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胃十二指肠受累于AL型淀粉样变性:病例报告及文献综述

Gastroduodenal Involvement in AL Amyloidosis: Case Report and Literature Review.

作者信息

Ramachandran Rajarajeshwari, Grantham Tyler, Isaac-Coss Giovannie, Etienne Denzil, Reddy Madhavi

机构信息

Brooklyn Hospital Center, NY, USA.

Staten Island University Hospital, NY, USA.

出版信息

J Investig Med High Impact Case Rep. 2024 Jan-Dec;12:23247096241237759. doi: 10.1177/23247096241237759.

Abstract

Gastrointestinal amyloidosis is a rare condition commonly found in the setting of systemic AL amyloidosis. Amyloid can deposit throughout the gastrointestinal tract and the resulting symptoms vary depending on the site of deposition. Gastrointestinal (GI) manifestations can range from weight loss or abdominal pain, to more serious complications like gastrointestinal bleeding, malabsorption, dysmotility, and obstruction. This case describes a patient with known history of IgG lambda AL amyloidosis, presenting with epigastric pain and unintentional weight loss found to have gastroduodenal amyloidosis. The definitive diagnosis of GI amyloidosis requires endoscopic biopsy with Congo red staining and visualization under polarized light microscopy. There are currently no specific guidelines for the management of GI amyloidosis. Generally, the goal is to treat the underlying cause of the amyloidosis along with symptom management. Our patient is being treated with cyclophosphamide, bortezomib, and dexamethasone (CyBorD) and started on hemodialysis due to progression of renal disease.

摘要

胃肠道淀粉样变性是一种罕见疾病,常见于系统性轻链型淀粉样变性的背景下。淀粉样物质可沉积于整个胃肠道,其产生的症状因沉积部位而异。胃肠道表现可从体重减轻或腹痛,到更严重的并发症,如胃肠道出血、吸收不良、动力障碍和梗阻。本病例描述了一名已知患有IgG λ轻链型淀粉样变性的患者,出现上腹部疼痛和非故意体重减轻,经检查发现患有胃十二指肠淀粉样变性。胃肠道淀粉样变性的确诊需要进行内镜活检,刚果红染色并在偏振光显微镜下观察。目前尚无针对胃肠道淀粉样变性治疗的具体指南。一般来说,目标是治疗淀粉样变性的潜在病因并进行症状管理。我们的患者正在接受环磷酰胺、硼替佐米和地塞米松(CyBorD)治疗,并因肾病进展开始进行血液透析。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6053/10929022/ed74be56dc2a/10.1177_23247096241237759-fig1.jpg

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Scand J Gastroenterol. 2009;44(6):708-11. doi: 10.1080/00365520902783717.

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