Fujimoto Ayumi, Miyazaki Kana, Yakushijin Kimikazu, Fujino Takahiro, Munakata Wataru, Ejima Yasuo, Maruyama Dai, Kubota Nobuko, Maeda Takeshi, Takizawa Jun, Hiramoto Nobuhiro, Takeuchi Masahiro, Sakai Rika, Fukuhara Noriko, Taguchi Senzo, Asano Naoko, Yamaguchi Motoko, Suzuki Ritsuro
Division of Hematology and Oncology, Department of Internal Medicine, Faculty of Medicine, Shimane University, Izumo, Japan.
Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan.
Leukemia. 2025 Apr;39(4):909-916. doi: 10.1038/s41375-025-02527-4. Epub 2025 Feb 17.
A retrospective study of extranodal natural killer/T-cell lymphoma (ENKL) patients diagnosed between 2014 and 2021 in Japan was conducted. Among 351 patients with sufficient data, 116 (33%) were in the advanced stage (5 in stage III and 111 in stage IV) at diagnosis, and were further analyzed. The median age was 60 years (range: 19-90), and 68 (59%) were male. Ninety-four (85%) of stage IV patients had two or more extranodal involvements. The most common first-line regimen was SMILE (steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide; 52%). The 2-year overall survival (OS) for all patients was 38.5%, which was significantly improved after 2017 (25.2% for 2014-2017 vs. 50.7% for 2018-2021; P = 0.008). Patients treated with SMILE showed better OS than those treated with DeVIC or CHOP (2y-OS: 57.1%, 35.8%, and 0%, respectively; P < 0.001). The prognosis was significantly better in patients who received hematopoietic stem cell transplantation (HSCT) than in those who did not (2-year OS: 68.3% vs. 17.6%, P < 0.001). Multivariate analysis showed SMILE and HSCT were significant factors for OS. In conclusion, the prognosis of advanced-stage ENKL has improved in recent years. The L-asparaginase-containing chemotherapy and subsequent HSCT is considered the recommended strategy.
对2014年至2021年期间在日本诊断出的结外自然杀伤/T细胞淋巴瘤（ENKL）患者进行了一项回顾性研究。在351例有足够数据的患者中，116例（33%）在诊断时处于晚期（III期5例，IV期111例），并对其进行了进一步分析。中位年龄为60岁（范围：19 - 90岁），男性68例（59%）。IV期患者中有94例（85%）有两个或更多结外受累部位。最常见的一线治疗方案是SMILE（类固醇、甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶和依托泊苷；52%）。所有患者的2年总生存率（OS）为38.5%，2017年后显著提高（2014 - 2017年为25.2%，2018 - 2021年为50.7%；P = 0.008）。接受SMILE治疗的患者的OS优于接受DeVIC或CHOP治疗的患者（2年OS分别为：57.1%、35.8%和0%；P < 0.001）。接受造血干细胞移植（HSCT）的患者的预后明显优于未接受移植的患者（2年OS：68.3%对17.6%，P < 0.001）。多因素分析显示SMILE和HSCT是OS的重要因素。总之，近年来晚期ENKL的预后有所改善。含L-天冬酰胺酶的化疗及随后的HSCT被认为是推荐的治疗策略。
