Extranodal Natural Killer/T-Cell Lymphomas: Current Approaches and Future Directions

Extranodal natural killer/T(NK/T)-cell lymphoma (ENKTL) is a rare subtype of non-Hodgkin lymphoma that typically presents with an isolated nasal mass, but a sizeable minority present with advanced stage disease and have a significantly poorer prognosis. Those with limited disease are standardly treated with chemotherapy and radiation while those with advanced stage disease are treated with L-asparaginase containing chemotherapy regimens. The addition of modern radiation therapy techniques and the incorporation of L-asparaginase into chemotherapy regimens have significantly improved outcomes in this disease, but relapses and death from relapsed disease remain frequent. Given the high rate of relapse, several novel therapies have been evaluated for the treatment of this disease. In this review, we explore the current standard of care for ENKTL as well as novel therapies that have been evaluated for its treatment and the biologic understanding behind these therapies.

Automated summary

Extranodal natural killer/T(NK/T)-cell lymphoma (ENKTL) is a rare subtype of non-Hodgkin lymphoma that usually presents with a nasal mass. Limited disease is treated with chemotherapy and radiation, while advanced stage disease is treated with L-asparaginase containing chemotherapy regimens. Modern radiation therapy techniques and the use of L-asparaginase in chemotherapy have improved outcomes, but relapse and relapse-related deaths remain frequent. Novel therapies have been evaluated for the treatment of this disease.