Zhang X, Tang F, Gao YY, Song DZ, Liang J. Hepatomegaly and jaundice as the presenting symptoms of systemic light-chain amyloidosis: A case report. World J Gastrointest Oncol 2024; 16(2): 550-556 [PMID: 38425387 DOI: 10.4251/wjgo.v16.i2.550]
Corresponding Author of This Article
Jing Liang, MD, Chief Physician, Department of Gastroenterology and Hepatology, The Third Central Hospital of Tianjin, No. 83 Jintang Road, Tianjin 300170, China. haolele77@sina.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Xu Zhang, Fei Tang, Yan-Ying Gao, Jing Liang, Department of Gastroenterology and Hepatology, The Third Central Hospital of Tianjin, Tianjin 300170, China
Xu Zhang, Fei Tang, Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases, Artificial Cell Engineering Technology Research Center, Tianjin Institute of Hepatobiliary Disease, Tianjin 300170, China
De-Zhao Song, Department of Interventional Radiology, The Third Central Hospital of Tianjin, Tianjin 300170, China
Co-first authors: Xu Zhang and Fei Tang.
Author contributions: Zhang X and Tang F contributed to the writing-original draft preparation; Gao YY and Song DZ contributed to the patient diagnosis and treatment; Song DZ contributed to the performing transjugular liver biopsy and balloon dilatation of the hepatic vein; Liang J contributed to the writing-reviewing and editing; Zhang X and Tang F contributed equally to this work as co-first authors; All authors have read and approve the final manuscript.
Supported byTianjin Key Medical Discipline (Specialty) Construction Project, No. TJYXZDXK-034A.
Informed consent statement: The patient provided informed written consent prior to this manuscript.
Conflict-of-interest statement: The authors declare no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jing Liang, MD, Chief Physician, Department of Gastroenterology and Hepatology, The Third Central Hospital of Tianjin, No. 83 Jintang Road, Tianjin 300170, China. haolele77@sina.com
Received: October 1, 2023 Peer-review started: October 1, 2023 First decision: December 5, 2023 Revised: December 11, 2023 Accepted: December 26, 2023 Article in press: December 26, 2023 Published online: February 15, 2024 Processing time: 123 Days and 19.4 Hours
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.
Core Tip: Light chain (AL) amyloidosis is a systemic disease, with heart, kidneys, and peripheral nerves being the most commonly affected organs. The proportion of patients with only liver involvement alone is quite low, and these patients are highly prone to misdiagnosis and missed diagnosis. We present a case of AL amyloidosis with isolated liver involvement and severe cholestasis as the predominant manifestations.
