NK-Cell Lymphomas

NK-cell malignancies are rare aggressive diseases associated with poor clinical outcome. There is a significant geographic variation in their incidence. At least a part of the reason for that is the fact that Epstein-Barr virus plays an important role in pathogenesis, and importantly, the plasma viral titer reflects disease burden and response to therapy. Extranodal NK/T-cell lymphoma, nasal type (ENKL), is the most common disease subtype in NK-cell malignancies. Conventional anthracycline-based chemotherapy was historically used for ENKL, only to produce dismal outcome. More recently, concurrent chemoradiation therapy for early-stage disease and non-anthracycline-based L-asparaginase containing chemotherapy have been studied, showing improved clinical response and survival, with long-term survival rates of 60-70% and 50-60%, respectively. Stem cell transplant can provide long-term disease control in recurrent or refractory disease settings, but the role of frontline use of such approach is yet to be determined. Several novel therapeutic approaches have shown promising results, and enrollment to clinical trials is the essential key to improve the treatment outcome in the future.