INTESTINE Vol.19 No.3(2-5)

特集名 腸管悪性リンパ腫
題名 臨床 (5) 消化管T細胞性リンパ腫の診断と治療─enteropathy-associated T-cell lymphomaおよびadult T-cell leukemia/lymphoma
発刊年月 2015年 05月
著者 高津 典孝 福岡大学筑紫病院消化器内科
著者 松井 敏幸 福岡大学筑紫病院消化器内科
著者 今村 健太郎 福岡大学筑紫病院病理部
著者 岩下 明德 福岡大学筑紫病院病理部
【 要旨 】 enteropathy-associated T-cell lymphoma;EATL(腸管症型T細胞性リンパ腫)は,腸管上皮内Tリンパ球を発生起源とする特徴的な疾患群で,空腸と回腸が好発部位である.消化管穿孔を契機に診断されることも多く,その予後はきわめて不良とされていたが,近年,バルーン内視鏡やカプセル内視鏡を用いた早期診断例も報告され,予後改善が期待される.adult T-cell leukemia/lymphoma;ATLL(成人T細胞性白血病/リンパ腫)は,大部分が全身疾患としての消化管病変であり,多彩な形態の病変を形成する.ATLLは,通常の化学療法に抵抗性で予後不良であったが,近年,同種造血幹細胞移植が長期生存を期待できる治療法として推奨され,ヒト化抗CCR4抗体の有効性も期待されている.
Theme Intestinal malignant lymphoma
Title Diagnosis and treatment of intestinal T cell lymphoma
Author Noritaka Takatsu Department of Gastroenterology, Fukuoka University Chikushi Hospital
Author Toshiyuki Matsui Department of Gastroenterology, Fukuoka University Chikushi Hospital
Author Kentaro Imamura Department of Pathology, Fukuoka University Chikushi Hospital
Author Akinori Iwashita Department of Pathology, Fukuoka University Chikushi Hospital
[ Summary ] Enteropathy-associated T-cell lymphoma (EATL) is a primary extranodal T-cell lymphoma arising in the intestine originating from intraepithelial T cells. EATL most commonly affects the jejunum and ileum. Approximately 40 % of patients seek care because of an acute abdomen owing to intestinal obstruction and/or perforation caused by a tumor, with acute peritonitis requiring an emergency surgical procedure. Septicemia due to acute peritonitis accounts for the dismal clinical prognosis in these cases.
Recently, some cases of EATL have been reported, in which early diagnosis was made possible by an endoscopic modality such as double-balloon enteroscopy and/or capsule endoscopy. For the optimal treatment of EATL, it is important to diagnose the disease at an early stage, by using these endoscopic modalities.
Systemic adult T-cell leukemia/lymphoma (ATLL), caused by the human T-lymphotropic virus type 1 (HTLV-1), often presents with secondary involvement of the gastrointestinal tract primary gastrointestinal T-cell lymphoma associated with HTLV-1 is rare. Systemic ATLL leads to various lesions in the gastrointestinal tract.
Patients with acute or lymphomatous-type ATLL have extremely poor prognosis, mainly because of resistance to conventional chemotherapies. Recently, allogeneic hematopoietic stem cell transplantation (HSCT) and a humanized anti-CCR4 monoclonal antibody have been explored as promising alternatives that can provide long-term remission in a subset of patients with ATLL.