Liver transplantation in a patient with mild haemophilia A and low-titres of factor VIII inhibitors treated with recombinant factor VIIa. The first Argentinean case.

摘要

Haemophilia A (HA) is a disease produced by a deficiency of factor VIII (FVIII), a plasma glycoprotein of 285 kDa synthesized mainly by the liver, which circulates in plasma forming complexes with the von Willebrand factor [1], Therapy for bleeding episodes in patients with HA includes plasma-derived concentrates of clotting FVIII; however, between 15% and 33% of these patients develop inhibitory antibodies against FVIII and become resistant to regular replacement therapy [2]. Recombinant activated factor VII (rFVIIa) was initially developed for patients with neutralizing antibodies (inhibitors) against coagulation FVIII and factor IX and is useful for a broad spectrum of haemorrhagic diseases due to its unique and selective mechanism of action [3],Before 1985, when the heating was not part of the regular processing of the plasma-derived concentrates, most of the haemo-philic patients who received clotting FVIII concentrates got infected by hepatitis C virus (HCV) [4]. Twenty percent of the people with HA and HCV infection progress to cirrhosis, 10% to liver failure and 2% to hepatocellular carcinoma, being these situations the most common indications for orthotopic liver transplantation (OLT) [5j.