Abstract
We report a 20-year-old woman who presented with a massive portal thrombosis that rapidly extended to the superior and inferior vein cava system causing an acute Budd-Chiari syndrome. The investigations concluded to a primary antiphospholipid syndrome without any other prothrombotic factors. The outcome was fatal, 18 months later, despite anticoagulation, with hepatorenal syndrome and severe liver failure. (c) 2008 Elsevier Masson SAS. Tous droits reserves.