The authors report a 5-year-old boy with Hodgkin disease and cholestatic ja
undice 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 xanthomat
a. The jaundice was found to be attributable to vanishing bile duct syndrom
e (VBDS). The extent of hepatic dysfunction precluded appropriate treatment
of the lymphoma with chemotherapy, and the boy died of liver failure. In t
he differential diagnosis of jaundice in children with Hodgkin disease, VBD
S should be considered.