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Intestinal surgery with activated recombinant factor VII prophylaxis in patients with haemophilia A and high responding inhibitors: a report of five cases

Slaoui, Mouniaa; Lambert, Thierryb; Stieltjes, Nataliec; Claeyssens, Ségolèned; Borel-Derlon, Annieaand the Study Group

Blood Coagulation & Fibrinolysis: December 2004 - Volume 15 - Issue 8 - p 687-691
CASE REPORT
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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 μg/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.

aHaemophilia Centre, University Hospital of Caen, bHaemophilia Centre, University Hospital of Kremlin-Bicêtre, cHaemophilia Centre, University Hospital of Paris-Cochin and dHaemophilia Centre, University Hospital of Toulouse, France.

Correspondence and requests for reprints to Annie Borel-Derlon, Centre de Traitement pour Hémophiles, Centre Hospitalo-Universitaire, Avenue de la Côte de Nacre, 14033 Caen, France. Tel: + 33 2 3106 4565; fax: + 33 2 3106 4566; e-mail: borelderlon-a@chu-caen.fr

Received 5 March 2004 Revised 14 June 2004 Accepted 18 June 2004

© 2004 Lippincott Williams & Wilkins, Inc.