Two cases of young patients with the chronic form of Budd-Chiari syndrome are reported. The first concerns a 22-year-old woman with a 6-month history of hepatomegaly, who had used oral contraceptives almost continuously during the five years preceding diagnosis. In a thorough diagnostic work-up, thromboses were detected in all but one of the hepatic veins, and a possible non-occluding thrombosis in the retrohepatic portion of the inferior vena cava. In the blood and bone marrow, findings were compatible with polycythaemia rubra vera, and a high anti-cardiolipin antibody titre was found. The second case concerns a 25-year-old male smoker with normal bone marrow, who had thromboses in at least two of the hepatic veins, though the inferior vena cava was not occluded. In both cases a mesocaval shunt was interposed with synthetic grafts, and postoperatively the patients are doing well--at sixteen and five months, respectively. Both are maintained on anticoagulants, and even without diuretics there has been no recurrence of ascites. The woman takes a small dose of hydroxy-urea to control her hypercoagulability. To our knowledge, hers is the first case to be reported of Budd-Chiari syndrome with hypercoagulability due to the concomitant presence of oral contraceptives, polycythaemia rubra vera and anti-phospholipid antibodies.

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