Use of recombinant factor VIIa (NovoSeven) in the treatment of two patients with type III von Willebrand's disease and an inhibitor against von Willebrand factor.
Two siblings affected by type III von Willebrand's disease with precipitating alloantibodies against von Willebrand's factor (vWF) and not susceptible to treatment with factor VIII/vWF concentrates received recombinant activated factor VII for oral surgery. This therapy, combined with antifibrinolytic drugs and local application of fibrin glue, seems to be effective and safe. It may be considered a promising approach to the management of this rare condition.
Ciavarella
N
N
Servizio di Coagulazione, Centro Emofilia, Policlinico-Universita, Bari, Italy.
Schiavoni
M
M
Valenzano
E
E
Mangini
F
F
Inchingolo
F
F
D000293:Adolescent; D000328:Adult; D015942:Factor VIIa / Q000627:therapeutic use*; D006801:Humans; D007518:Isoantibodies / Q000097:blood*; D008297:Male; D019106:Postoperative Hemorrhage / Q000517:prevention & control*; D011994:Recombinant Proteins / Q000627:therapeutic use; D013515:Surgery, Oral; D014842:von Willebrand Diseases / Q000188:drug therapy* / Q000276:immunology
D007518:Isoantibodies; D011994:Recombinant Proteins; D015942:Factor VIIa