Nodal gamma/delta T cell lymphoma in complete remission following allogeneic bone marrow transplantation from an HLA-matched unrelated donor

Gamma/delta T cell lymphoma is very rare, and usually occurs as an extranod al tumor. We describe the case of a 16-year-old Japanese man with an unusua l nodal gamma/delta T cell lymphoma with generalized lymphadenopathy and bo ne marrow involvement. No tumor involvement was observed in the liver, sple en, or nasal cavity, Examination for surface antigens on lymphoma cells rev ealed a unique phenotype, positive for CD3 and T cell receptor (TCR) gamma/ delta, but negative for CD2. Genotypic analysis revealed the tumor to be of monoclonal origin and characterized by TCR gamma-chain gene rearrangement, but there was no rearrangement of the TCR beta-chain gene. Our patient's t umor responded to combination chemotherapy and subsequent allogeneic bone m arrow transplantation from an HLA-matched unrelated donor. He has remained well and free of disease for 35 months. Copyright (C) 2001 S. Karger AG, Ba sel.