Safety and Efficacy of Continuous Infusion of a Combined Factor VIII – von Willebrand Factor (vWF) Concentrate (Haemate-P™) in Patients with von Willebrand Disease

Aharon Lubetsky; Sam Schulman; David Varon; Uri Martinowitz; Gili Kenet; Sanford Gitel; Aida Inbal

doi:10.1055/s-0037-1614448

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1999; 81(02): 229-233
DOI: 10.1055/s-0037-1614448

Review Articles

Schattauer GmbH

Safety and Efficacy of Continuous Infusion of a Combined Factor VIII – von Willebrand Factor (vWF) Concentrate (Haemate-P™) in Patients with von Willebrand Disease

Aharon Lubetsky

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

,

Sam Schulman

²Haemophilia Center, Department of Medicine, Karolinska Hospital, Stockholm, Sweden

,

David Varon

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

,

Uri Martinowitz

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

,

Gili Kenet

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

,

Sanford Gitel

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

,

Aida Inbal

¹From the Institute of Thrombosis and Hemostasis and National Haemophilia Center, Sheba Medical Center, Tel-Hashomer, and Sackler School of Medicine, Tel-Aviv University, Israel

› Author Affiliations

Abstract

Summary

We studied the safety and efficacy of treatment with continuous infusion of a von Willebrand factor (vWF) concentrate Haemate-P in patients with von Willebrand disease (vWD). Three patients with mild and 5 patients with severe forms of vWD, were treated with continuous infusion of Haemate-P by minipump. The indications for treatment were: to prevent bleeding during 9 surgical procedures or 1 vaginal delivery in 6 patients and to treat 2 bleeding episodes in 2 patients. The patients were monitored daily for factor VIII (FVIII:C) and ristocetin cofactor (vWF:RCo) levels and the infusion rate was adjusted to maintain the desired therapeutic level of vWF:RCo. The treatment was effective in preventing surgical bleeding and controlling bleeding episodes. All factor VIII:C and most of the vWF:RCo levels measured during the study period were above the target therapeutic levels. A significant decrease in clearance of FVIII:C and vWF:RCo was observed over the treatment period. Haemate-P consumption averaged 24.3 ± 7.9 vWF:RCo U/kg/day which is approximately half the expected dose had intermittent bolus injections been used. We suggest that continuous Haemate-P infusion is superior to intermittent bolus injections for the treatment of vWD patients by virtue of its efficiency, simplicity and considerable savings.

Full Text

References

References
1 Montgomery RR, Coller BS. Von Willebrand Disease. In: Hemostasis and Thrombosis. Basic Principles and Clinical Practice. Colman RW, Hirsh J, Marder VJ, Salzman EW. eds Philadelphia: J.B. Lippincott; 1994: 134-68.
2 Scott JP, Montgomery RR. Therapy of von Willebrand disease. Semin Thromb Haemost 1993; 19: 37-47.
3 Fukui H, Nishino M, Terada S, Nishikubo T, Yoshioka A, Kinoshita S, Niinomi K, Yoshioka K. Hemostatic effect of heat-treated factor VIII concentrate (Haemate-P) in von Willebrand's disease. Blut 1988; 56: 171-8.
4 Berntorp E, Nilsson IM. Use of High-Purity Factor VIII Concentrate (Haemate-P) in von Willebrand's Disease. Vox sanguinis 1989; 56: 212-7.
5 Martinowitz U, Schulman S. Coagulation factor concentrates by continuous infusion. Transfusion Medicine Reviews 1997; 11: 56-63.
6 Martinowitz U, Schulman S. Continuous infusion of coagulation products. International Journal of Pediatric Hematology/Oncology 1994; 1: 471-8.
7 Naqvi A, EndreS-Brooks J, Montgomery RR, Stein AM, Sparling C, Klaassen R, Blanchette VS. Successful cardiac surgery in a young girl with type 2A von Willebrand disease using continuous infusion of Hemate-P. Thromb Haemost 1997; 77 Suppl 514. (Abstr).
8 Montoro JB, Altisent C, Pineda A, Rodriguez S, Tusell JM. Continuous infusion of factor VIII concentrate to a patient with severe von Willebrand disease. Transfusion 1993; 33: 443 (Abstr).
9 Auerswald G, Kreuz W, Auberger K, Deinhardt U, Roth U. Continuous infusion of factor VIII after elective surgery in children with Haemophilia A and von Willebrand disease. Thromb Haemost 1995; 73: 1024 (Abstr).
10 Smith MP, Rice KM, Bromige ES, Lawn M, Beresford-Webb R, Spence K, Kahir K, Hann I, Savidge GF. Continuous infusion therapy with very high purity von Willebrand factor concentrate in patients with severe von Willebrand disease. Blood Coagulation and Fibrinolysis 1997; 8: 6-12.
11 Bowie EJW, Owen CA. Standardization of the bleeding time. Scand J Haematol 1980; 37 Suppl 87-94.
12 Zimmerman TS, Hoyer LW, Dickson L, Edgington TS. Determination of the von Willebrand's disease antigen (factor VIII-related antigen) in plasma by quantitative immunoelectrophoresis. J Lab Clin Med 1975; 86: 152-8.
13 Evans RJ, Austen DEG. Assay of ristocetin cofactor activity using fixed platelets and a platelet counting technique. Br J Hematol 1977; 37: 289-94.
14 Hardisty RM, MacPherson CA. A one stage factor VIII assay and its use on venous and capillary plasma. Thromb Diath Haemorrh 1962; 7: 215-28.
15 Matucci A, Messori A, Donati-Cori G, Longo G, Vannini S, Morfini M, Tendi E, Rossi-ferrini PL. Kinetic evaluation of four Factor VIII concentrates by model-independent methods. Scand J Haematol 1985; 34: 22-8.
16 Longo G, Matucci A, Messori A, Morfini M, Rossi-Ferrini PL. Pharmacokinetics of a new heat-treated concentrate of factor VIII estimated by model-independent methods. Thromb Res 1986; 42: 471-6.
17 Varon D, Schulman S, Bashari D, Martinowitz U. Home therapy with continuous infusion of of factor VIII after minor surgery or serious haemorrhage. Hemophilia 1996; 2: 207-10.
18 Scharrer I, Vigh T, Aygorsen-Pursun E. Experience with Haemate P in von Willebrand's disease in adults. Haemostasis 1994; 24: 298-303.
19 Kreuz W, Mentzer D, Becker S, Scharrer I, Kornhuber B. Haemate P^® in children with von Willebrand's disease. Haemostasis 1994; 24: 304-10.
20 Pai M, Robinson S, Teitel J, Bergman GE. Haemate P^® dosing trends among 97 canadian von Willebrand disease (vWD) patients. Thromb Haemost 1997; 77 Suppl. 513 (Abstr).