淋巴瘤相关噬血细胞综合征临床特征和预后分析  

Clinical characteristics and prognosis of lymphoma‑associated hemophagocytic syndrome

作　　者：赵艾琳 李萌 李林峰 干欣霭 王婕[1] 李赫[1] 沈恺[1] 杨云帆 牛挺[1] Zhao Ailin;Li Meng;Li Linfeng;Gan Xin′ai;Wang Jie;Li He;Shen Kai;Yang Yunfan;Niu Ting(Department of Hematology,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院血液内科,成都610041

出　　处：《中华医学杂志》2022年第28期2173-2180,共8页National Medical Journal of China

基　　金：国家血液系统疾病临床医学研究中心转化研究课题(2021WWB03);中国博士后科学基金(2021M692310);四川大学华西医院学科卓越发展1.3.5工程(ZYJC21007);四川大学华西医院临床研究孵化项目(19HXFH030)。

摘　　要：目的探讨淋巴瘤相关噬血细胞综合征(LAHS)患者的临床特点、治疗及预后影响因素。方法收集四川大学华西医院2010年1月至2021年10月诊断为LAHS患者的临床资料,分析其基线临床特点、治疗选择、总缓解率(ORR)、总生存期(OS),并对影响预后的因素进行单因素及多因素分析。结果共94例患者纳入本研究,男59例、女35例,诊断为噬血细胞综合征的年龄为(40.5±17.3)岁。其中T/自然杀伤(NK)细胞淋巴瘤74例、B细胞淋巴瘤15例、霍奇金淋巴瘤5例。T/NK细胞LAHS患者诊断年龄为(37.9±16.2)岁,B细胞LAHS患者的诊断年龄为(55.9±14.0)岁,T/NK细胞LAHS患者比B细胞LAHS患者诊断年龄小(P<0.001)。T/NK细胞LAHS患者基线纤维蛋白原低于B细胞LAHS患者[1.34(0.86,2.44)g/L比2.20(1.75,4.25)g/L,P=0.008]。35例行噬血细胞综合征特异方案与抗淋巴瘤特异方案的联合治疗,31例行噬血细胞综合征特异的治疗,8例行抗淋巴瘤特异的治疗,7例仅行糖皮质激素治疗,总体患者ORR为49.4%,中位OS为61 d,先行抗噬血细胞综合征后转为抗淋巴瘤治疗者ORR(69.0%比38.7%,P=0.019)及中位OS最优(192.0比24.5 d,P=0.028),优于抗噬血细胞综合征治疗者,并且有优于抗淋巴瘤治疗的趋势。结外鼻型NK/T细胞淋巴瘤或侵袭性NK/T细胞白血病是LAHS预后的危险因素(HR=0.113,95%CI:0.018~0.728,P=0.022)。结论LAHS预后差,应迅速抗噬血细胞综合征后尽早启动抗淋巴瘤治疗。Subjective To investigate clinical characteristics,treatment,and prognosis of lymphoma‑associated hemophagocytic syndrome(LAHS)patients.Methods The clinical data of patients diagnosed with LAHS from January 2010 to October 2021 in West China Hospital were retrospectively analyzed.Clinical characteristics,treatment,overall response rate(ORR),and overall survival(OS)were investigated.Univariate and multivariate analysis of potential factors were conducted.Results Of all 94 patients included,59 were male and 35 were female.The age at hemophagocytic lymphohistiocytosis(HLH)diagnosis was(40.5±17.3)years.Seventy‑four cases were T/NK cell lymphoma;15 were B cell lymphoma;5 were Hodgkin lymphoma.The age at HLH diagnosis of T/NK cell LAHS patients was(37.9±16.2)years,while that of B cell LAHS patients was(55.9±14.0)years.T/NK cell LAHS patients were significantly younger than B cell LAHS patients(P<0.001).Baseline fibrinogen of T/NK cell LAHS patients was 1.34(0.86,2.44)g/L,while that of B cell LAHS patients was 2.20(1.75,4.25)g/L.T/NK cell LAHS patients showed significantly lower fibrinogen levels than B cell LAHS patients(P=0.008).Combined treatment of anti‑HLH and anti‑lymphoma treatment was conducted in 35 patients;anti‑HLH treatment was conducted in 31 patients;anti‑lymphoma treatment was conducted in 8 patients;glucocorticoid treatment was conducted in 7 patients.ORR was 49.4%,and the median OS was 61 days for overall patients.Patients who received anti‑HLH treatment and turned to anti‑lymphoma treatment early displayed the best ORR and OS,significantly higher than those of anti‑HLH patients(69.0 vs 38.7%,P=0.019,and 192.0 vs 24.5 days,P=0.028,respectively),which were also insignificantly higher than those of anti‑lymphoma patients.Extranodal NK/T‑cell lymphoma or aggressive natural killer cell leukemia was the risk factor of LAHS prognosis(HR=0.113,95%CI:0.018-0.728,P=0.022).Conclusions Prognosis of LAHS patients is poor.Anti‑lymphoma treatment should be initiated as soon as HLH is rapidl

关 键 词：淋巴瘤 噬血细胞综合征 淋巴瘤 结外NK‑T细胞 预后 生存 回顾性队列研究 

分 类 号：R733.1[医药卫生—肿瘤]