Vanishing bile duct syndrome : a possible mechanism for intrahepatic cholestasis in Hodgkin's lymphoma

摘要: A syndrome of idiopathic intrahepatic cholestasis occurs in some patients with Hodgkin's lymphoma. The underlying mechanism is poorly understood. In this paper we describe three lymphoma whom severe unknown pathogenesis developed. two cases jaundice was the presenting symptom; all died intractable liver damage. were initially thought to have Hodgkin's-associated cholestasis, but subsequent review histological material revealed advanced vanishing bile duct addition cholestasis. Ten specimens obtained from 4 97 wk after onset (seven needle biopsies, one wedge biopsy, postmortem livers). seven taken within 30 jaundice, portal tracts characteristically had a "burned-out" appearance without secondary periportal changes related chronic Three biopsy more than 1 yr showed evidence progressive damage form fibrous expansion, marginal ductular proliferation and copper-associated protein deposition. More 80% small lacked recognizable ducts final patients. These observations suggest that should be included list diseases associated loss ducts. possibility considered differential diagnosis unexplained disease.