Vanishing Bile Duct Syndrome in a Patient With Recurrent Hodgkin Lymphoma.

Abstract

Vanishing bile duct syndrome (VBDS) is an acquired syndrome characterized by clinical and laboratory signs of cholestasis with pathologic findings of interlobular bile duct paucity in liver biopsy specimens. VBDS can result from a variety of conditions including infections, autoimmune diseases, adverse drug reactions, and neoplastic processes. Hodgkin lymphoma (HL) is a rare cause of VBDS. The mechanism by which HL leads to VBDS remains unknown. Development of VBDS in patients with HL portends an extremely poor prognosis due to the risk of progression to fulminant hepatic failure. Treatment of the underlying lymphoma has been demonstrated to offer increased probability of recovery from VBDS. The decision to treat and choice of treatment of the underlying lymphoma is often complicated by the hepatic dysfunction characteristic of VBDS. We present the case of a patient who presented with dyspnea and jaundice in the context of recurrent HL and VBDS. We additionally review the literature on HL complicated by VBDS with specific focus on treatment paradigms for management of these patients.