Pediatric Von Willebrand Disease Guidelines

Updated: Jun 23, 2021
  • Author: Suchitra S Acharya, MD, MBBS; Chief Editor: Hassan M Yaish, MD  more...
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Guidelines

Guidelines Summary

ASH/ISTH/NHF/WFH guidelines

Guidelines from 2021 on the diagnosis of von Willebrand disease, as developed by a multidisciplinary panel from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH), include the following [20] :

  • When the probability of von Willebrand disease is low, an initial screen with a validated bleeding-assessment tool (BAT) is recommended to assess which patients require specific blood testing (ie, von Willebrand factor antigen, platelet-dependent von Willebrand factor activity, factor VIII coagulant activity) over nonstandardized clinical assessment
  • When there is an intermediate probability of von Willebrand disease, it is suggested that a BAT not be relied upon to decide whether specific blood testing should be ordered
  • When there is a high probability of von Willebrand disease, it is recommended that a BAT not be relied upon to decide whether specific blood testing should be ordered

Guidelines from 2021 on the management of von Willebrand disease from the aforementioned ASH/ISTH/NHF/WFH panel include the following [21] :

  • When von Willebrand disease is associated with a history of severe and frequent bleeds, the use of long-term prophylaxis, rather than no prophylaxis, is suggested
  • When patients with von Willebrand and cardiovascular disease need antiplatelet agents or anticoagulant therapy, the provision of the necessary antiplatelet or anticoagulant treatment is suggested over no therapy
  • It is suggested that in women with type 1 von Willebrand disease or low von Willebrand factor, tranexamic acid be used during the postpartum period, as opposed to not using it (a suggestion that may apply to type 2 and type 3 von Willebrand disease as well)