Extranodal NK/T Cell Lymphoma: Evidence-based Review of Safety and Toxicity of the Available Regimens.

Extranodal natural killer/T cell lymphoma (ENKTL) is a rare type of mature T and NK neoplasm. It is more prevalent in Asia and Latin America where the Epstein-Barr virus is endemic and has been linked to the disease. Most studies have emerged from those 2 regions to evaluate best management options. There are no standards of care in the management owing to the lack of unified approach depending on the treatment region and availability of different therapy options. Several reviews have focused on the outcome of the different chemotherapy combinations. There are no systematic reviews of the toxicity of those regimens despite the fact that many of them incorporate the use of asparaginase and/or radiation in combination with chemotherapy. We have found that although integration of asparaginase and/or radiation have led to improved outcome, it was done at the expense of increased toxicity. The most recent studies are showing promising outcomes while decreasing toxicity.