Vanishing bile duct syndrome as a manifestation of Hodgkin's lymphoma: a case report and review of the literature.

Abstract

Vanishing bile duct syndrome (VBDS) is characterized by cholestasis and progressive destruction of the intrahepatic bile ducts (ductopenia). The current definition of ductopenia is the loss of interlobular bile ducts in more than 50% of portal tracts. Ductopenia is believed, at a molecular level, to result from the misbalance in cell regeneration and apoptosis. In the literature various etiologies have been reported to cause ductopenia, with Hodgkin's lymphoma (HL) being listed as a rare example. How HL causes ductopenia remains ambiguous, and seems to be related to a paraneoplastic phenomenon causing cytokine release from lymphoma cells, not tumor infiltration or obstructive lymphadenopathy. VBDS is generally considered irreversible, unlike its histopathological counterpart, idiopathic cholestasis, where ductopenia is not present and liver function improves with therapy. Therefore, a distinction between the two is warranted. There have been only 19 case reports in the English literature associating VBDS with HL. Here we report a 64-year-old female patient who presented with distributive shock and jaundice. Initial laboratory values revealed leukocytosis, mild transaminase elevation with significantly elevated alkaline phosphatase, along with direct hyperbilirubinemia. During hospital stay, the patient's liver function progressively worsened. Further workup did not reveal ductal dilation or obstruction and there were unremarkable results for infectious and autoimmune etiologies. Imaging studies with biopsy revealed extensive lymphadenopathy consistent with HL; liver biopsy showed cholestasis and ductopenia. Despite chemotherapy the patient succumbed to progressive liver failure and sepsis.