ArticleBlood2024
Type 1 VWD classification revisited: novel insights from combined analysis of the LoVIC and WiN studies.
Article in Blood, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 citations in OpenAlex.
- DDAVP response and its determinants in bleeding disorders: a systematic review and meta-analysis.Blood · 2025Pooled it
- Seventh Åland Island Meeting on von Willebrand Disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2026Article
- A multicenter prospective observational cohort study to improve diagnostic yield and clinical management of persons with a Bleeding Disorder of Unknown Cause (BDUC-iN study protocol).Research and practice in thrombosis and haemostasis · 2026Observational
- The interplay of von Willebrand disease and women's reproductive health.Blood advances · 2026Review
- Landscape and Spectrum of VWF Variants in Type 2 Von Willebrand Disease: Insights from a German Patient Cohort.Thrombosis and haemostasis · 2026Article
- Molecular genetic testing in von Willebrand disease: past, present, and beyond.Haematologica · 2026Review
- Clinical and laboratory diagnosis of von Willebrand disease.Haematologica · 2026Review
- (Un) Diagnosing von Willebrand disease.Hematology. American Society of Hematology. Education Program · 2025Review
- Article
- Effect of age on ISTH-BAT scores and low VWF diagnosis in the Zimmerman Program.Blood advances · 2025Article
- Implementation and clinical utility of multigene panels for bleeding, platelet, and thrombotic disorders.Journal of thrombosis and haemostasis : JTH · 2025Review
- Article
- Clinical utility of panel-based genetic sequencing for von Willebrand disease.Research and practice in thrombosis and haemostasis · 2025Article
- Rewriting the script: gene therapy and genome editing for von Willebrand Disease.Frontiers in genome editing · 2025Review
- Low von Willebrand factor-unraveling an enigma wrapped in a conundrum.Journal of thrombosis and haemostasis : JTH · 2024Review
- Patient-reported data on the severity of Von Willebrand disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2024Article
- von Willebrand disease.Nature reviews. Disease primers · 2024Review
- O-glycan determinants regulate VWF trafficking to Weibel-Palade bodies.Blood advances · 2024Article
- Illustrated State-of-the-Art Capsules of the ISTH 2024 Congress.Research and practice in thrombosis and haemostasis · 2024Article
- Assessing the Impact of the 2021 VWD Guidelines on the Diagnosis/Management of Low VWF Patients.Haemophilia : the official journal of the World Federation of HemophiliaArticle
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Authors and funding
23 authors at 13 institutions in 5 countries.
Funding
Abstract
abstractThere is significant ongoing debate regarding type 1 von Willebrand disease (VWD) defintion. Previous guidelines recommended patients with von Willebrand factor (VWF) levels <30 IU/dL be diagnosed type 1 VWD, whereas patients with significant bleeding and VWF levels from 30 to 50 IU/dL be diagnosed with low VWF. To elucidate the relationship between type 1 VWD and low VWF in the context of age-induced increases in VWF levels, we combined data sets from 2 national cohort studies: 162 patients with low VWF from the Low VWF in Ireland Cohort (LoVIC) and 403 patients with type 1 VWD from the Willebrand in The Netherlands (WiN) studies. In 47% of type 1 VWD participants, VWF levels remained <30 IU/dL despite increasing age. Conversely, VWF levels increased to the low VWF range (30-50 IU/dL) in 30% and normalized (>50 IU/dL) in 23% of type 1 VWD cases. Crucially, absolute VWF antigen (VWF:Ag) levels and increase of VWF:Ag per year overlapped between low VWF and normalized type 1 VWD participants. Moreover, multiple regression analysis demonstrated that VWF:Ag levels in low VWF and normalized type 1 VWD patients would not have been different had they been diagnosed at the same age (β = 0.00; 95% confidence interval, -0.03 to 0.04). Consistently, no difference was found in the prevalence of VWF sequence variants; factor VIII activity/VWF:Ag or VWF propeptide/VWF:Ag ratios; or desmopressin responses between low VWF and normalized type 1 VWD patients. In conclusion, our findings demonstrate that low VWF does not constitute a discrete clinical or pathological entity. Rather, it is part of an age-dependent type 1 VWD evolving phenotype. Collectively, these data have important implications for future VWD classification criteria.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.