Prior to the 2000, patients with extranodal NK/T-cell lymphoma, nasal type (ENKL) were typically treated with anthracycline-containing chemotherapy, such as CHOP therapy, and the therapeutic outcomes were unsatisfactory. Since the early 2000s, next-generation therapies without anthracyclines have been developed and tested in clinical trials, markedly changing ENKL treatment. A retrospective, Next-Generation Therapy for NK/T-Cell Lymphoma in East Asia (NKEA) Part A, study in Japan investigated the current state of ENKL management. The results revealed that radiotherapy and dexamethasone, etoposide, ifosfamide, and carboplatin (RT-DeVIC) concurrent chemoradiotherapy was selected as the first-line therapy in 66% patients with localized ENKL, diagnosed between 2010 and 2013 in 31 institutes in Japan. The 5-year overall survival and progression-free survival rates in 150 patients treated with RT-DeVIC in clinical practice were 72% and 61%, respectively, confirming the results of an RT-DeVIC clinical trial. Furthermore, the NKEA study highlighted several limitations of current ENKL management strategies. Now is the time to explore effective therapies for ENKL beyond the current next-generation therapies. International cooperation, utilizing the strengths of each country's treatment protocols, will contribute to advancement in ENKL treatment.
Keywords: Chemoradiotherapy; ENKL treatment guidelines; L-asparaginase; NK/T-cell lymphoma.