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Semin Thromb Hemost 2006; 32(6): 636-645
DOI: 10.1055/s-2006-949669
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Guidelines for the Evaluation of Intravenous Desmopressin and von Willebrand Factor/Factor VIII Concentrate in the Treatment and Prophylaxis of Bleedings in von Willebrand Disease Types 1, 2, and 3

Jan J. Michiels1 , 2 , Alain Gadisseur1 , Marc van der Planken1 , Wilfried Schroyens1 , Ann van de Velden1 , Zwi Berneman1
  • 1Hemostasis Thrombosis Research, Department of Hematology, University Hospital Antwerp, Antwerp Belgium
  • 2Goodheart Institute, Hemostasis Thrombosis Science Center, Rotterdam, The Netherlands
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Publication History

Publication Date:
15 September 2006 (online)

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ABSTRACT

The current standard for the diagnosis and management of patients with congenital von Willebrand disease (vWD) includes bleeding times (BTs), PFA-100 closure time (PFA-CT), factor (F) VIII:coagulant activity (C), vWF:antigen (Ag), vWF:ristocetin cofactor activity (RCo), a sensitive vWF:collagen-binding activity (CB), ristocetin-induced platelet aggregation (RIPA), analysis of vWF multimers in low- and high-resolution agarose gels, and the response to desmopressin. Guidelines and recommendations for prophylaxis and treatment of bleedings in vWD patients with vWF/FVIII concentrates should be derived from analysis of the content of these concentrates and from pharmacokinetic studies in different types of vWD patients with severe type 1, 2, or 3 vWD. The vWF/FVIII concentrates should be characterized by labeling with FVIII:C, vWF:RCo, vWF:CB, and vWF multimeric pattern, which will determine their predicted efficacy and safety in prospective management studies. Because the bleeding tendency is moderate in type 2 and severe in type 3 vWD, and because the FVIII:C levels are subnormal in type 2 and very low in type 3 vWD patients, new guidelines using vWF:RCo unit dosing for the prophylaxis and treatment of bleeding episodes are proposed. Such guidelines should be stratified for the severity of bleeding, the type of surgery (either minor or major), and also for the severity and type of vWD (i.e., either type 2 or 3 vWD).

KEYWORDS

Von Willebrand factor - von Willebrand disease - ristocetin cofactor activity - von Willebrand collagen-binding activity - bleeding time - desmopressin (DDAVP) - von Willebrand factor concentrates

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Jan Jacques MichielsM.D. Ph.D. 

Goodheart Institute, Hemostasis Thrombosis Science Center, Erasmus Tower

Veenmos 13, 3069 AT Rotterdam, The Netherlands

Email: postbus@goodheartcenter.demon.nl