title: Development and validation of technical assays of von Willebrand disease in routine laboratory.
abstract: Von Willebrand disease (MW) is the most common constitutional bleeding disorders. It is caused by a quantitative or qualitative abnormality of the von Willebrand factor (VWF). The laboratory assessment of the disease combines a FVIII assay, and a determination of the antigen and activity of VWF. The analytical validation of VWF:Ag, VWF:Act, vWF:CB is reported in this work and demonstrates good test performance of all three assays, with a coefficient of variation lower than 10% for both the repeatability and reproducibility, stability with a deviation of less than 5% from the target value after six hours at room temperature. The dosages are linear through the following ranges: 2% to 346% for VWF:Ag, 3% to 170% for VWF:Act, and 0.07% to 259% for VWF:CB. The usual values determined on 32 control subjects are in the range of reference values published in the literature. However as the number of control samples tested is small, we will adopt the reference values of the literature of 50 to 200% in routine. The other functional test VWF:CB will be used in our daily practice to differentiate the type of 2M 2A type. However, given that the type 2M is extremely rare, we think it is rather aimed at specialized laboratories in which a large number of patients referred.
authors.affiliation: Laboratoire d'hémostase, IRIS-Lab, site CHU Brugmann, Université Libre de Bruxelles, Belgique.
authors.forename: Sara
authors.initials: S
authors.lastname: Derdabi
authors.affiliation: Laboratoire d'hémostase, IRIS-Lab, site CHU Brugmann, Université Libre de Bruxelles, Belgique.
authors.forename: Laurence
authors.initials: L
authors.lastname: Rozen
authors.affiliation: Laboratoire d'hémostase, IRIS-Lab, site CHU Brugmann, Université Libre de Bruxelles, Belgique.
authors.forename: Carole
authors.initials: C
authors.lastname: Nagant
authors.affiliation: Laboratoire d'hémostase, IRIS-Lab, site CHU Brugmann, Université Libre de Bruxelles, Belgique.
authors.forename: Anne
authors.initials: A
authors.lastname: Demulder
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D003955:Diagnostic Tests, Routine / Q000379:methods / Q000592:standards; D005260:Female; D006403:Hematologic Tests / Q000379:methods / Q000592:standards; D006801:Humans; D007753:Laboratories / Q000592:standards; D008297:Male; D008875:Middle Aged; D012016:Reference Values; D015203:Reproducibility of Results; D055815:Young Adult; D014842:von Willebrand Diseases / Q000175:diagnosis*; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D014841:von Willebrand Factor
keywords: VWF:Act; VWF:Ag; VWF:CB; Von Willebrand disease