Jaundice caused by the vanishing bile duct syndrome in a child with Hodgkin lymphoma

The authors report a 5-year-old boy with Hodgkin disease and cholestatic jaundice that predated the start of treatment for his lymphoma. His clinical course was punctuated by relentless progression of jaundice, characterized by obstructive pattern liver function tests, severe pruritus, intermittent fever, and marked hypercholesterolemia with development of palmar xanthomata. The jaundice was found to be attributable to vanishing bile duct syndrome (VBDS). The extent of hepatic dysfunction precluded appropriate treatment of the lymphoma with chemotherapy, and the boy died of liver failure. In the differential diagnosis of jaundice in children with Hodgkin disease, VBDS should be considered.