원발성 소장 림프종 치료 중 발생한 천공으로 수술 후 진단된 장병증 연관 T-세포 림프종 1예
A Case of Enteropathy-Associated T-cell Lymphoma (EATL) Presenting Perforation after Chemotherapy for Primary Intestinal Lymphoma
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초록
원발성 위장관 T-세포 림프종은 드문 질환이며 이의 다양한 조직학적 형태 중 하나인 장병증 연관 T-세포 림프종(enteropathy-associated T-cell lymphoma, EATL)은 흔히 특이적인 점막 궤양을 동반하고 실리악 스프루(celiac sprue)와 관련된 임상양상을 나타내기도 하며 예후도 매우 나쁜 드문 질환이다. 저자들은 갑작스런 혈변으로 내원한 51세 환자에서 대장내시경검사 결과 회장의 궤양성 병변을 발견하였고, 내시경적 생검조직 검사결과 원발성 회장 림프종 진단하에 항암화학요법 중 소장천공이 발생하여 개복수술 및 회장절제 수술을 시행하였으며, 조직학적으로 EATL로 최종 진단된 증례를 경험하였기에 문헌 고찰과 함께 보고한다.
Abstract
Intestinal T-cell lymphomas are fairly uncommon, and can sometimes be associated with enteropathy. Enteropathy- associated T-cell lymphoma (EATL) is commonly accompanied by a nonspecific mucosal ulceration, similar to that observed as a complication of celiac disease. The clinical course of EATL is quite unfavorable, and tends to have a generally poor prognosis. When a tumor invades the bowel wall and is treated with corticosteroids and chemotherapy, cell lysis with perforation often occurs, particularly in case of lymphoma. Recent data indicate that extensive resection may improve local control, and eliminate the risk of early mortality due to visceral perforation or hemorrhaging in unresected lesions during chemotherapy. Here, we report the case of a 51-year-old male who was diagnosed with primary gastrointestinal lymphoma after colonoscopy, and presented with EATL after emergent exploratory laparatomy. We also include a review of the literature regarding this uncommon entity. (Korean J Gastrointest Endosc 2006;32:287-292)
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