Background: Important diagnostic and clinical aspects of moderately reduced von Willebrand factor (VWF) levels are still unknown. There is no clear evidence which cutoff value (0.50 vs 0.60 IU/ml) should be used to diagnose “low VWF.” Also, the incidence of bleeding after the diagnosis has been made, and risk factors for bleeding are unknown yet. Objectives: To investigate the incidence of postsurgical bleeding, postpartum hemorrhage (PPH), and traumatic and spontaneous bleeding after low VWF diagnosis, and to develop a risk score to predict future bleeding. Methods: We performed a cohort study in patients with historically lowest VWF levels of 0.31 to 0.60 IU/ml. Clinical data of patients were retrospectively collected. Results: We included 439 patients with low VWF. During a follow-up of 6.3 ± 3.7 years, 259 surgical procedures, 81 deliveries, and 109 spontaneous and traumatic bleeding episodes were reported. The incidence of postsurgical bleeding was 2.7%, whereas 10% of deliveries was complicated by PPH. Overall, 65 patients (14.8%) had bleeding requiring treatment, which was not different between patients with historically lowest VWF levels of 0.31–0.50 and 0.51–0.60 IU/ml (p =.154). Age <18 years, abnormal bleeding score at diagnosis, and being referred for bleeding symptoms at the time of diagnosis were independent risk factors for bleeding during follow-up, and therefore included in the risk score. Conclusions: The cutoff value of low VWF diagnosis should be set at 0.60 IU/ml. Furthermore, a risk score is developed to identify individuals with a high risk for bleeding after low VWF diagnosis.

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Journal of Thrombosis and Haemostasis
Department of Hematology

Atiq, F., Wuijster, E. (Esmee), de Maat, M., Kruip, M., Cnossen, M., & Leebeek, F. (2020). Criteria for low von Willebrand factor diagnosis and risk score to predict future bleeding. Journal of Thrombosis and Haemostasis. doi:10.1111/jth.15227