Gastrointestinal mantle cell lymphoma

Mantle cell lymphoma (MCL) is a B cell lymphoma derived from the mantle zone of the lymph node follicle. MCL is immunohistochemically positive for CD5 and cyclin D1 and negative for CD10. These markers are useful for a definitive diagnosis. Specific gene abnormalities include a t (11 ; 14) (q13 ; q32) translocation. About 75% of MCL cases are diagnosed at an advanced stage III or IV, with gastrointestinal involvement detected at a high rate. A review of 71 Japanese patients with gastrointestinal involvement of MCL revealed that 67% were diagnosed at stage IV. Gastrointestinal involvement included stomach (2/3) and colon lesions (2/5). Esophageal involvement was uncommon (4 cases). Stomach lesions varied morphologically, including protruded, ulcerative, and fold-thickening types and multiple lymphomatous polyposis (MLP). MCL is often observed as MLP from the duodenum to the colon. The prognosis of MCL is poorer among B cell lymphoma cases, with a median survival of 48-68 months. A hyper-CVAD/MA regimen (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternating with high doses of methotrexate and cytarabine) was designed to improve the therapeutic effects of the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) or rituximab plus CHOP. Following this therapy, high-dose chemotherapy with autologous peripheral blood stem cell transplantation is performed to improve the rates of complete remission and relapse-free survival.