Vanishing bile duct syndrome following pembrolizumab infusion: case report and review of the literature

Immunotherapy ◽  
2021 ◽  
Author(s):  
Maria Gemelli ◽  
Marco Carbone ◽  
Maria I Abbate ◽  
Maddalena Mancin ◽  
Nicola Zucchini ◽  
...  

PD-1/PD-L1 inhibitors demonstrate high efficacy in non-small-cell lung cancer and are now routinely used in clinical practice. Severe immune-related adverse events are reported in about 5% of patients, requiring hospitalization and possibly leading to death. We present a rare case of vanishing bile duct syndrome that arose a few days after the first pembrolizumab infusion. Laboratory tests and radiological imaging studies were performed to orient diagnosis and monitor the disease, while the evidence of ductal loss on the histological sample was pathognomonic for vanishing bile duct syndrome. High-dose steroid therapy and immunosuppressors were administered, resulting in scarce efficacy. Prompt recognition and management of similar conditions is crucial to avoid fatal events. Further studies are needed to investigate new drugs for steroid-refractory conditions.

2000 ◽  
Vol 118 (5) ◽  
pp. 154-157 ◽  
Author(s):  
Marta Soares Rossini ◽  
Irene Lorand-Metze ◽  
Gislaine Borba Oliveira ◽  
Cármino Antonio De Souza

CONTEXT: Liver damage is relatively common in patients affected by Hodgkin's disease. A smaller proportion of cases develops jaundice. Recently, the vanishing bile duct syndrome was described in Hodgkin's disease. The mechanisms of this severe complication have been poorly understood until now. OBJECTIVE: To describe a rare case of intra-hepatic cholestasis due to vanishing bile duct syndrome. DESIGN: Case report. CASE REPORT: A 38-year-old male patient affected by Hodgkin's disease. Liver biopsy showed no detectable Hodgkin's disease. Intra-hepatic cholestasis was found and none of the six portal tracts analyzed contained normal bile ducts. The treatment was based on conventional and high-dose escalation chemotherapy. The patient died from an irreversible liver failure while in complete remission from Hodgkin's disease.


2013 ◽  
Vol 99 (4) ◽  
pp. e164-e168 ◽  
Author(s):  
Kamyar Nader ◽  
Shaffer Mok ◽  
Ankur Kalra ◽  
Antoine Harb ◽  
Roland Schwarting ◽  
...  

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Sorbarikor Piawah ◽  
Colby Hyland ◽  
Sarah E. Umetsu ◽  
Laura J. Esserman ◽  
Hope S. Rugo ◽  
...  

2018 ◽  
Vol 5 (1) ◽  
pp. e95
Author(s):  
Daniel Sedhom ◽  
Amanda Herrmann ◽  
Melroy DʼSouza ◽  
Billie Fyfe ◽  
Francisco Zaldana ◽  
...  

Hepatology ◽  
1987 ◽  
Vol 7 (3) ◽  
pp. 476-483 ◽  
Author(s):  
Jurgen Ludwig ◽  
Russell H. Wiesner ◽  
Kenneth P. Batts ◽  
James D. Perkins ◽  
Ruud A. F. Krom

Author(s):  
Fernando Bessone ◽  
Nidia Hernández ◽  
Mario Tanno ◽  
Marcelo G. Roma

AbstractThe most concerned issue in the context of drug/herb-induced chronic cholestasis is vanishing bile duct syndrome. The progressive destruction of intrahepatic bile ducts leading to ductopenia is usually not dose dependent, and has a delayed onset that should be suspected when abnormal serum cholestasis enzyme levels persist despite drug withdrawal. Immune-mediated cholangiocyte injury, direct cholangiocyte damage by drugs or their metabolites once in bile, and sustained exposure to toxic bile salts when biliary epithelium protective defenses are impaired are the main mechanisms of cholangiolar damage. Current therapeutic alternatives are scarce and have not shown consistent beneficial effects so far. This review will summarize the current literature on the main diagnostic tools of ductopenia and its histological features, and the differential diagnostic with other ductopenic diseases. In addition, pathomechanisms will be addressed, as well as the connection between them and the supportive and curative strategies for ductopenia management.


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