ANTIPHOSPHOLIPID-SYNDROME AS THE 2ND CAUSE OF NON-TUMOROUS BUDD-CHIARI-SYNDROME

In a prospective study of 22 patients with non-tumorous Budd-Chiari sy ndrome, four were found to have the antiphospholipid syndrome with no other cause of hepatic vein thrombosis. Ail four patients were young w omen. The antiphospholipid syndrome was secondary to systemic lupus in one case, to a ''lupus-like disease'' in another, and apparently prim ary in the remaining two cases. Two patients died. The other two are i n good health on chronic oral anticoagulation. In our experience, the antiphospholipid syndrome is a frequent cause, after myeloproliferativ e disorders, of non-tumorous Budd-Chiari syndrome. In such patients, l ong-term anticoagulation may prevent recurrence or extension of thromb osis. (C) Journal of Hepatology.