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

Author:

Zhang Xu,Tang Fei,Gao Yan-Ying,Song De-Zhao,Liang Jing

Abstract

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 misdiagnosis 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, additional diagnostic procedures, including liver biopsy and bone marrow-examination, revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression. The disease course was progressive 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.

Publisher

Baishideng Publishing Group Inc.

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. A Case Report of Al-Amyloidosis with «Hepatic Disguise» of Nephrotic Syndrome;The Russian Archives of Internal Medicine;2024-06-12

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