Objective: To analyze clinical features and outcomes of non-upper aerodigestive tract NK/T-cell lymphoma (NUAT-NKTCL).
Methods: Clinical data of 44 patients with NUAT-NKTCL diagnosed at Peking University Cancer Hospital between 1999 and 2013 were retrospectively analyzed.
Results: Of the 44 patients, there were 31 males and 13 females with a median age of 39 years (range, 15 to 82 years). 27 patients (61.4%) were stage III/IV, 28(63.6%) with B symptoms, 12(27.3%) ECOG ≥ 2, 18 (40.9%) IPI score ≥ 3, and 48.8% patients had elevated serum lactate dehydrogenase. The common primary sites were skin (21/44, 47.2%) and intestinal tract (11/44, 25.0%). All the 44 patients received systemic chemotherapy. After a median follow-up of 13.5 months (range, 0.3-121.0 months), 32 patients died, and the median overall survival (OS) was 16 months with 1-year OS rate as 54.1%. CR rate of the 26 patients received CHOP or CHOPE regimens as the first line chemotherapy was 19.2% (5/26). Then L-asparaginase (L-ASP)- based regimens were used for salvage treatment, with CR rate of 47.7% and the median OS of 13 months. CR rate of the other 18 patients received L-ASP-based regimens in the firstline therapy was 55.6% (10/18) with the median OS of 16 months. Using L-ASP in firstline treatment obviously improved CR rate (P=0.015), but did not affect OS (P=0.774).
Conclusion: Although L-ASP improved the efficacy of NUAT-NKTCL, but the prognosis remained dismal. Thus, more effective treatment strategies are required for NUAT-NKTCL.
目的: 了解非上呼吸消化道原发NK/T细胞淋巴瘤（NUAT-NKTCL）患者的临床特征及生存情况。
方法: 收集44例NUAT-NKTCL患者的临床资料，对其临床特征、疗效及生存进行相关性分析。
结果: 44例患者中男31例，女13例，中位年龄39（15～82）岁，Ⅲ/Ⅳ期者27例（61.4%）。就诊时主要症状为皮肤肿物（21/44）、腹痛（11/44）、睾丸肿大（3/44）及间断咳嗽（2/44）等。存在B症状者28例（63.6%），ECOG评分≥2分者12例（27.3%），IPI评分≥3分者18例（40.9%），LDH升高者占48.8%（21/43）。全组患者均接受化疗为主的治疗，中位随访13.5（0.3～121.0）个月，共有32例患者死亡，中位生存时间为16个月，1年总生存率为54.1%。按照一线治疗方案分组：CHOP或CHOPE方案治疗组26例，完全缓解（CR）5例（19.2%），其中有19例患者接受左旋门冬酰胺酶（L-ASP）为主的挽救化疗，9例（47.7%）获CR，中位随访时间为11个月，中位生存时间为13个月；L-ASP为主的方案一线治疗组18例，CR 10例（55.6%），中位随访时间为15个月，中位生存时间为16个月。后者较前者CR率提高（P= 0.015），但两组患者总生存时间差异无统计学意义（P=0.774）。
结论: NUAT-NKTCL侵袭性强，患者起病时分期晚。与传统方案比较采用L-ASP为主的方案可提高NUAT-NKTCL患者的疗效，但其长期生存与上呼吸消化道原发患者相比仍不理想，需要探索更为有效的治疗策略。