Hepatomegaly and jaundice as the presenting symptoms of systemic light-chain amyloidosis: A case report  

作　　者：Xu Zhang Fei Tang Yan-Ying Gao De-Zhao Song Jing Liang 

机构地区：[1]Department of Gastroenterology and Hepatology,The Third Central Hospital of Tianjin,Tianjin 300170,China [2]Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases,Artificial Cell Engineering Technology Research Center,Tianjin Institute of Hepatobiliary Disease,Tianjin 300170,China [3]Department of Interventional Radiology,The Third Central Hospital of Tianjin,Tianjin 300170,China

出　　处：《World Journal of Gastrointestinal Oncology》2024年第2期550-556,共7页世界胃肠肿瘤学杂志（英文版）（电子版）

基　　金：Tianjin Key Medical Discipline(Specialty)Construction Project,No.TJYXZDXK-034A.

摘　　要：BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.

关 键 词：JAUNDICE HEPATOMEGALY Liver amyloidosis Kappa light chain Pseudo-Budd-Chiari syndrome Case report 

分 类 号：R575[医药卫生—消化系统]