{"title":"AL 淀粉样变性的胃十二指肠受累:病例报告与文献综述","authors":"Rajarajeshwari Ramachandran, Tyler Grantham, Giovannie Isaac-Coss, Denzil Etienne, Madhavi Reddy","doi":"10.1177/23247096241237759","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":16198,"journal":{"name":"Journal of investigative medicine high impact case reports","volume":null,"pages":null},"PeriodicalIF":0.9000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10929022/pdf/","citationCount":"0","resultStr":"{\"title\":\"Gastroduodenal Involvement in AL Amyloidosis: Case Report and Literature Review.\",\"authors\":\"Rajarajeshwari Ramachandran, Tyler Grantham, Giovannie Isaac-Coss, Denzil Etienne, Madhavi Reddy\",\"doi\":\"10.1177/23247096241237759\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":16198,\"journal\":{\"name\":\"Journal of investigative medicine high impact case reports\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10929022/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of investigative medicine high impact case reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1177/23247096241237759\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of investigative medicine high impact case reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/23247096241237759","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
摘要
胃肠道淀粉样变性是一种罕见病,常见于全身性 AL 淀粉样变性。淀粉样蛋白可沉积于整个胃肠道,根据沉积部位的不同,所导致的症状也不尽相同。胃肠道(GI)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、蠕动障碍和梗阻。本病例描述了一名已知有 IgG λ AL 淀粉样变性病史的患者,因上腹疼痛和意外消瘦而被发现患有胃十二指肠淀粉样变性。胃十二指肠淀粉样变性的明确诊断需要内镜活检和刚果红染色,并在偏光显微镜下观察。目前还没有治疗消化道淀粉样变性的具体指南。一般来说,目标是治疗淀粉样变性的潜在病因,同时对症治疗。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,由于肾病进展,患者开始接受血液透析。
Gastroduodenal Involvement in AL Amyloidosis: Case Report and Literature Review.
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.
期刊介绍:
The AFMR is committed to enhancing the training and career development of our members and to furthering its mission to facilitate the conduct of research to improve medical care. Case reports represent an important avenue for trainees (interns, residents, and fellows) and early-stage faculty to demonstrate productive, scholarly activity.