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

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
• heavy menstrual bleed
• postpartum hemorrhage
• tranexamic acid
• neuraxial anesthesia
• guidelines

Article highlights

• The 2021 ASH/ISTH/NHF/WFH guidelines on von Willebrand disease have updated the latest recommendations on diagnosis and management.

• The article focuses mainly on the burden of bleeding complications in women with VWD, the recommendations on their management in this era of guidelines, and the evidence behind it.

• Heavy menstrual bleed in the adolescent population is especially highlighted with management strategies, and hormonal methods are emphasized.

• Post-partum management with antifibrinolytics is emphasized, and the review on neuraxial anesthesia is highlighted.

Declaration of interests

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.