title: Allogeneic bone marrow transplantation for chemotherapy-refractory hepatosplenic gammadelta T-cell lymphoma: case report and review of the literature.
abstract: 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.
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pathology, Vanderbilt University, Nashville, TN 37232, USA. jennifer.domm@vanderbilt.edu
authors.forename: Jennifer Ann
authors.initials: JA
authors.lastname: Domm
authors.forename: Maryann
authors.initials: M
authors.lastname: Thompson
authors.forename: John Frank
authors.initials: JF
authors.lastname: Kuttesch
authors.forename: Sari
authors.initials: S
authors.lastname: Acra
authors.forename: Haydar
authors.initials: H
authors.lastname: Frangoul
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002648:Child; D003131:Combined Modality Therapy; D019008:Drug Resistance, Neoplasm; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000601:surgery*; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000276:immunology / Q000601:surgery*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000276:immunology*; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000601:surgery*; D014184:Transplantation, Homologous; D014916:Whole-Body Irradiation
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta