von Willebrand Disease
Diagnosis of von Willebrand disease
Timely diagnosis of von Willebrand Disease enables adequate treatment and is cost-effective.1 Gynecologists can make a decisive contribution by recognizing von Willebrand Disease in patients at an early stage and referring them to hematology if necessary. Diagnosis of von Willebrand Disease is based on a medical history, followed by a multistep laboratory blood analysis.2
Anamnesis for the assessment of the bleeding phenotype
The assessment of the bleeding phenotype starts with a detailed history of all bleeding symptoms of the patient.2 The medical history questionnaire provided by CSL Behring is used to assess bleeding symptoms in adults. It is based on suggestions of a working group initiated by the International Society on Thrombosis and Haemostasis (ISTH) and the Scientific and Standardization Committee (SCC).3
Download medical history questionnaire (in German)
Download medical history questionnaire (in French)
Laboratory diagnosis and classification
Because of the complexity of von Willebrand disease, diagnosis and classification are challenging.
When von Willebrand disease is suspected, the first level of testing includes measurement of VWF antigen level (VWF:Ag), platelet-binding activity of vWF (e.g., [VWF:RCo] assay), and factor VIII activity (FVIII:C). If these first-stage tests show clear abnormalities, a diagnosis of von Willebrand disease can be made.
Precise subdivision of the type and subtype of the disease can be made using further tests, such as vWF multimer analysis, platelet aggregometry, or genotyping.2
References:
1. Sidonio et al. Cost-Utility Analysis of von Willebrand Disease Screening in Adolescents with Menorrhagia. J Peds. 2010;157:456-460. 2. Leebeek WG, Eikenboom CJ. Von Willebrand’s Disease. N Engl J Med. 2016;375:2067-80. 3. Rodeghiero F, et al. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders.ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. J Thromb Haemost. 2010 Sep;8(9):2063-5.