Diagnosis of von Willebrand disease

von Willebrand disease (VWD) is the most common inherited bleeding disorder, resulting from a deficiency and/or dysfunction of von Willebrand factor (VWF). Since its first description 100 years ago, the diagnosis of VWD has evolved due to advancements in laboratory diagnostic tests and clinical guidelines and recommendations. There are 3 established diagnostic criteria for VWD: a personal history of excessive mucocutaneous bleeding, a family history of bleeding, and reduced VWF levels. Over the years, the diagnosis of VWD has been challenging, due to a lack of consensus and barriers to achieving an accurate and timely diagnosis. In 2021 evidence-based clinical practice guidelines were developed. These guidelines have provided clarity and consensus but there continues to be opposing views and challenges. Additionally, there remain barriers to achieving a diagnosis of VWD that need to be addressed.