ACUTE BUDD-CHIARI SYNDROME TREATED BY LIVER-TRANSPLANTATION IN A WOMAN HOMOZYGOUS FOR FACTOR-V LEIDEN

We describe the first case of Budd-Chiari syndrome due to homozygosity for factor V Leiden resulting in resistance to activated protein C. T his is now recognized as the most common procoagulant disorder, and ma y account for many cases of Budd-Chiari syndrome previously thought to be idiopathic or due to a latent myeloproliferative disorder. A furth er unique feature of this case is that the patient required orthotopic liver transplantation following failure of portacaval shunting and pr ogressive hepatic necrosis. We demonstrated that liver transplantation resulted in correction of the serum coagulation abnormality; however, it is unlikely to have cured the disorder as platelet factor V would still be of the Leiden phenotype.