title: 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.
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).
authors.affiliation: Hemostasis Thrombosis Research, Department of Hematology, University Hospital Antwerp, Antwerp, Belgium. postbus@goodheartcenter.demon.nl
authors.forename: Jan J
authors.initials: JJ
authors.lastname: Michiels
authors.forename: Alain
authors.initials: A
authors.lastname: Gadisseur
authors.forename: Marc
authors.initials: M
authors.lastname: van der Planken
authors.forename: Wilfried
authors.initials: W
authors.lastname: Schroyens
authors.forename: Ann
authors.initials: A
authors.lastname: van de Velden
authors.forename: Zwi
authors.initials: Z
authors.lastname: Berneman
mesh_terms: D003029:Coagulants / Q000627:therapeutic use*; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage* / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use*; D017408:Guidelines as Topic; D006470:Hemorrhage / Q000188:drug therapy* / Q000517:prevention & control*; D006801:Humans; D007262:Infusions, Intravenous; D014842:von Willebrand Diseases / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000627:therapeutic use*
chemical_list: D003029:Coagulants; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin