淀粉样变性：严重胆汁淤积和急性肝衰竭的罕见病因。

Kim Hyun Jae, Tomaszewski Marcel, Lam Eric C, Xiong Wei, Moosavi Sarvee

Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.

Department of Gastroenterology, University of British Columbia, Vancouver, British Columbia, Canada.

ACG Case Rep J. 2020 Dec 4;7(12):e00479. doi: 10.14309/crj.0000000000000479. eCollection 2020 Dec.

Although hepatic involvement in light chain-associated amyloidosis is common, clinical manifestations of hepatic amyloidosis are rare. In most cases, hepatomegaly serves as a clue to diagnosis. We report a unique case of a 48-year-old man from China with jaundice and noncirrhotic portal hypertension, with rapidly progressive liver failure, in the absence of hepatomegaly, secondary to systemic light chain-associated amyloidosis associated with multiple myeloma.

尽管轻链相关性淀粉样变性累及肝脏很常见，但肝淀粉样变性的临床表现却很少见。在大多数情况下，肝肿大是诊断的线索。我们报告了一例独特的病例，一名来自中国的48岁男性，患有黄疸和非肝硬化性门静脉高压症，继发于与多发性骨髓瘤相关的系统性轻链相关性淀粉样变性，在没有肝肿大的情况下出现快速进展的肝功能衰竭。