Yamaguchi Motoko, Miyazaki Kana
Department of Hematology and Oncology, Mie University Graduate School of Medicine.
J Clin Exp Hematop. 2017 Dec 27;57(3):98-108. doi: 10.3960/jslrt.17018. Epub 2017 Jul 6.
Extranodal NK/T-cell lymphoma, nasal type (ENKL), is a form of lymphoma characterized by preferential extranodal involvement, Epstein-Barr virus (EBV) association, and geographic diversity in incidence. ENKL tumor cells express P-glycoprotein, which is related to multidrug resistance (MDR). This MDR phenomenon is thought to be the major reason why ENKL is resistant to anthracycline-containing chemotherapies and has led researchers to explore novel therapeutic strategies. Since the early 2000s, next-generation therapies, including upfront radiotherapy, chemotherapy, or concurrent chemoradiotherapy using non-MDR-related drugs, have markedly changed the management of ENKL. However, a recent large retrospective study in Japan revealed several limitations of next-generation therapies, in particular that they resulted in almost no improvement of early disease progression. This review will summarize the current management of ENKL, primarily based on clinical trial results, and provide clues for better future management.
结外NK/T细胞淋巴瘤，鼻型（ENKL）是一种淋巴瘤，其特征为优先累及结外部位、与爱泼斯坦-巴尔病毒（EBV）相关以及发病率存在地域差异。ENKL肿瘤细胞表达P-糖蛋白，这与多药耐药（MDR）有关。这种MDR现象被认为是ENKL对含蒽环类化疗药物耐药的主要原因，并且促使研究人员探索新的治疗策略。自21世纪初以来，包括 upfront放疗、化疗或使用与MDR无关药物的同步放化疗在内的新一代疗法显著改变了ENKL的治疗方式。然而，日本最近一项大型回顾性研究揭示了新一代疗法的几个局限性，特别是它们几乎未能改善早期疾病进展。本综述将主要基于临床试验结果总结ENKL的当前治疗方法，并为未来更好的治疗提供线索。
