Nakano Yuta, Kawamoto Ryosuke, Ito Eisaku, Matukawa Kayoko
Department of Nephrology, Ome Municipal General Hospital, Tokyo, JPN.
Department of Nephrology, Tokyo Medical and Dental University, Tokyo, JPN.
Cureus. 2023 Aug 23;15(8):e44001. doi: 10.7759/cureus.44001. eCollection 2023 Aug.
Amyloid light chain (AL) amyloidosis is a rare disorder caused by the deposit of misfolded light chain proteins. AL amyloidosis causes multiple organ involvement and rarely causes fatal liver failure. We present a 68-year-old man who showed cholestatic liver injury and was diagnosed with AL amyloidosis. Due to rapidly progressing cholestatic liver involvement, the patient died five days after the renal biopsy. Preclinically, there was hypercholesterolemia, and levels of gamma-glutamyltransferase (GGT) were elevated. Previous studies have suggested hypercholesterolemia and elevated GGT levels in patients with AL amyloidosis and liver involvement; however, its clinical relevance remains unknown. Our report suggests that in addition to serum kappa/lambda, the combination of new-onset GGT level elevation and hypercholesterolemia could be preclinical characteristics of cholestatic liver involvement in AL amyloidosis.
淀粉样轻链(AL)淀粉样变性是一种由错误折叠的轻链蛋白沉积引起的罕见疾病。AL淀粉样变性会导致多器官受累,很少引起致命性肝功能衰竭。我们报告了一名68岁男性,其出现胆汁淤积性肝损伤,并被诊断为AL淀粉样变性。由于胆汁淤积性肝脏受累迅速进展,该患者在肾活检后五天死亡。临床前,患者存在高胆固醇血症,γ-谷氨酰转移酶(GGT)水平升高。先前的研究表明,AL淀粉样变性合并肝脏受累的患者存在高胆固醇血症和GGT水平升高;然而,其临床相关性仍不清楚。我们的报告表明,除血清κ/λ外,新发GGT水平升高和高胆固醇血症的组合可能是AL淀粉样变性胆汁淤积性肝脏受累的临床前特征。