title: Vanishing bile duct syndrome in a Hodgkin's lymphoma patient with fatal outcome despite lymphoma remission.
abstract: Vanishing bile duct syndrome (VBDS) is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts (ductopenia) leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma (HL). We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.
authors.affiliation: Department of Medicine, Division of Hematology/Oncology, College of Medicine and King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
authors.forename: Aamer
authors.initials: A
authors.lastname: Aleem
authors.forename: Mohammad
authors.initials: M
authors.lastname: Al-Katari
authors.forename: Khalid
authors.initials: K
authors.lastname: Alsaleh
authors.forename: Khalid
authors.initials: K
authors.lastname: AlSwat
authors.forename: Abdulmalik
authors.initials: A
authors.lastname: Al-Sheikh
mesh_terms: D000328:Adult; D001649:Bile Duct Diseases / Q000150:complications* / Q000175:diagnosis; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D001706:Biopsy; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D006689:Hodgkin Disease / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D017093:Liver Failure / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D013577:Syndrome