Title : Overview of 2021 guidelines on the diagnosis of Von Willebrand Disease (VWD)
Abstract:
Von Willebrand Disease (VWD) is one of the most prevalent bleeding disorders in humans, affecting up to 1% of the general population. It arises from qualitative or quantitative abnormalities of the von Willebrand factor (VWF), a crucial glycoprotein involved in hemostasis. However, achieving an accurate and timely diagnosis has been a significant challenge for individuals with VWD.
In 2021, the American Society of Hematology (ASH), International Society on Thrombosis and Hemostasis (ISTH), National Hemophilia Foundation (NHF), and World Federation of Hemophilia (WFH) jointly released new guidelines on the diagnosis of VWD. These guidelines were developed through a collaborative effort to ensure the quality and credibility of the recommendations. The diagnostic guidelines consist of 10 key questions and 11 recommendations that were carefully formulated based on an Evidence to Decision framework. They highlight the importance of using a physiologic framework and integrated testing approaches to diagnose VWD effectively. One notable aspect of the guideline development process was the emphasis on collaboration between various groups. This collaboration aimed to gather expertise and resources from different stakeholders, ensuring the wide implementation and credibility of the guidelines. Notably, the inclusion of patient input was a unique and valuable aspect of this collaboration. Patients were involved as panel members, and their perspectives were considered through stakeholder surveys and public comments. This patient-centric approach provides a more comprehensive understanding of the disease and its impact on individuals' lives.
The guidelines define the diagnostic thresholds for suspected type 1 and type 2 VWD. They offer a diagnostic algorithm that outlines the key steps in diagnosing the disease, providing healthcare professionals with a practical framework to follow. The recommendations emphasize the use of newer assays with platelet-dependent VWF activity, highlighting their advantages over the traditional Ristocetin cofactor assay. These newer assays offer improved accuracy and specificity, contributing to more reliable diagnoses.
By adhering to these guidelines, healthcare professionals can overcome the challenges associated with diagnosing VWD. The recommendations provide a standardized approach, ensuring that patients receive accurate diagnoses in a timely manner. This is crucial for effective management and improving the quality of life for individuals with VWD.
Audience Take Away Notes:
- Quick introduction and background of Von Willebrand Disease (VWD).
- Familiarize oneself with the new 2021 diagnostic guidelines for VWD.
- Learn the development and objectives of the 2021 guidelines on the diagnosis of VWD.
- Overview of the algorithm from the diagnostic guidelines, focusing on diagnostic thresholds and utilization of newer assays with platelet-dependent VWF activity.
- How testing helps to diagnose VWD using a physiologic framework.
