Vanishing Bile Duct Syndrome in the Presence of Hodgkin Lymphoma.

Abstract

Vanishing bile duct syndrome (VBDS) is an acquired condition characterized by the destruction and loss of intrahepatic bile ducts resulting in cholestasis. VBDS has been described in various conditions including neoplastic and immunologic disorders, infections, hepatic ischemia, and drug toxicity. The diagnosis is confirmed by liver biopsy revealing the loss of interlobular bile ducts in greater than 50% of portal tracts. Prognosis is variable and often unpredictable but appears to be influenced by the etiology of bile duct destruction and overall patient health. VBDS has been described as a rare paraneoplastic process in patients with Hodgkin lymphoma. This case describes a 26-year-old female who presented with a neck mass, jaundice, and pruritus. Initial workup revealed direct hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and elevated international normalized ratio. She went on to receive a diagnosis of stage II classical Hodgkin lymphoma, nodular sclerosing subtype, and biopsy-proven VBDS. Over the course of chemotherapy, complete metabolic resolution of Hodgkin lymphoma and complete normalization of bilirubin were achieved. She was given gemcitabine and cyclophosphamide as a liver sparing regimen initially with some improvement in liver function tests and a reduction in lymph node volumes. She received six cycles of adriamycin/bleomycin/vinblastine/dacarbazine (ABVD) with complete remission attained after four cycles by positron emission tomography/computed tomography criteria. This report illustrates asafe chemotherapy regimen in the presence of marked liver dysfunction. Workup for VBDS including liver biopsy should be pursued in Hodgkin lymphoma patients with evidence of cholestasis in the absence of extrahepatic bile duct damage or other known etiology of liver injury.