本研究调查了晚期非淋巴结型自然杀伤/ T细胞淋巴瘤（ENKTL）的治疗与预后。在中位随访时间为75.03个月的情况下，对195例新诊断的III/IV期ENKTL患者，中位总生存期（mOS）为19.43个月，预计的1年、2年、3年和5年总生存率分别为59.5%、46.3%、41.8%和35.1%。化疗（CT）+放疗（RT）与单独化疗（CT）相比（P=0.007），造血干细胞移植（HSCT）与无HSCT相比（P<0.001），都能提高总生存期。对于60岁及以下不适宜进行HSCT的患者，其他完全缓解的治疗可导致可比较的总生存期（P=0.141）。曾接受过奇达米德治疗的9例患者实现了中位无进展生存期（mPFS）和中位总生存期（mOS）分别为53.63个月（范围，3.47-92.33个月）和54.80个月（范围，5.50-95.70个月），而4例接受奇达米德维持治疗（MT）的患者实现了mPFS和mOS分别为55.83个月（范围，53.27-92.33个月）和60.65个月（范围，53.70-95.70个月），这可能为非HSCT患者提供了另一种选择。非蒽环素（ANT）-与ANT-、阿斯帕拉金酸酶（Aspa）-与非Aspa-以及吉西他滨（Gem）-与非Gem-基治疗相比，分别延长了进展无病生存期（P=0.031；P=0.005；P=0.009）和总生存期（P=0.010；P=0.086；P=0.003）。多变量分析表明，Gem-基治疗改善了进展无病生存期（HR=0.691，P=0.061）和总生存期（HR=0.624，P=0.037）。与仅含有Gem或Aspa的方案相比，Gem + Aspa联合方案稍微改善了进展无病生存期和总生存期（P>0.05）。提出了“强化疗法”的一线治疗方案，包括化疗（特别是Gem + Aspa方案）、放疗、HSCT和替代奇达米德MT，并且可以改善晚期ENKTL的长期生存。进行中的前瞻性临床研究可能进一步揭示奇达米德MT的价值。© 2023 UICC.

The study investigated the treatment and prognosis of advanced-stage extranodal natural killer/T-cell lymphoma (ENKTL). With a median follow-up of 75.03 months, the median overall survival (mOS) for the 195 newly diagnosed stage III/IV ENKTL patients was 19.43 months, and estimated 1-, 2-, 3- and 5-year OS were 59.5%, 46.3%, 41.8% and 35.1%, respectively. Chemotherapy (CT) + radiotherapy (RT) compared to CT alone (P = .007), and hematopoietic stem cell transplantation (HSCT) compared to non-HSCT (P < .001), both improved OS. For patients ≤60 years and ineligible for HSCT, other therapies with complete remission led to comparable OS (P = .141). Nine patients ever treated with chidamide achieved a median progression-free survival (mPFS) and mOS of 53.63 (range, 3.47-92.33) and 54.80 (range, 5.50-95.70) months, and four with chidamide maintenance therapy (MT) achieved a mPFS and mOS of 55.83 (range, 53.27-92.33) and 60.65 (range, 53.70-95.70) months, possibly providing an alternative option for non-HSCT patients. Non-anthracycline (ANT)- compared to ANT-, asparaginase (Aspa)- compared to non-Aspa- and gemcitabine (Gem)- compared to non-Gem-based regimens, prolonged PFS (P = .031; P = .005; P = .009) and OS (P = .010; P = .086; P = .003), respectively. Multivariate analysis demonstrated that Gem-based regimens improved PFS (HR = 0.691, P = .061) and OS (HR = 0.624, P = .037). Gem + Aspa combinations slightly improved PFS and OS compared to regimens containing Gem or Aspa alone (P > 0.05). First-line "intensive therapy," including CT (particularly Gem + Aspa regimens), RT, HSCT and alternative chidamide MT, was proposed and could improve long-term survival for advanced-stage ENKTLs. Ongoing prospective clinical studies may shed further light on the value of chidamide MT.© 2023 UICC.