单中心结外NK/T细胞淋巴瘤患者临床特点及基于培门冬酶化疗的预后分析

Clinical Characteristics and Prognostic Analysis of Extranodal NK/T-Cell Lymphoma Patients Treated with Pegaspargase Based Chemotherapy

目的:探讨结外NK/T细胞淋巴瘤患者的临床特点,以及应用以培门冬酶为基础的化疗方案的患者的疗效、生存情况及预后影响因素。方法:回顾性分析2015年1月至2019年6月于北京协和医院病理确诊和治疗的61例结外NK/T细胞淋巴瘤患者的临床资料,探究其临床特点、生存情况及预后的影响因素。结果:整组患者男女比例为2.8∶1,中位发病年龄为46(17-67)岁。Ann Arbor分期Ⅰ/Ⅱ期患者30例,Ⅲ/Ⅳ期患者31例,鼻型与非鼻型的比例为4.1∶1。结外受累常见部位为皮肤及皮下组织(26.2%)、肝脏(14.8%)、肺(13.1%)及胃肠道(13.1%)。中枢受累比例为9.8%,骨髓受累的比例为11.5%。中位随访时间为22(1-53)个月,整组患者2年无进展生存(PFS)率和总生存(OS)率分别为51.6%和53.2%。Ⅰ/Ⅱ期患者2年OS率为87.5%,而Ⅲ/Ⅳ期患者2年OS率仅为21.2%,差异具有统计学意义(P <0.0001)。非鼻型患者合并不良预后因素的比例更高,其中位OS仅为3个月。多因素分析结果显示,年龄> 60岁和Ann Arbor分期Ⅲ/Ⅳ期为影响结外NK/T细胞淋巴瘤患者PFS及OS的独立不良预后因素(HR=3.681,95%CI 1.322-10.250;HR=4.611,95%CI 1.118-19.009)。结论:以培门冬酶为基础的化疗方案显著改善了结外NK/T细胞淋巴瘤患者的生存。Ⅰ-Ⅱ期患者已达到较好的2年总生存率,而Ⅲ-Ⅳ期及非鼻型的患者预后仍较差。年龄> 60岁和Ann Arbor分期Ⅲ/Ⅳ期为ENKL患者预后的独立不良因素。

Objective:To investigate the clinical characteristics of patients with extranodal NK/T-cell lymphoma(ENKL),and to analyze the factors that affecting the survival and prognostic of patients treated with pegaspargase based chemotherapy.Methods:The clinical data of 61 ENKL patients treated in Peking Union Medical College Hospital from January 2015 to June 2019 were enrolled and retrospectively analyzed.The clinical characteristics,survival rate and influencing factors of prognostic in patients were investigated.Results:The male and female ratio in the whole group was 2.8:1.The median age was 46 years old(range,17-67 years old).30 patients were in stage Ⅰ/Ⅱ,while 31 patients were in stage Ⅲ/Ⅳ.The ratio of nasal and non-nasal type was 4.1:1.The common sites of extranodal involvement were skin and subcutaneous tissue(26.2%),liver(14.8%),lung(13.1%) and gastrointestinal tract(13.1%).9.8% of patients showed central nervous system involvement and 11.5% showed bone marrow involvement.The median follow-up time was 22 months(range,1-53 months).The 2-year PFS and OS rates of patients in the whole group were 51.6% and 53.2%,respectively.The 2-year OS rate of patients at stage Ⅰ/Ⅱ was87.5%,while that of patients at stage Ⅲ/Ⅳ was only 21.2%,the difference showed statistically significant(P <0.0001).Patients with non-nasal type disease showed a higher proportion in adverse factors of prognosis,of whom the median OS was only 3 months.Multivariate analysis showed that age>60 years old and Ann Arbor stage Ⅲ-Ⅳ were the independent adverse factors that affecting the prognosis of PFS and OS(HR=3.681,95% CI 1.322-10.250;HR=4.611,95% CI 1.118-19.009).Conclusion:The survival of ENKL patients has been significantly improved by pegaspargase based chemotherapy.Patients with stage Ⅰ/Ⅱ disease have achieved a relatively good 2-year OS rate of87.5%,but the prognosis of stage Ⅲ/Ⅳ and normasal type patients are still poor.Age>60 years old and Ann Arbor stage Ⅲ/Ⅳ are independent adverse prognostic factors for ENKL patients.