INTESTINE Vol.19 No.3(2-4)

Theme Intestinal malignant lymphoma
Title Mantle cell lymphoma
Publish Date 2015/05
Author Hiroyuki Okada Department of Endoscopy, Okayama University Hospital
Author Masaya Iwamuro Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Author Takehiro Tanaka Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Author Eisei Kondo Department of Hematology, Oncology and Respiratory Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
Author Tadashi Yoshino Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences
[ Summary ] Mantle cell lymphoma (MCL) is a B-cell lymphoma derived from the lymph node follicle mantle zone. Specific gene abnormalities associated with this disease include the t(11;14)(q13;q32) translocation. About 75 % of MCL cases are diagnosed at advanced stage (III or IV), with a high rate of gastrointestinal involvement.
In a review of 71 Japanese patients with gastrointestinal involvement from MCL, 67 % of the cases were diagnosed at stage IV. Gastrointestinal involvement included stomach (2/3 of the cases) and colon (2/5 of the cases). Esophageal involvement was uncommon (only 4 cases). MCL was often observed in the intestine as multiple lymphomatous polyposis (MLP).
The prognosis of MCL is the poorest among all B-cell lymphoma types, with a median survival of 48-68 months. The hyper-CVAD/MA regimen (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternated with high doses of methotrexate and cytarabine) was designed to improve the therapeutic effects of the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone) or of rituximab plus CHOP. Following this treatment, high-dose chemotherapy with autologous peripheral blood stem cell transplantation is performed to improve complete remission rates and relapse-free survival.
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