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Allogeneic bone marrow transplantation for chemotherapy-refractory hepatosplenic gammadelta T-cell lymphoma: case report and review of the literature.

Domm JA, Thompson M, Kuttesch JF, Acra S, Frangoul H
J Pediatr Hematol Oncol. 2005 27 (11): 607-10

PMID: 16282893 · DOI:10.1097/01.mph.0000187431.37369.f5

Hepatosplenic gammadelta T-cell lymphoma is an uncommon pediatric disease and is associated with an aggressive and often fatal course. The authors describe the case of an 8-year-old girl who presented with transaminitis and hepatosplenomegaly. Liver biopsy and peripheral blood flow cytometry were diagnostic of hepatosplenic gammadelta T-cell lymphoma. She was treated with multi-agent chemotherapy with cyclophosphamide, vincristine, prednisone, doxorubicin, and high-dose methotrexate but failed to achieve durable remission. She underwent an allogeneic bone marrow transplant from her HLA-identical brother with a preparative regimen including total body irradiation and cyclophosphamide. She is currently alive and has remained in remission for 30 months after transplantation. The authors also review the literature for similar pediatric cases.

MeSH Terms (14)

Antineoplastic Combined Chemotherapy Protocols Bone Marrow Transplantation Child Combined Modality Therapy Drug Resistance, Neoplasm Female Flow Cytometry Humans Liver Neoplasms Lymphoma, T-Cell Receptors, Antigen, T-Cell, gamma-delta Splenic Neoplasms Transplantation, Homologous Whole-Body Irradiation

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