Successful Treatment of Patients with von Willebrand Disease using a High-Purity Double-Virus Inactivated Factor VIII/von Willebrand Factor Concentrate (Immunate^®)

ABSTRACT

Fourteen patients with different types of von Willebrand disease (vWD) having acute bleeds or elective surgery were treated with Immunate^℗, a double-virus inactivated factor VIII/von Willebrand factor (FVIII/vWF) concentrate. The concentrate was applied as a bolus or via continuous infusion. FVIII activity (FVIIIc), vWF antigen (vWF:Ag), ristocetin cofactor activity (vWF:RCo), collagen binding activity (vWF:CB), activated partial thromboplastin time (aPTT), and von Willebrand multimers (vW-multimers) were monitored for 48 hours. Pharmacokinetic analyses were performed. The clinical efficacy was rated excellent or good. Bleeding complications occurred in 3 patients due to an additional FXIII deficiency in one patient, to a surgically induced bleed in another patient, and a rather short substitution period in the third patient. There were no serious adverse experiences. One patient showed a phlebitic reaction at the site of venous access after more than 100 hours of continuous infusion, requiring a change to application via bolus.

Von Willebrand disease - surgery - acute bleeds - Immunate - clinical efficacy

REFERENCES

• 1 Auerswald G, Anders O, Budde U, et al. Clinical study of the biological efficiency of a high-purity FVIII:C-vWF complex concentrate in non-bleeding patients with von Willebrand disease. In: Scharrer I, Schramm W, eds. 28 Hämophilie-Symposion, Hamburg 1997 Berlin: Springer-Verlag 1999: 240-247
• 2 Hofmann K, Strassburger J-U. A 4.5 years old patient with von Willebrand disease type 2A, subtype IIE undergoing two-sided herniotomy according to Rehbein under continuous infusion of Immunate STIM plus. XVIIth Congr Int Soc Thromb Haemost, Washington, D.C., August 14-21, 1999 (Abst)
• 3 Mader R, Streif W, Kyrle P. Therapie eines Kindes mit von Willebrand-Jürgens-Syndrom Typ II mit hochgereinigtem FVIII-Konzentrat.  Paediatr Paedol . 1995;  30 88
• 4 Beeck H, Werling C, Hellstern P, Saggau W, Heene D. Kombinierte Behandlung mit Immunate STIM plus und Minirin bei einem Patienten mit mittelschwerem von Willebrand-Syndrom im Rahmen eines ausgedehnten kardiochirurgischen Eingriffs. In: Scharrer I, Schramm W, eds. 27 Hämophilie-Symposion, Hamburg 1996, Berlin: Springer-Verlag 1998 : 416-420
• 5 Sas S, Ceglédi A, Nemes L. Treatment of von Willebrand patients with Immunate. XXIIIrd Int Congress World Fed Hemoph, The Hague, May 17-22 1998 Abst
• 6 Thomas K, Urbancik W, Turecek P. Continuous infusion of FVIII and FIX concentrates: in vitro analysis of clinically relevant parameters.  Haemophilia . 1999;  5 17-25
• 7 Macfarlane D, Stibbe J, Kirby E. A method for assaying von Willebrand factor (ristocetin cofactor).  Thrombosis et Diathesis Haemorrhagica . 1975;  4 306-308
• 8 Evans R, Austen D. Assay of ristocetin co-factor using fixed platelets and a platelet counting technique.  Br J Haematol . 1977;  37 289-294
• 9 Siekmann J, Turecek P, Schwarz H P. The determination of von Willebrand factor activity by collagen binding assay.  Haemophilia . 1998;  4(Suppl 3) 15-24
• 10 Ruggderi Z, Zimmermann T. The complex multimeric composition of factor VIII/von Willebrand factor.  Blood . 1981;  57 1140-1143
• 11 Aihara M, Sawada Y, Ueno K. Visualization of von Willebrand factor multimers by immunoenzymatic stain using avidin-biotin peroxidase complex.  Thromb Haemost . 1986;  55 263-267
• 12 Gibaldi M, Perrier D. Pharmacokinetics, 2nd ed.  New York: Marcel Dekker 1982
• 13 Lee M, Poon W, Kingdon H. A two-phase linear regression model for biologic half-life data.  J Lab Clin Med . 1990;  115 745-748