A primary antiphospholipid syndrome presented with portal vein thrombosis.

Antiphospholipid Antibody Syndrome (APS) is defined by arterial and venous thrombosis, recurrent spontaneous abortions and thrombocytopenia associated with persistence of antiphospholipid antibodies. Thrombosis may involve vi rtually all arterial or venous sites, but deep vein thrombosis of the lower limbs are the most common; however, unusual thrombi that involve the porta l vein have been described. We report females with documented portal vein t hrombosis and primary APS. The treatment of these patients is difficult bec ause of the risk of bleeding and the recurrent thrombosis if they don't rec eieve appropiate long-term anti-coagulant therapy.