title: Hepatosplenic alphabeta T cell lymphoma.
abstract: A 32-year-old male with chronic hepatitis B was admitted to a hospital with cellulitis in the right leg in September 2006. Pancytopenia, hepatosplenomegaly, and systemic superficial lymph node swelling were noted, and he was referred to our hospital. He developed fever and liver dysfunction in June 2007 and underwent a splenectomy. His pancytopenia subsequently improved. A pathologic diagnosis of hepatosplenic alphabeta T cell lymphoma was made by examining spleen tissue and biopsy specimens of the liver and mesenteric lymph node. He had stage IVB disease because neoplastic T cells were noted in the bone marrow. The response of the lymphoma to conventional chemotherapy including the CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) and DeVIC (dexamethasone, etoposide, ifoshamide, carboplatin) regimens was poor and transient. A partial remission was obtained with an ESHAP (etoposide, cisplatin, cytarabine, methylprednisolone) regimen. Therefore, we planned a bone marrow transplantation (BMT) from an HLA-haploidentical sibling donor. He was moved to the Department of Hematology, Hyogo Medical College, to receive this BMT as part of a clinical trial. During the conditioning procedure for the transplantation, however, he died of septicemia. Since hepatosplenic alphabeta T cell lymphoma is very rare with only 23 reported cases to date, herein we report this case and discuss the therapeutic strategy.
authors.affiliation: Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, 4-6 Minatojima-Nakamachi, Chuo-ku, Kobe 650-0046, Japan.
authors.forename: Yuya
authors.initials: Y
authors.lastname: Nagai
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Ikegame
authors.forename: Minako
authors.initials: M
authors.lastname: Mori
authors.forename: Daichi
authors.initials: D
authors.lastname: Inoue
authors.forename: Takaharu
authors.initials: T
authors.lastname: Kimura
authors.forename: Sonoko
authors.initials: S
authors.lastname: Shimoji
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Togami
authors.forename: Sumie
authors.initials: S
authors.lastname: Tabata
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kurata
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsushita
authors.forename: Kenichi
authors.initials: K
authors.lastname: Nagai
authors.forename: Hiroyasu
authors.initials: H
authors.lastname: Ogawa
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001706:Biopsy; D016026:Bone Marrow Transplantation; D016190:Carboplatin / Q000008:administration & dosage; D002945:Cisplatin / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008113:Liver Neoplasms / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D049268:Positron-Emission Tomography; D011239:Prednisolone / Q000008:administration & dosage; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000276:immunology*; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D013601:T-Lymphocytes / Q000276:immunology*; D014057:Tomography, X-Ray Computed; D019172:Transplantation Conditioning; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta; D003561:Cytarabine; D014750:Vincristine; D005047:Etoposide; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; D016190:Carboplatin; D002945:Cisplatin; D007069:Ifosfamide; D008775:Methylprednisolone