| 特集名 | 腸管悪性リンパ腫 | |
|---|---|---|
| 題名 | 臨床 (4) Mantle cell lymphoma | |
| 発刊年月 | 2015年 05月 | |
| 著者 | 岡田 裕之 | 岡山大学病院光学医療診療部 |
| 著者 | 岩室 雅也 | 岡山大学大学院医歯薬学総合研究科消化器・肝臓内科学 |
| 著者 | 田中 健大 | 岡山大学大学院医歯薬学総合研究科病理学 |
| 著者 | 近藤 英生 | 岡山大学大学院医歯薬学総合研究科血液・腫瘍・呼吸器内科学 |
| 著者 | 吉野 正 | 岡山大学大学院医歯薬学総合研究科病理学 |
| 【 要旨 】 | Mantle cell lymphoma（MCL）はリンパ濾胞のマントル層を構成するB細胞性リンパ腫である．遺伝子異常としてはt（11；14）（q13；q32）転座がある．約75％が診断時にすでにⅢ/Ⅳ期の進行期であると報告されており，消化管浸潤は高率に認められる．腸管病変としてはmultiple lymphomatous polyposisを呈する場合が多い．MCLの生存率の中央値は48～68カ月と報告されている．通常のCHOP療法，あるいはリツキシマブ併用CHOP療法の治療強度を高めるためにhyper-CVAD/MA療法が考案され，長期生存がみられている．また，本治療後に自己末梢血幹細胞移植併用の大量化学療法を行うことにより，完全寛解率，無再発生存率が向上することが報告されている． | |
| Theme | Intestinal malignant lymphoma | |
|---|---|---|
| Title | Mantle cell lymphoma | |
| 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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