Vanishing bile duct syndrome in non-Hodgkin lymphoma: a case report.

Non-Hodgkin lymphoma (NHL) related vanishing bile duct syndrome (VBDS) is a rare condition that often leads to liver failure and death. A 64-year-old man with history of rheumatic heart disease complicated by steno-aortic insufficiency, mild mitral stenosis, atrial fibrillation, left kidney damage, bilateral glaucoma, left internal jugular vein thrombosis, bronchopneumonia, NHL type B (stage IV) treated with chemo and radiotherapy was admitted to our department for jaundice with predominantly cholestatic component. Liver biopsy allowed a diagnosis of VBDS and patient was treated with ursodesossicolic acid (UDCA) 20 mg/kg/day associated with prednisone 1 mg/kg/day with a transient decrease of bilirubin and cholestasis, but a rapid worsening of general clinical conditions followed by severe acute liver failure (ALF), unresponsive to pharmacological therapy, leads to death our patient.