Department of Internal Medicine, Loghman Hakim Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Pathology, Loghman Hakim Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Iran J Med Sci. 2022 Jan;47(1):73-77. doi: 10.30476/ijms.2021.88694.1942.
Gastrointestinal amyloidosis is a condition caused by the deposition of extracellular protein fragments. It can be associated with complex and diverse pathways and can have numerous manifestations and etiologies. Hepatic amyloid light-chain (AL) amyloidosis is a rare disorder characterized by the deposition of the insoluble amyloid protein in the liver. The clinical presentations of AL amyloidosis are frequently non-specific. In this case report, we describe a patient with amyloidosis, who initially presented with an unusual case of severe intrahepatic cholestasis, which followed a rapidly progressive clinical course that was associated with the acute hypercalcemic crisis. The diagnosis of amyloidosis was made after the liver and bone biopsies were performed. Our findings revealed that AL amyloidosis should be considered, when a patient presents with cholestatic hepatitis, renal failure, and hypercalcemia.
胃肠道淀粉样变性是一种由细胞外蛋白质片段沉积引起的疾病。它可能与复杂多样的途径有关，并具有多种表现和病因。肝发性轻链（AL）淀粉样变性是一种罕见的疾病，其特征是肝脏中不可溶的淀粉样蛋白沉积。AL 淀粉样变性的临床表现通常是非特异性的。在本病例报告中，我们描述了一例淀粉样变性患者，该患者最初表现为一种不寻常的严重肝内胆汁淤积症，随后出现快速进展的临床病程，并伴有急性高钙血症危象。在进行肝脏和骨活检后诊断为淀粉样变性。我们的研究结果表明，当患者出现胆汁淤积性肝炎、肾衰竭和高钙血症时，应考虑 AL 淀粉样变性。
