Extensive portocava thrombosis revealing a primary antiphospholipid syndrome: a case report

• Published in: La revue de medecine interne Vol. 29; no. 6; pp. 504 - 507
• Main Authors: Belkahla, N, Maamouri, N, Ouerghi, H, Cheikh, I, Hamida, S Ben, Bouzid, H, Ammar, A B
• Format: Journal Article
• Language: English; French
• Published: France 01.06.2008
• Subjects: Adult; Antibodies, Anticardiolipin - blood; Antibodies, Antiphospholipid - blood; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Antiphospholipid Syndrome - immunology; Budd-Chiari Syndrome - diagnosis; Budd-Chiari Syndrome - etiology; Budd-Chiari Syndrome - immunology; Fatal Outcome; Female; Hepatorenal Syndrome - etiology; Humans; Liver Failure - etiology; Portal Vein - pathology; Vena Cava, Inferior - pathology; Venous Thrombosis - diagnosis; Venous Thrombosis - etiology
• ISSN: 0248-8663
• DOI: 10.1016/j.revmed.2008.01.005

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.