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伴有小肠反复出血和穿孔的肠病相关性T细胞淋巴瘤。

Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation.

作者信息

Pun Amy H, Kasmeridis Harry, Rieger Nicholas, Loganathan Arun

机构信息

Discipline of Surgery, The Queen Elizabeth Hospital, Adelaide, SA, Australia.

Anatomical Pathology, The Queen Elizabeth Hospital, Adelaide, SA, Australia.

出版信息

J Surg Case Rep. 2014 Mar 20;2014(3):rju013. doi: 10.1093/jscr/rju013.

Abstract

Enteropathy-associated T cell lymphoma (EATCL) is an intestinal neoplasm of intra-epithelial T lymphocytes associated with coeliac disease. Although the incidence is rare, EATCL runs an aggressive disease course and produces multi-focal ulcerative lesions most commonly in the proximal small bowel. As such, patients may present with intestinal perforation, obstruction or haemorrhage. Management of EATCL requires a combination of early diagnosis and treatment by surgical resection followed by chemotherapy to achieve treatment success. Overall however, the treatment completion rate remains at 50% and EATCL carries a poor prognosis with a 5-year survival rate of <20%.

摘要

肠病相关T细胞淋巴瘤(EATCL)是一种与乳糜泻相关的上皮内T淋巴细胞肠道肿瘤。尽管发病率很低,但EATCL病程进展迅速,最常于近端小肠产生多灶性溃疡性病变。因此,患者可能出现肠穿孔、肠梗阻或出血。EATCL的治疗需要早期诊断并结合手术切除及化疗,以取得治疗成功。然而总体而言,治疗完成率仍为50%,EATCL预后较差,5年生存率<20%。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec73/4197923/8a63a0daf8ba/rju01301.jpg

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