Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 14, 2017; 23(2): 366-372
Published online Jan 14, 2017. doi: 10.3748/wjg.v23.i2.366
Vanishing bile duct syndrome in Hodgkin’s lymphoma: A case report and literature review
Mena Bakhit, Thomas R McCarty, Sunhee Park, Basile Njei, Margaret Cho, Raffi Karagozian, AnnMarie Liapakis
Mena Bakhit, Thomas R McCarty, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06520, United States
Sunhee Park, Basile Njei, AnnMarie Liapakis, Section of Digestive Diseases, Yale University School of Medicine, New Haven, CT 06520, United States
Basile Njei, Investigative Medicine Program, Yale Center of Clinical Investigation, New Haven, CT 06520, United States
Margaret Cho, Department of Anatomic Pathology, Yale University School of Medicine, New Haven, CT 06520, United States
Raffi Karagozian, Saint Francis Hospital, Gastroenterology and Hepatology, Hartford, CT 62048, United States
Author contributions: All authors contributed equally to this work.
Supported by National Institute of Health, NIH 5 T32 DK 7356-37 (BN).
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at Yale-New Haven Hospital.
Conflict-of-interest statement: No authors have personal, financial, or other conflicts of interest to disclose.
Open-Access: 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/
Correspondence to: AnnMarie Liapakis, MD, Section of Digestive Diseases, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06520, United States. annmarie.liapakis@yale.edu
Telephone: +1-860-6793878 Fax: +1-860-6793159
Received: April 26, 2016
Peer-review started: April 26, 2016
First decision: June 20, 2016
Revised: July 4, 2016
Accepted: August 5, 2016
Article in press: August 5, 2016
Published online: January 14, 2017
Processing time: 261 Days and 5.8 Hours
Abstract

Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.

Keywords: Cholestasis; Bile ductopenia; Vanishing bile duct syndrome; Hodgkin’s lymphoma; Liver

Core tip: Vanishing bile duct syndrome (VBDS) is a rare form of liver injury and can be caused by multiple etiologies including malignancy. It is therefore critical for physicians to create a broad differential when VBDS is suspected and diagnosed. Liver biopsy is critical and should not be deferred. Once the diagnosis of VBDS is confirmed on biopsy, aggressive therapy, adjunctive medical management of cholestasis, and supportive care is indicated as achieving remission and symptom management in Hodgkin’s lymphoma -associated VBDS is crucial. If hepatic recovery does not occur, liver transplantation should be considered.