Von Willebrand Disease: Gaining a global perspective

Area of Science:

• Hematology
• Global Health
• Epidemiology

Background:

• Recent guidelines highlight challenges in diagnosing and managing von Willebrand Disease (VWD).
• Accurate international counts of people with VWD (PwVWD) are needed to target support and improve diagnosis.
• Understanding global VWD registration is crucial for addressing unmet clinical and research needs.

Purpose of the Study:

• To analyze international VWD registration rates.
• To assess the influence of income, geography, age, and sex on VWD registration.
• To inform World Federation of Haemophilia (WFH) strategies for VWD care.

Main Methods:

• Analysis of data from the 2018/2019 WFH Annual Global Survey (AGS).
• Global perspective on VWD registration rates and demographic profiles.

Main Results:

• Registration rates for PwVWD are lowest in South Asia and highest in Europe/Central Asia, but generally below expected prevalence.
• National income status significantly impacts VWD registration, correlating with healthcare infrastructure.
• Females are the majority of PwVWD globally, but males predominate in low-income countries, potentially due to bleeding stigma.
• Paediatric registrations are higher in North America, MENA, and South Asia.
• Type 3 VWD diagnoses are disproportionately high in low-income countries, indicating diagnosis of severe cases only.

Conclusions:

• Significant international disparities in PwVWD registration rates exist, influenced by national income and Hemophilia Treatment Centre (HTC) networks.
• Understanding these registration patterns is key to targeting advocacy efforts for improved VWD awareness, diagnosis, and support worldwide.