SynthesisBlood advances2022
von Willebrand factor levels in the diagnosis of von Willebrand disease: a systematic review and meta-analysis.
Synthesis in Blood advances, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Laboratory assays of VWF activity and use of desmopressin trials in the diagnosis of VWD: a systematic review and meta-analysis.Blood advances · 2022Pooled it
- Seventh Åland Island Meeting on von Willebrand Disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2026Article
- The interplay of von Willebrand disease and women's reproductive health.Blood advances · 2026Review
- Clinical and laboratory diagnosis of von Willebrand disease.Haematologica · 2026Review
- Venous-significant intracranial hemorrhage in neonatal HIE treated with therapeutic hypothermia: imaging phenotypes, diagnostic pitfalls, and prognostic implications.Frontiers in neuroscience · 2026Review
- 2025 ASH ISTH NBDF WFH monitoring report on the 2021 clinical guidelines on the diagnosis and management of von Willebrand disease.Blood advances · 2025Review
- Von Willebrand disease (VWD) and pregnancy: a comprehensive overview.Thrombosis journal · 2025Review
- Systematic reviews of the literature: an introduction to current methods.American journal of epidemiology · 2025Article
- Low von Willebrand factor-unraveling an enigma wrapped in a conundrum.Journal of thrombosis and haemostasis : JTH · 2024Review
- Perioperative considerations in the paediatric patient with congenital and acquired coagulopathy.BJA open · 2024Review
- Does Desmopressin Reduce Intraoperative Bleeding in Patients Who Undergo Nasal Surgery? A Systematic Review and Meta-Analysis.Journal of rhinology : official journal of the Korean Rhinologic Society · 2024Review
- Knee replacement surgery in a patient with acquired von Willebrand disease: a case study with recommendations for patient management.Annals of medicine and surgery (2012) · 2024Article
- Laboratory diagnosis of von Willebrand disease in the age of the new guidelines: considerations based on geography and resources.Research and practice in thrombosis and haemostasis · 2023Review
- Clinical and laboratory presentation of von Willebrand disease: Experience from a single center in Saudi Arabia.Journal of Taibah University Medical Sciences · 2023Article
- The VWF binding aptamer rondoraptivon pegol increases platelet counts and VWF/FVIII in type 2B von Willebrand disease.Blood advances · 2022Article
- Perioperative Outcomes of Patients with Bleeding Disorders Undergoing Major Surgery at an Academic Hemophilia Treatment Center.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisArticle
Corrections and comments
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Authors and funding
23 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
von Willebrand disease (VWD) is associated with significant morbidity as a result of excessive mucocutaneous bleeding. Early diagnosis and treatment are important to prevent and treat these symptoms. We systematically reviewed the accuracy of diagnostic tests using different cutoff values of von Willebrand factor antigen (VWF:Ag) and platelet-dependent von Willebrand factor (VWF) activity assays in the diagnosis of VWD. We searched Cochrane Central Register for Controlled Trials, MEDLINE, and Embase databases for eligible studies. We pooled estimates of sensitivity and specificity and reported patient-important outcomes when relevant. This review included 21 studies that evaluated VWD diagnosis. The results showed low certainty in the evidence for a net health benefit from reconsidering the diagnosis of VWD vs removing the disease diagnosis in patients with VWF levels that have normalized with age. For the diagnosis of type 1 VWD, VWF sequence variants were detected in 75% to 82% of patients with VWF:Ag < 0.30 IU/mL and in 44% to 60% of patients with VWF:Ag between 0.30 and 0.50 IU/mL. A sensitivity of 0.90 (95% confidence interval [CI], 0.83-0.94) and a specificity of 0.91 (95% CI, 0.76-0.97) were observed for a platelet-dependent VWF activity/VWF:Ag ratio < 0.7 in detecting type 2 VWD (moderate certainty in the test accuracy results). VWF:Ag and platelet-dependent activity are continuous variables that are associated with an increase in bleeding risk with decreasing levels. This systematic review shows that using a VWF activity/VWF:Ag ratio < 0.7 vs lower cutoff levels in patients with an abnormal initial VWD screen is more accurate for the diagnosis of type 2 VWD.
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