Vanishing Bile Duct Syndrome Associated With Non-Hodgkin’s Lymphoma and Hepatitis E Virus Infection

Abstract

The vanishing bile duct syndrome (VBDS) is a condition secondary to inciting triggers resulting in destruction and eventual disappearance of intrahepatic bile ducts leading to cholestasis. The overall outcome varies and often depends on the nature of the precipitating cause. VBDS has been found to be associated with adverse drug reactions, infectious diseases, autoimmune diseases, ischemia, and humoral factors associated with malignancies and is often irreversible. The objective of this clinical case report is to highlight the need for a broad differential to include VBDS in similar scenarios to aid rapid diagnosis and management. We hope this could lead to a more favourable outcome for patients presenting with VBDS such as the one described in this case report with concurrent non-Hodgkin's lymphoma and infection with hepatitis E virus. To the best of our knowledge, this is the first ever reported case of VBDS associated with non-Hodgkin's lymphoma and hepatitis E virus infection.

Histopathological Features Predicting Long-term Clinical Outcomes in Patients with Vanishing Bile Duct Syndrome

“… 3 , 4 Limited studies, including case reports or small case series of neoplastic diseases (particularly lymphoma) or drug-induced liver injury (DILI) has also associated these etiologies with VBDS. 1 , 5 , 6 In 2017, the DILI Network published a 10-year prospective study on drug or supplement induced bile duct loss and at least 12 cases of VBDS were identified in the 363 patients studied. 7 However, the study was only based on DILI data, which would not include many other potential VBDS etiologies.…”

Histopathological features predicated long-term clinical outcomes in patients with vanishing bile duct syndrome

“…It is regarded as an ominous histological sign usually associated with poor prognosis. At present, VBDS has been primarily described in case reports or small case series in the patients of neoplastic diseases (particularly lymphoma) or in drug-induced liver injury (DILI) [1,3]. Obviously, insu cient information exists on its clinical course, long-term prognosis and histological features associated with poor prognosis.…”