Nodal Epstein-Barr virus-positive T/NK-cell lymphoma (EB-nTNKL) is an extremely rare disease characterized by an aggressive clinical course and poor prognosis, for which treatment strategies have not yet been established. We herein report a young man with EB-nTNKL. Although initial chemotherapies, including L-asparaginase, failed to produce a good response, subsequent myeloablative allogeneic hematopoietic stem cell transplantation (alloHSCT) resulted in favorable disease control and a long-term disease-free survival. The prompt performance of alloHSCT using an available donor source at that time, regardless of whether or not the initial chemotherapy was effective, could be critical to saving patients with this otherwise fatal disease.
Keywords: Epstein-Barr virus-positive T/NK-cell tumors; L-asparaginase; allogeneic hematopoietic stem cell transplantation; nodal Epstein-Barr virus-positive T/NK-cell lymphoma.