胃淀粉样变性伴多发性骨髓瘤:上消化道出血的罕见原因

IF 0.3 Q4 GASTROENTEROLOGY & HEPATOLOGY
Huei-Ling Fang, Cheng-Kuan Lin, Yuan-Bin Yu, Min-Hsiang Chang
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引用次数: 0

摘要

淀粉样变性是指细胞外蛋白质原纤维沉积过多,是一种与组织损伤和功能障碍相关的综合征。轻链淀粉样变性是系统性淀粉样变性中最常见的类型。最常见的器官是肾脏、心脏、肝脏、自主神经系统和胃肠道。系统性AL淀粉样变性患者通常有腹泻和呕吐等胃肠道症状。此外,无症状的胃淀粉样蛋白沉积常见于系统性AL淀粉样变性患者。总结了我院一名80岁女性胃肠道AL淀粉样变性患者的临床表现、实验室结果和治疗。综述了该病的病因、临床特点、诊断、治疗及预后等方面的相关文献。患者因黑便被转诊至上内镜检查数周。可见大面积不规则增厚的胃褶,胃底有溃疡和易碎的息肉样肿块,显示淀粉样蛋白沉积。进一步的检查显示没有涉及其他器官。血清免疫电泳和骨髓活检证实了多发性骨髓瘤,临床上认为它会导致胃淀粉样变性。患者接受了美法仑、泼尼松和沙利度胺治疗。治疗后,黑便得到改善,胃质量变平,血清κ轻链水平和κ/λ比值降低。在没有先前确定的骨髓瘤诊断的患者中,胃淀粉样变性在上内窥镜检查中很少出现。早期发现胃肠道淀粉样变性将最终改善这种罕见疾病患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Gastric amyloidosis associated with multiple myeloma: A rare cause of upper gastrointestinal bleeding

Gastric amyloidosis associated with multiple myeloma: A rare cause of upper gastrointestinal bleeding

Amyloidosis is defined as an excess extracellular deposit of protein fibrils, an associated syndrome with tissue injury and dysfunction. Light chain (AL) amyloidosis is the most common type of systemic amyloidosis. The most frequently involved organs are the kidneys, heart, liver, autonomous nervous system, and gastrointestinal tract. Systemic AL amyloidosis patients often have GI symptoms such as diarrhea and vomiting. Furthermore, asymptomatic gastric amyloid deposits are commonly found in patients with systemic AL amyloidosis. The clinical manifestations, laboratory results, and treatment of an 80-year-old female patient with AL amyloidosis of the gastrointestinal tract in our hospital were summarized. Relevant literatures on the etiology, clinical features, diagnosis, treatment, and prognosis of this disease were reviewed. The patient was referred to upper endoscopy because of melena for weeks. Large areas of irregular thickened gastric folds with overlying ulcers and friable polypoid masses at the fundus were seen, showing amyloid depositions. Further workup revealed that no involvement of other organs. Serum immunoelectrophoresis and bone marrow biopsy confirmed multiple myeloma, which was clinically thought to cause gastric amyloidosis. The patient was treated with melphalan, prednisolone, and thalidomide. After treatment, the melena was improved, the flattening gastric mass and a reduction in the serum kappa light chain level and kappa/lambda ratio were observed. Gastric amyloidosis is rarely seen at upper endoscopy in patients without a previously established diagnosis of myeloma. Early detection of gastrointestinal amyloidosis will ultimately improve the outcomes of patients with the rare disease.

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来源期刊
Advances in Digestive Medicine
Advances in Digestive Medicine GASTROENTEROLOGY & HEPATOLOGY-
自引率
33.30%
发文量
42
期刊介绍: Advances in Digestive Medicine is the official peer-reviewed journal of GEST, DEST and TASL. Missions of AIDM are to enhance the quality of patient care, to promote researches in gastroenterology, endoscopy and hepatology related fields, and to develop platforms for digestive science. Specific areas of interest are included, but not limited to: • Acid-related disease • Small intestinal disease • Digestive cancer • Diagnostic & therapeutic endoscopy • Enteral nutrition • Innovation in endoscopic technology • Functional GI • Hepatitis • GI images • Liver cirrhosis • Gut hormone • NASH • Helicobacter pylori • Cancer screening • IBD • Laparoscopic surgery • Infectious disease of digestive tract • Genetics and metabolic disorder • Microbiota • Regenerative medicine • Pancreaticobiliary disease • Guideline & consensus.
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