Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency

Year 2011, Volume: 3 Issue: 2, 1 - 4, 07.04.2011

Baris Malbora , Zekai Avcı Betul Orhan Serdar Arda Namik Ozbek

Abstract

Factor XI (FXI) deficiency is a rare inherited bleeding disorder caused by a mutation in the FXI gene, which is located on the distal arm of chromosome 4 (4q35). Factor XI (FXI) deficiency is defined when the activity of FXI in plasma is less than 15 U/dL. We report our experience about perioperative use of recombinant activated factor VII (rFVIIa) in a 17 year-old with congenital FXI deficiency and appendicitis. After appendectomy, the first intravenous dose of rFVIIa was administered. This same dosage was repeated and he was discharged on postoperative 72nd hour without complications.

Keywords

appendectomy , factor XI deficiency , recombinant factor VIIa

References

• Salomon O, Seligsohn U. New observations on factor XI deficiency. Haemophilia 2004;10:184-7.
• Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost 2009;7 (Suppl. 1): 84–7.
• Bolton-Maggs PH, Patterson DA, Wensley RT, Tuddenham EG. Definition of the bleeding tendency in factor XI deficient kindreds-a clinical and laboratory study. Thromb Haemos 1995;73:194-202.
• Niamh M. O’Connell. Factor XI deficiency-from molecular genetics to clinical management. Blood Coagul Fibrinolysis 2003; 14 (suppl 1): 59-64.
• Ramadan KM, McNulty O, Anderson JA, Jones FG, Winter PC. A novel factor XI deficiency in a symptomatic patient. Blood Coagul Fibrinolysis 2006; 17: 499-502.
• Sidi A, Seligsoh U, Jonas P, Many M. Factor XI deficiency, detection and management during urological surgery Urol 1978; 119: 528-30.
• Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia 2000; 6: 100-9.
• Avci Z, Malbora B, Gokdemir M, Ozkan S, Ozbek N. Successful Use of Recombinant Factor VIIa (NovoSeven) During Cardiac Surgery in a Pediatric Patient with Congenital Factor XI Deficiency. Pediatr Cardiol 2008; 29: 220–2. ®
• Berliner S, Horowitz I, Martinowitz U, Brenner B, Seligsohn U. Dental surgery in patients with severe factor XI deficiency without plasma replacement therapy. Blood Coagul Fibrinol 1992; 3: 465-8.
• David AL, Paterson-Brown S, Letsky A. Factor XI deficiency presenting in pregnancy: diagnosis and management. Br J Obstet Gynaecol 2002; 109: 840–3.
• Schulman S, Nemeth G. An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency. Haemophilia 2006; 12: 223-7.