Extranodal natural killer (NK)/
T cell lymphoma (ENKTL) is a distinct subtype of Non-Hodgkin’s
lymphoma mainly involving the nasal area. Since the entity was first recognized,
treatment strategies have been evolving from
anthracycline-based
chemotherapy and
radiotherapy to L-
asparaginase containing regimens and recently
immune checkpoint inhibitors. With the currently used combined
chemotherapy and
radiotherapy, more than 70% of
patients with localized
disease can be cured. L-
asparaginase containing regimens have significantly improved
treatment outcomes among
patients with advanced
disease. However, the
treatment outcomes of
patients with
disease refractory to L-
asparaginase containing regimens or
who experience
recurrence remain poor. In this article, we cover the current
treatments for ENKTL and emerging
treatment approaches.