Evaluation of coagulation equilibrium at baseline and during factor VIII and factor IX replacement in haemophiliacs

Abstract

We designed a prospective unicentre study to evaluate the safety and efficacy of continuous infusion of different factor VIII (FVIII) and FIX concentrates in haemophilia A (n = 9) and haemophilia B (n = 4) patients undergoing surgical procedures. This study was designed to assess the potential risk of developing thromboembolic complications during different types of surgery and to provide some comparative data with respect to continuous infusion of clotting factor concentrates. Heparin prophylaxis was not used in most cases. As pointed out by others, we did not find any significant changes in prothrombin fragment F1+2 and D-dimers during a pharmacokinetic study using a bolus dose of 50 U/kg of a very high purity clotting factor concentrate. Moreover, prothrombin F1+2 and D-dimer serial assays were also carried out postoperatively, and compared with levels in control non-haemophilic patients who had undergone similar surgery with heparin prophylaxis. In haemophilia patients, despite (in most cases) an absence of heparin prophylaxis, no thrombotic complications occurred, and neither the coagulation cascade nor the fibrinolytic system were significantly over-activated, compared with the control group. From a clinical standpoint, all patients achieved excellent haemostasis without clinical evidence of thrombosis. This study emphasizes the convenient and safe administration of highly-purified FVIII and FIX concentrates in haemophiliacs undergoing surgical procedures, and constitutes a small comparative database for the evaluation of new products.