Deeper Insights into Vanishing Bile Duct Syndrome in Lymphoma: A Perplexing Entity

• Vanishing bile duct syndrome is an unusual entity characterized by progressive destruction of the intrahepatic bile ducts (ductopenia), which ultimately results in cholestasis. The pathogenesis is unclear and related to immunologic injury of bile ducts. The prognosis is poor, and the case reports of many patients who died of the illness have been reported. • We present a case to illustrate an important message that timely treatment with appropriate chemotherapy, despite the presence of liver dysfunction, can be lifesaving for critically ill patients with lymphoma associated with vanishing bile duct syndrome. • Along with the case presentation, we provide an extensive review of published studies and the published data of cases to give a better understanding of this entity. Using the data from several of these anecdotal case reports, we formulated clinical guidelines to assist oncologists globally to treat this potentially curable disorder.