上呼吸消化道外结外鼻型NK/T细胞淋巴瘤临床特征和预后

Clinical features and treatment outcome of extranodal nasal-type NK/T-cell lymphoma of the extra-upper aerodigestive tract

目的探讨上呼吸消化道外结外鼻型NK/T细胞淋巴瘤患者的临床病理特征和预后。方法收集2001年11月至2015年12月,中国淋巴瘤协作组数据库中159例原发上呼吸消化道外结外鼻型NK/T细胞淋巴瘤患者的临床资料,分析其临床病理特征、治疗和预后。生存分析采用Kaplan-Meier法和Log rank检验,多因素分析采用Cox回归模型。结果上呼吸消化道外结外鼻型NK/T细胞淋巴瘤多原发于皮肤软组织(106/159,66.7%)和胃肠道(31/159,19.5%)。Ann ArborⅢ~Ⅳ期和乳酸脱氢酶(LDH)升高的患者比例较高,分别为64.2%(102/159)和47.8%(76/159)。全组患者的3年总生存率为43.6%,3年无进展生存率为27.9%。原发皮肤软组织和胃肠道结外鼻型NK/T细胞淋巴瘤患者的3年生存率分别为41.0%和59.4%(P=0.281),3年无进展生存率分别为24.8%和48.3%(P=0.109)。综合治疗较单纯化疗提高了全组患者的3年生存率(58.4%和33.9%,P=0.001)和3年无进展生存率(40.7%和20.7%,P=0.008)。LDH升高、Ann Arbor分期和≥2个结外部位受侵为总生存的独立危险因素(P<0.05),LDH升高和≥2个结外部位受侵为无进展生存的独立危险因素(P<0.05)。失败模式以远处结外器官复发进展为主。结论上呼吸消化道外结外鼻型NK/T细胞淋巴瘤具有独特的临床特征和较差的预后,放疗或手术±化疗较单纯化疗可能提高上呼吸消化道外结外NK/T细胞淋巴瘤患者的疗效。

Objective To investigate the clinical features and prognosis of extranodal nasal-type NK/T-cell lymphoma of the extra-upper aerodigestive tract(extra-UADT NKTCL).Methods The clinical data of 159 patients with extra-UADT NKTCL from the China Lymphoma Collaborative Group(CLCG)database between November 2001 and December 2015 were retrospectively analyzed.Kaplan-Meier survival analysis and Log-rank test were used to evaluate the prognosis.The Cox regression model is used for multi-factor analysis.Results Extra-UADT NKTCL commonly occurs in skin and soft tissues(106/159,66.7%)and gastrointestinal tract(31/159,19.5%).The incidences of elevated lactate dehydrogenase(LDH)and Ann ArborⅢ~Ⅳstage were 47.8%(76/159)and 64.2%(102/159),respectively.The 3-year overall survival(OS)and progression-free survival(PFS)rates were 43.6%and 27.9%,respectively.The corresponding OS rates of primary skin/soft tissue site and gastrointestinal tract site were 41.0%and 59.4%(P=0.281),while the PFS rates were 24.8%and 48.3%,respectively(P=0.109).Combined modality treatment improved the 3-year OS of all the patients(58.4%vs 33.9%,P=0.001)and 3-year PFS(40.7%vs 20.7%,P=0.008)when compared with chemotherapy alone.LDH elevation,Ann Arbor synthesising and≥2 junction external bits were intrusive as independent risk factors for total survival(P<0.05),LDH elevation and≥2 junction outer bits were intrusive as independent risk factors for progressionless survival(P<0.05).The distant extranodal dissemination was the primary failure patterns.Conclusions Extra-UADT NKTCL appears to have distinct clinical characteristics and poor outcome.Compared with chemotherapy alone,combined modality treatment may improve the prognosis of patients with extra-UADT NKTCL.