Reversible Hepatic Decompensation in Primary Biliary Cirrhosis Due to Hypercoagulability

摘要

Patients with decompensated liver disease due to primary biliary cirrhosis (PBC) rarely recover without liver transplantation. nnObjective: To describe 2 sisters with PBC who developed decompensated liver disease associated with a hypercoagulable state (heterozygosity for the factor V Leiden mutation) that resolved with anticoagulation. nnCase Report: The first patient was a 47-year-old woman with stage II PBC that was diagnosed in 1996. She was asymptomatic, had detectable antimitochondrial antibodies, and had normal serum bilirubin and albumin levels. Despite treatment with ursodiol and colchicine and trials of methotrexate and then prednisone (for a possible overlap syndrome), her liver disease worsened (1). By 2000, she had developed biopsy-proven cirrhosis, ascites, grade-2 esophageal varices, and lower extremity edema. Her albumin level was 26 g/L, platelet count was 64 × 109 cells/L, and prothrombin international normalized ratio was 1.4. Furosemide, spironolactone, and propranolol were added to therapy, and she was evaluated for liver transplantation. Magnetic resonance angiography revealed a right intrahepatic portal venous thrombus. She was found to be heterozygous for the factor V Leiden mutation.