[Extensive portocava thrombosis revealing a primary antiphospholipid syndrome: a case report].

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.