Hepatosplenic alphabeta T cell lymphoma.
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.
Nagai
Yuya
Y
Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, 4-6 Minatojima-Nakamachi, Chuo-ku, Kobe 650-0046, Japan.
Ikegame
Kazuhiro
K
Mori
Minako
M
Inoue
Daichi
D
Kimura
Takaharu
T
Shimoji
Sonoko
S
Togami
Katsuhiro
K
Tabata
Sumie
S
Kurata
Masayuki
M
Imai
Yukihiro
Y
Matsushita
Akiko
A
Nagai
Kenichi
K
Ogawa
Hiroyasu
H
Takahashi
Takayuki
T
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
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