title: Intestinal surgery with activated recombinant factor VII prophylaxis in patients with haemophilia A and high responding inhibitors: a report of five cases.
abstract: Five patients with severe haemophilia A and high responding inhibitors underwent laparoscopic or open surgery on the digestive tract (appendicectomy, cholecystectomy, partial colectomy, or haemorrhoidectomy) with recombinant activated factor VII (rFVIIa) prophylaxis. rFVIIa was administered at a dose of 92-127 mug/kg prior to surgery and then every 2 h for 18-56 h before increasing the dosing interval. One patient was switched to a continuous infusion after 48 h of rFVIIa boluses. rFVIIa treatment lasted between 5 and 14 days in four patients, with good or excellent efficacy (total dose, 3.13-9.28 mg/kg). The fifth patient, who underwent surgery for prolapsed haemorrhoids, bled on day 6 and day 10 after the procedure, despite a satisfactory prothrombin time and factor VII coagulant level. The rFVIIa dose regimen was increased after the second bleeding episode, then the bleeding rapidly ceased after this modification to the treatment regimen. The total dose of rFVIIa used was 12.65 mg/kg, and treatment lasted 17 days. Antifibrinolytic treatment was used concomitantly in all five patients. Clinical and biological tolerability was excellent, and no increase in the anti-factor VIII inhibitor titre was observed. These results suggest that rFVIIa prophylaxis is effective in haemophilia A patients with factor VIII inhibitors who are undergoing elective or emergency intestinal surgery. Further studies are required to optimize the dose regimen and treatment period according to the surgical indication and technique.
authors.affiliation: Haemophilia Centre, University Hospital of Caen, France.
authors.forename: Mounia
authors.initials: M
authors.lastname: Slaoui
authors.forename: Thierry
authors.initials: T
authors.lastname: Lambert
authors.forename: Natalie
authors.initials: N
authors.lastname: Stieltjes
authors.forename: Ségolène
authors.initials: S
authors.lastname: Claeyssens
authors.forename: Annie
authors.initials: A
authors.lastname: Borel-Derlon
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D004341:Drug Evaluation; D005167:Factor VII / Q000008:administration & dosage* / Q000032:analysis / Q000493:pharmacokinetics; D015942:Factor VIIa; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007422:Intestines / Q000601:surgery*; D008875:Middle Aged; D011292:Premedication; D011994:Recombinant Proteins / Q000008:administration & dosage*; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa