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

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.