Extranodal natural killer/T-cell lymphoma, nasal type (ENKL), is a rare subtype of non-Hodgkin lymphoma, and its treatment outcome was previously poor. Novel treatment strategies have improved the outcomes of ENKL remarkably in the last decade. Nowadays, patients with localized nasal ENKL are recommended treatment with concurrent chemoradiotherapy, and their 5-year overall survival rate is approximately 70%. In patients with advanced or relapsed/refractory disease, the efficacy of l -asparaginase–containing therapy has been confirmed. However, there still remain unmet needs in the treatment of ENKL. Continued efforts should be made to further improvements in the treatment of ENKL.
Key points
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Extranodal NK/T-cell lymphoma, nasal type (ENKL) is a predominantly extranodal lymphoma and is associated with Epstein-Barr virus.
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ENKL is a rare subtype of lymphoma (<1% of all NHL) with poor treatment outcome.
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Concurrent chemoradiotherapy containing MDR-nonrelated agents is recommended as an initial therapy in patients with newly diagnosed localized nasal ENKL.
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l -Asparaginase-containing regimen (eg, SMILE, AspaMetDex) is recommended as an induction therapy in patients with advanced or relapsed/refractory disease.
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