Extranodal T/NK lymphomas comprise infrequent and highly aggressive entities such as extranodal NK/T-cell lymphoma nasal type, enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, intestinal T-cell lymphoma NOS, and hepatosplenic T-cell lymphoma. Except for NK/T lymphoma nasal type, there is scarce evidence to support a specific therapeutic regimen in first line and relapse. As the only potentially curative therapy is allogeneic hematopoietic stem cell transplantation, it should be assessed in relapsing/refractory NK/T lymphoma nasal type and in the first line after remission in the other extranodal NK/T lymphomas.
Part of the book: Peripheral T-cell Lymphomas