ABSTRACT
Vanishing bile duct syndrome [VBDS] is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts [ductopenia] leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma [HL]. We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.
Subject(s)
Humans , Male , Hodgkin Disease/complications , Cholestasis/etiology , Fatal Outcome , Bile Duct Diseases/diagnosis , Bile Duct Diseases/etiology , Bile Duct Diseases/mortality , Hyperbilirubinemia/etiology , Antineoplastic Combined Chemotherapy ProtocolsABSTRACT
A colangite é uma afecção do trato biliar com significativa morbi-mortalidade associada. Muitos pacientes com colangite aguda respondem à antibioticoterapia. Entretanto, aqueles com a forma severa ou tóxica da doença usualmente não respondem, necessitando de drenagem biliar de emergência