Thromb Haemost 2005; 94(06): 1177-1180
DOI: 10.1160/TH05-05-0342
Blood Coagulation, Fibrinolysis and Cellular Haemostasis
Schattauer GmbH

Continuous infusion of recombinant factor VIIa for surgery in patients with deficiency of factor VII

Sam Schulman
1   Coagulation Unit, Department of Haematology and
,
Geir E. Tjønnfjord
3   Section of Haematology, Department of Internal Medicine, Rikshospitalet University Hospital, Oslo, Norway
,
Richard Wallensten
2   Department of Orthopaedic Surgery, Karolinska University Hospital, Stockholm, Sweden
,
Uri Martinowitz
4   The National Hemophilia Center, The Chaim Sheba Medical Center, Tel Hashomer, Israel
,
Gili Kenet
4   The National Hemophilia Center, The Chaim Sheba Medical Center, Tel Hashomer, Israel
› Author Affiliations
Further Information

Publication History

Received 17 May 2005

Accepted after resubmission 22 August 2005

Publication Date:
07 December 2017 (online)

Summary

The administration of recombinant activated factor VII (rFVIIa) by continuous infusion has provided a safe and convenient alternative to bolus injections in haemophiliacs with inhibitors, but it has only been reported in a single case with congenital factorVII (FVII) deficiency. The results of 12 consecutive surgical procedures in 7 patients with congenital FVII deficiency are reported here. rFVIIa was always given in continuous infusion, aiming at plasma FVII activity of 0.5 IU/mL. Treatment was given for 2 to 7 days with a mean total dose of 7.8 mg rFVIIa. Blood loss was as expected from the different types of procedures and the only thromboembolic complication was a superficial thrombophlebitis at the infusion site. This mode of substitution was therefore safe, effective and well tolerated.

 
  • References

  • 1 Hedner U, Glazer S, Pingel K. et al Successful use of recombinant factor VIIa in a patient with severe haemophilia A during synovectomy. Lancet 1988; 2: 1193.
  • 2 Bauer K. Treatment of factor VII deficiency with recombinant factor VIIa. Haemostasis 1996; 26 (Suppl. 01) 155-8.
  • 3 Ingerslev J, Knudsen L, Hvid I. et al Use of recombinant factor VIIa in factor-VII-deficient patients. Haemophilia 1997; 3: 215-8.
  • 4 Jiménez-Yuste V, Villar A, Morado M. et al Continuous infusion of recombinant activated factor VII during caesarian section delivery in a patient withcongenital factor VII deficiency. Haemophilia 2000; 6: 588-90.
  • 5 Hedner U. Dosing with recombinant factor VIIa based on current evidence. Semin Hematol 2004; 41 (Suppl. 01) 35-9.
  • 6 Wong WY, Huang WC, Miller R. et al Clinical efficacy and recovery levels of recombinant FVIIa (Novo-Seven) in the treatment of intracranial haemorrhage in severe neonatal FVII deficiency. Haemophilia 2000; 6: 50-4.
  • 7 White B, O'Connor H, Smith OP. Successful use of recombinant VIIa (Novoseven) and endometrial ablation in a patient with intractable menorrhagia secondary to FVII deficiency. Blood Coagul Fibrinolysis 2000; 11: 155-7.
  • 8 Scharrer I. Recombinant factor VIIa for patients with inhibitors to factor VIII or IX or factor VII deficiency. Haemophilia 1999; 5: 253-9.
  • 9 Schulman S, Bech Jensen M, Varon D. et al Feasibility of using recombinant factor VIIa in continuous infusion. Thromb Haemost 1996; 75: 432-6.
  • 10 Schulman S. the rFVIIa-CI group. Safety, efficacy and lessons from continuous infusion with rFVIIa. Haemophilia 1998; 4: 564-7.
  • 11 Schulman S, Johnsson H, Lindmarker P. Thrombotic complications after substitution with a FVII concentrate. Thromb Haemost 1991; 66: 619 (Letter).
  • 12 Mariani G, Herrmann FH, Schulman S. et al International Factor VII Deficiency Study Group. Thrombosis in inherited factor VII deficiency. J Thromb Haemost 2003; 1: 2153-8.
  • 13 Mazoyer E, Kevorkian J-P, Biyick E. et al Efficacy and safety of continuous infusion of factor VII concentrate in a factor VII-deficient patient at high risk for arterial thrombosis. Blood Coagul Fibrinolysis 2004; 15: 519-20.
  • 14 Eikenboom JC, Bos CF, Briët E. Peri-operative replacement therapy with factor VII concentrate in a patient with severe factor VII deficiency. Thromb Haemost 1992; 67: 285-6.
  • 15 Schulman S. Continuous infusion. Haemophilia 2003; 9: 368-75.
  • 16 Brummel Ziedins K, Rivard GE, Pouliot RL. et al Factor VIIa replacement therapy in factor VII deficiency. J Thromb Haemost 2004; 2: 1735-44.
  • 17 Gershwin ME, Gude JK. Deep vein thrombosis and pulmonary embolism in congenital factor VII deficiency. N Engl J Med 1973; 288: 141-2.
  • 18 Nakagawa K, Furusawa A, Momono S. et al Gastrectomy in a patient with congenital factor VII deficiency – a case report. Nippon Ganka Gakkai Zasshi 1990; 91: 771-3.
  • 19 Aledort LM. Comparative thrombotic event incidence after infusion of recombinant factor VIIa versus factor VIII inhibitor bypass activity. J Thromb Haemost 2004; 2: 1700-8.
  • 20 Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004; 2: 899-909.