Venous and Arterial Thrombo-Embolic Events in Patients With von Willebrand Disease From Western France: The TWIGO Study.

INTRODUCTION

Patients with von Willebrand disease (VWD) are prone to bleeding, yet they may also experience thrombotic events, whose nature, frequency, and optimal management remain poorly characterised.

AIM: To describe the nature and frequency of venous and arterial thrombotic events in VWD patients.

METHODS

This multicentre retrospective study analyzed thrombotic events in 1345 patients with constitutional VWD and von Willebrand factor (VWF) activity ≤30 IU/dL from the French BERHLINGO database. Venous events included deep vein thrombosis (DVT) and pulmonary embolism (PE); arterial events included angina, myocardial infarction (MI), ischaemic stroke (ICVA), transient ischaemic attack (TIA), and peripheral artery disease (PAD). Risk factors, therapeutic strategies, efficacy, and bleeding complications were also assessed.

RESULTS

We identified 35 thrombotic events: 30 arterial (12 angina, 6 MI, 7 PAD, 4 ICVA, 1 TIA) in 20 patients, and 5 venous (3 PE±DVT, 2 DVT) in 4 patients (1.8% prevalence). The mean patient age was 61.8±14 years. Most arterial events (70%) occurred in men; all venous events were provoked in women. Therapeutic adjustments were made for 10 arterial events (28.6%: 4 withholding, 6 low-dose). Of the 35 events, 11 (31.4%) were recurrences (second or subsequent) in the same patient. Overall, 8/24 patients (33.3%) had multiple events, always at the same site, including twice (18.2%) after therapeutic adjustments. Only trauma-induced bleeding was reported.

CONCLUSION

Although rare, thrombosis in VWD patients is associated with age and gender. Given the low bleeding risk, therapeutic approaches similar to those in the general population may be considered.

TRIAL REGISTRATION: Cardiovascular and Venous Thromboembolism Disease in Patients with Von Willebrand Disease in the French West (TWIGO); ClinicalTrials.gov ID NCT05773638.