原发性肺NK/T细胞淋巴瘤24例临床分析

Clinical analyses of 24 patients with primary pulmonary NK/T-cell lymphoma

目的探讨原发性肺NK/T细胞淋巴瘤的临床特点、最佳治疗方案及预后因素,以提高对原发性肺NK/T细胞淋巴瘤的认识。方法回顾性分析2011年4月至2019年5月收治的24例明确诊断为原发性肺NK/T细胞淋巴瘤患者的临床资料,并对其临床特征、治疗及预后进行分析。采用Kaplan-Meier进行生存分析,应用Cox比例风险回归模型确定影响总生存(OS)期和疾病无进展生存(PFS)期的预后因素。结果①入组的24例患者中男16例,女8例,中位年龄为49(4~76)岁。②患者初始症状大多为发热(66.7%)、咳嗽、呼吸困难。主要的胸部影像学表现为单侧(45.8%)或双侧(54.2%)肺部浸润、结节或肿块。③24例患者中20例接受放化疗或造血干细胞移植,4例接受姑息治疗。所有患者的中位OS期为9.5(0.1~26.0)个月,1年OS率为45.8%。以门冬酰胺酶为基础化疗方案的总有效率(ORR)为88.2%。④单因素分析显示,年龄≤60岁患者的OS和PFS显著优于年龄>60岁的患者(P值分别为0.002、0.004),美国东部肿瘤协作组体能状态评分(ECOG评分)≤2分患者的OS和PFS显著优于ECOG评分>2分患者(P值分别为0.042、0.004)。多因素分析显示,年龄>60岁和ECOG评分>2分与不良OS和PFS相关(OS:P值分别为0.024、0.024;PFS:P值分别为0.035、0.024)。结论原发性肺NK/T细胞淋巴瘤罕见且预后差,以门冬酰胺酶为基础的化疗方案疗效显著,年龄>60岁和ECOG评分>2分是患者独立的不良预后因素。

Objective To explore the clinical characteristics,the best treatment and prognostic factors of primary pulmonary NK/T-cell lymphoma.Methods A total of 24 cases with primary pulmonary NK/T-cell lymphoma from April 2011 to May 2019 were analyzed retrospectively.Survival analysis was performed using the Kaplan-Meier method and groups were compared using the log-rank test.Multivariate analysis using Cox proportional hazard regression model was conducted to confirm independent prognostic factors for overall survival(OS)and progression-free survival(PFS).Results①The cohort of 24 patients included 16 male and 8 female with a median age of 49 years(range,4-76 years)old.②Most patients initially presented with a fever(66.7%),cough and dyspnea.Chest imaging manifestations were primarily unilateral(45.8%)or bilateral(54.2%)pulmonary consolidation,nodules or mass.③20 patients received chemotherapy,radiotherapy or hematopoietic stem cell transplantation,the rest 4 cases palliative treatment.Median OS was 9.5 months(range,0.1-26.0 months).The estimated 1-year OS rate was 45.8%.Overall response rate of patients treated with asparaginase-based regimen was 88.2%.④In univariate survival analysis,age≤60 was prognostic for longer OS and PFS,compared with age>60(P=0.002 and 0.004,respectively);ECOG≤2 was prognostic for longer OS and PFS,compared with ECOG>2(P=0.042 and 0.004,respectively).In multivariate survival analysis,age>60 and ECOG>2 were significantly correlated with inferior OS and PFS(OS:P=0.024 and 0.024,respectively;PFS:P=0.035 and 0.024,respectively).Conclusions Primary pulmonary NK/T-cell lymphoma was a rare disease with poor prognosis.Asparaginase-based regimens appeared to be effective.Age and ECOG served as independent prognostic factors for primary pulmonary NK/T-cell lymphoma patients.