。基于MaxStat算法获取连续变量的最佳截断值;单因素、多因素分析采用Cox比例风险回归模型;采用Kaplan-Meier法进行生存分析,并行log-rank检验。结果86例成年LAHS患者中男性50例(58.1%),女性36例(41.9%);患者中位年龄57岁(19~76岁),中位总生存(OS)时间1.67个月(95%CI 0.09~3.24个月),最常见的病理类型为弥漫大B细胞淋巴瘤(58例,67.44%)。基于MaxStat算法,患者年龄、清蛋白、血肌酐、乳酸脱氢酶、纤维蛋白原和血小板计数的最佳截断值分别为64岁、30.1 g/L、67μmol/L、1045 U/L、4.58 g/L和72×109/L。多因素分析表明,年龄、乳酸脱氢酶、清蛋白和纤维蛋白原水平是OS的独立影响因素(均P<0.05)。结论LAHS病情凶险,进展快。年龄≥64岁、乳酸脱氢酶≥1045 U/L、纤维蛋白原≥4.58 g/L、清蛋白<30.1 g/L的患者的生存情况较差。Objective To explore the prognostic influencing factors of adult lymphoma-associated hemophagocytic syndrome(LAHS)based on multicenter data.Methods The clinical data of 86 LAHS patients diagnosed in 9 medical centers of Huaihai Lymphoma Working Group from January 2015 to August 2020 were retrospectively analyzed.The optimal cut-off value of continuous variables was obtained based on MaxStat algorithm.Cox proportional hazard regression model was used for univariate and multivariate analyses.Kaplan-Meier method was used for survival analysis,and log-rank test was performed.Results Among the 86 adult LAHS patients,50(58.1%)were males and 36(41.9%)were females,the median age of the patients was 57 years old(19-76 years old),and the median overall survival(OS)time was 1.67 months(95%CI 0.09-3.24 months).The most common pathologic type was diffuse large B-cell lymphoma(58 cases,67.44%).Based on MaxStat algorithm,the optimal cut-off values of age,albumin,serum creatinine,lactate dehydrogenase,fibrinogen and platelet count were 64 years old,30.1 g/L,67μmol/L,1045 U/L,4.58 g/L and 72×109/L,respectively.Multivariate analysis showed that patient's age,lactate dehydrogenase,albumin and fibrinogen levels were independent influencing factors for OS(all P<0.05).Conclusions LAHS is dangerous and progresses quickly.Patients with age≥64 years old,lactate dehydrogenase≥1045 U/L,fibrinogen≥4.58 g/L and albumin<30.1 g/L have poor survival.

关 键 词：淋巴细胞增多症 噬血细胞性 淋巴瘤 成年人 预后 

分 类 号：R733.1[医药卫生—肿瘤]