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 Arbor Ⅲ~Ⅳ stage 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.
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 Arbor Ⅲ～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。.
Keywords: Chemotherapy; Lymphoma, NK/T-cell; Prognosis; Radiotherapy.