SESSION TYPE: Critical Care Student/Resident Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Vanishing bile duct syndrome (VBDS) is a group of disorders characterized by progressive destruction, ductopenia, and ultimately cholestasis. We describe a case of a 64-year-old woman, diagnosed to have VBDS as a manifestation of Hodgkin’s lymphoma (HL).
CASE PRESENTATION: A 64-year-old women was transferred to our intensive care unit with distributive shock on vasopressor support, cholestatic liver injury, and extensive lymphadenopathy. The patient had jaundice 3 weeks prior to current presentation. Surgical history was significant for cholecystectomy. Physical examination was remarkable for jaundice, and bilateral lower extremity edema. There was no evidence of hepatosplenomegaly, or lymphadenopathy. Laboratory investigations revealed elevated alkaline phosphatase with direct hyperbilirubinemia, and synthetic hepatic dysfunction. Lactate dehydrogenase levels were elevated at 257 U/L . The patient remained febrile, hypotensive, and on norepinephrine; intravenous piperacillin-tazobactam was initiated for presumed biliary sepsis. Patient was intubated and mechanically ventilated. There was no ultrasonographic evidence of ductal obstruction or dilation. A computed tomography illustrated extensive mediastinal, retroperitoneal, and mesenteric lymphadenopathy. Liver core biopsy showed cholestasis with relative paucity of bile ducts, and no evidence of tumor infiltration (Figures 1). Biopsy of the mediastinal lymph node was obtained via video-assisted thoracoscopic surgery; histopathology was consistent with HL (Figures 2). A diagnosis of VBDS was made, as a paraneoplastic manifestation of HL. A regimen of gemcitabine, and carboplatin with dexamethasone was initiated. The patient succumbed to her illness on day 4 of chemotherapy.
DISCUSSION: Ductopenia is present when there is loss of interlobular bile ducts in greater than 50% of small portal tracts. Multiple etiologies, including HL, have been described as cause of ductopenia. Cholestasis related to HL can be classified into two subgroups; VBDS and idiopathic cholestasis (IC). Idiopathic cholestasis is characterized by cholestasis with mixed inflammatory infiltrates without ductopenia, and has a better prognosis than VBDS. A systemic review of 37 cases of HL related VBDS/IC demonstrates good outcomes following definitive therapy in 30% cases . The patients with this disease, therefore, should proceed to definitive treatment of HL.
CONCLUSIONS: Vanishing bile duct syndrome is a cytokine-mediated, paraneoplastic manifestation of HL that presents as cholestatic liver injury. A high index of suspicion is necessary in the right clinical setting, with evidence of ductopenia on liver biopsy to establish the diagnosis. Therapy is targeted at the definitive treatment of HL. The condition is associated with high mortality despite treatment.
1) Ballonoff A, Kavanagh B, et al.Hodgkin lymphoma-related VBDS/IC : statistical analysis of all published cases and literature review. Acta Oncol. 2008;47(5):962-70.
DISCLOSURE: The following authors have nothing to disclose: Nayan Desai, Ankur Kalra, Kamyar Nader, Andres Ferber
No Product/Research Disclosure InformationCooper University Hospital, UMDNJ, Camden, NJ