Koncentraty czynników krzepnięcia o przedłużonym działaniu w leczeniu hemofilii

The treatment of patients with haemophilia A and B is based on substitution of factor VIII and factor IX. The half-life of factor VIII (about 11 hours) and factor IX (about 18 hours) are rather short, so frequency of prophylactic infusions of factor VIII and factor IX concentrates are not less than 3 or 2 times a week. The development of new factor VIII and factor IX concentrates with longer half-lives makes possible the prolongation of time between doses and the improvement of prophylaxis efficacy. The fusion of factor IX to polyethylene glycol, Fc fragment of immunoglobulin G or albumin prolonged half-life of this coagulation protein to almost 100 hours. The half-life of factor VIII concentrate, with recombinant factor VIII combined with polyethylene glycol or Fc fragment of immunoglobulin G is prolonged to about 19 hours. Modification of factor VIII structure to produce single-chain protein with increased binding to von Willebrand factor is a new promising project as well. The introduction of coagulation concentrates with prolonged half-life will improve the quality of life of patients with severe type of haemophilia.