The diagnosis, natural history, and management of von Willebrand disease in women in the age of guidelines

Expert Rev Hematol. 2023 Jun;16(6):435-450. doi: 10.1080/17474086.2023.2166925. Epub 2023 Jan 12.

Abstract

Introduction: Women and girls with bleeding disorders face multiple bleeding challenges throughout their life. The most significant morbidity and mortality are due to heavy menstrual bleeding and postpartum hemorrhage in their reproductive years. The ASH/ISTH/NHF/WFH 2021 guidelines on diagnosing and managing von Willebrand disease (VWD) provide several new updates.

Areas covered: Women with VWD have a higher prevalence of heavy menstrual bleeding. The subpopulation of adolescents is particularly vulnerable, as the diagnosis is often delayed with increased comorbidity of iron deficiency anemia and associated symptoms. A detailed review is done on the prevalence of bleeding-related complications, especially heavy menstrual bleeding (HMB) and post-partum hemorrhage (PPH). The management strategies are also reviewed in detail, with a specific focus on the target factor levels and the use of antifibrinolytics.

Expert opinion: The 2021 ASH/ISTH/NHF/WFH diagnostic and management recommendations are reviewed with a specific focus on hormonal methods of HMB management and antifibrinolytics in this situation. The reviewed topics include neuraxial anesthesia, factor cutoff, and tranexamic acid use in the postpartum period.

Keywords: Von Willebrand disease; guidelines; heavy menstrual bleed; neuraxial anesthesia; postpartum hemorrhage; tranexamic acid.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Antifibrinolytic Agents* / therapeutic use
  • Female
  • Humans
  • Menorrhagia* / diagnosis
  • Menorrhagia* / etiology
  • Menorrhagia* / therapy
  • Postpartum Hemorrhage*
  • Pregnancy
  • von Willebrand Diseases* / diagnosis
  • von Willebrand Diseases* / epidemiology
  • von Willebrand Diseases* / therapy

Substances

  • Antifibrinolytic Agents