Von Willebrand disease diagnosis: from complexity to simplicity

Von Willebrand disease (VWD) is a complex disorder in terms of both its pathophysiology and treatment. The broad recognition and treatment of patients with VWD is often hindered by the complicated diagnostic process. Therefore, a more streamlined approach is needed, particularly in settings with limited expertise or resources. One practical initial strategy is to prioritize measuring von Willebrand factor (VWF) activity using a robust assay, alongside VWF antigen and factor (F)VIII levels, and administering a test dose of desmopressin. Once a diagnosis has been established, 3 variables should be considered for patients requiring replacement therapy: the patient's endogenous VWF activity and FVIII levels, and the quality of the infused multimers, as reflected by the VWF activity/antigen ratio of the concentrate. This simplified framework can facilitate the global diagnosis and management of VWD patients. However, we acknowledge the limitations, particularly with regard to accurately subtyping patients with type 2 VWD, since precise classification is essential for optimal care.