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. In our case we supposed that this syndrome represents the paraneoplastic epiphenomenon of NHL with severe duct damage, related to lymphomatous release of toxic cytokines. In our clinical practice we must not forget that VBDS can be related to hepatic damage also induced during NHL. A special attention to abnormal liver function is necessary to diagnose this syndrome, because often the tests are interpreted as disseminated (stage IV) disease and not as a possible expression of VBDS which could, in some cases, be attenuated by UDCA and by steroids therapy.