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13年的出血情况:肠道内的淀粉样血管病、血管发育异常及后天性凝血障碍。

The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut.

作者信息

Simms Larnelle N, Suarez Laura Suzanne K, Deeb Khaled, Proenza Jose

机构信息

Internal Medicine Resident, University of Miami Palm Beach Regional GME Consortium, Atlantis, FL, USA.

Department of Internal Medicine, West Palm Beach Veterans Affairs Medical Center, West Palm Beach, FL, USA.

出版信息

SAGE Open Med Case Rep. 2021 Sep 1;9:2050313X211040018. doi: 10.1177/2050313X211040018. eCollection 2021.

Abstract

Amyloidosis is a disorder characterized by extracellular deposits of proteins that are prone to aggregate and form insoluble fibrils. Amyloid deposits limited to a single organ or tissue without the involvement of any other site in the body is uncommon. We report a 75-year-old man with previously treated non-Hodgkin's lymphoma who presented with recurrent gastrointestinal hemorrhage. Histopathology showed amyloid deposition within vascular malformations. His bleeding continued with the cause rooted in the fundamental building blocks-clotting factors. We discuss the interplay of the pathophysiology of lymphoma, amyloidosis, and factor X deficiency in a patient with preexisting angiodysplasias leading to refractory gastrointestinal bleeding. To our knowledge, there are only 3 reported cases of concomitant amyloidosis and angiodysplasia in the colon, and none involving the small bowel.

摘要

淀粉样变性是一种以蛋白质细胞外沉积为特征的疾病,这些蛋白质易于聚集并形成不溶性纤维。淀粉样沉积物局限于单个器官或组织而不涉及身体其他任何部位的情况并不常见。我们报告了一名75岁男性,既往有非霍奇金淋巴瘤治疗史,出现反复胃肠道出血。组织病理学显示血管畸形内有淀粉样沉积。他的出血持续存在,病因源于凝血因子这一基本要素。我们讨论了一名已有血管发育异常的患者中淋巴瘤、淀粉样变性和X因子缺乏的病理生理相互作用,这种相互作用导致难治性胃肠道出血。据我们所知,仅有3例结肠同时存在淀粉样变性和血管发育异常的报道,且均未累及小肠。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cfc4/8414616/9c412b2df301/10.1177_2050313X211040018-fig1.jpg

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