Yamaguchi Motoko, Oguchi Masahiko, Suzuki Ritsuro
Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan.
Department of Radiation Oncology, Cancer Institute Hospital of the Japanese Foundation for Cancer Research, Tokyo, Japan.
Best Pract Res Clin Haematol. 2018 Sep;31(3):315-321. doi: 10.1016/j.beha.2018.07.002. Epub 2018 Jul 3.
Extranodal NK/T-cell lymphoma, nasal type (ENKL), is a rare lymphoma subtype of peripheral T/NK-cell lymphoma that is very common in East Asia and Latin America. Two-thirds of patients have localized disease in the nasal cavity or adjacent sites. Large retrospective studies have revealed the clinicopathologic features of ENKL patients, identified risk factors for short survival time, and developed prognostic models. Next-generation sequencing studies have provided a comprehensive list of recurrent mutations in ENKL. Since the early 2000s, disease-specific therapeutic approaches have been developed, and the standard of care for ENKL has markedly changed. Non-anthracycline-containing chemotherapy with or without radiotherapy is the current standard approach for ENKL treatment. Emerging therapies, including the use of immune checkpoint inhibitors, are being investigated.
结外NK/T细胞淋巴瘤，鼻型（ENKL），是外周T/NK细胞淋巴瘤中一种罕见的淋巴瘤亚型，在东亚和拉丁美洲非常常见。三分之二的患者在鼻腔或邻近部位有局限性疾病。大型回顾性研究揭示了ENKL患者的临床病理特征，确定了生存时间短的危险因素，并建立了预后模型。二代测序研究提供了ENKL中复发性突变的完整列表。自21世纪初以来，已开发出针对该疾病的治疗方法，ENKL的治疗标准已发生显著变化。含或不含放疗的非蒽环类化疗是目前ENKL治疗的标准方法。包括使用免疫检查点抑制剂在内的新兴疗法正在研究中。
