Bhanot Kunal, Taheem Muhesh, Perry Robert, Wong Christopher, Muehlschlegel Charlotte, Cheent Kuldeep
Department of Gastroenterology, Frimley Park Hospital, Portsmouth Road, Frimley, Camberley, UK.
Department of Surgery, North Middlesex University Hospital, Sterling Way, London, UK.
J Surg Case Rep. 2022 Jul 8;2022(7):rjac264. doi: 10.1093/jscr/rjac264. eCollection 2022 Jul.
Amyloid light-chain (AL) amyloidosis is a multisystem disease with obstructive jaundice and gastrointestinal (GI) involvement being uncommon initial presentations. Patients with AL amyloidosis seldom have jaundice and advanced GI tract involvement as their presenting symptoms. This case report describes an 82-year-old lady who presented with a 6-month history of early satiety, weight loss, xerostomia and progressive jaundice. Imaging did not suggest a biliary cause but demonstrated hepatomegaly and ascites. Oesophagogastroduodenoscopy revealed a duodenal stricture. Duodenal and liver biopsies were consistent with amyloid deposition. Multiple myeloma was confirmed to be the underlying cause. Significant cholestatic liver dysfunction and a duodenal stricture have not been previously described as simultaneous manifestations of amyloidosis. This case also highlights the difficulty in treating multiple myeloma as the cause of AL amyloidosis in the context of liver dysfunction, given that many chemotherapy agents undergo hepatic metabolism.
淀粉样轻链(AL)淀粉样变性是一种多系统疾病,梗阻性黄疸和胃肠道受累作为初始表现并不常见。AL淀粉样变性患者很少以黄疸和晚期胃肠道受累作为首发症状。本病例报告描述了一位82岁女性,她有6个月的早饱、体重减轻、口干和进行性黄疸病史。影像学检查未提示胆道病因,但显示肝脏肿大和腹水。食管胃十二指肠镜检查发现十二指肠狭窄。十二指肠和肝脏活检与淀粉样沉积一致。确诊潜在病因是多发性骨髓瘤。严重胆汁淤积性肝功能障碍和十二指肠狭窄此前尚未被描述为淀粉样变性的同时表现。本病例还凸显了在肝功能障碍的情况下,将多发性骨髓瘤作为AL淀粉样变性病因进行治疗的困难,因为许多化疗药物都经过肝脏代谢。