ArticleJournal of thrombosis and haemostasis : JTH2021
ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.
Article in Journal of thrombosis and haemostasis : JTH, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 47 papers, 2 of them syntheses that pooled it.
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Who cites it
47 citing papers in PubMed, 2 syntheses or guidelines pooled it, 77 citations in OpenAlex.
- Vascular Complications of Long COVID-From Endothelial Dysfunction to Systemic Thrombosis: A Systematic Review.International journal of molecular sciences · 2025Pooled it
- Prognostic value of von Willebrand factor and ADAMTS13 in patients with COVID-19: A systematic review and meta-analysis.Thrombosis research · 2022Pooled it
- Coagulation Profile of Convalescent Plasma Donors and Recipients.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisTrial
- Kidney Stone Disease: Epigenetic Dysregulation in Homocystinuria and Mitochondrial Sulfur Trans-Sulfuration Ablation Driven by COVID-19 Pathophysiology.Biomolecules · 2025Review
- Pituitary Apoplexy in a Child with Short Stature and Possible Recent SARS-CoV-2 Infection.Diagnostics (Basel, Switzerland) · 2025Article
- Comparative cohort study of post-acute COVID-19 infection with a nested, randomized controlled trial of ivabradine for those with postural orthostatic tachycardia syndrome (the COVIVA study).Frontiers in neurology · 2025Article
- Pituitary Apoplexy: a re-appraisal of risk factors and best management strategies in the COVID-19 era.Pituitary · 2024Review
- ADAMTS13 or Caplacizumab Reduces the Accumulation of Neutrophil Extracellular Traps and Thrombus in Whole Blood of COVID-19 Patients under Flow.Thrombosis and haemostasis · 2024Article
- Isaridin E Protects against Sepsis by Inhibiting Von Willebrand Factor-Induced Endothelial Hyperpermeability and Platelet-Endothelium Interaction.Marine drugs · 2024Article
- Combined coagulation and inflammation markers as predictors of venous thrombo-embolism and death in COVID-19.Frontiers in medicine · 2024Article
- VWF-ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion.Blood advances · 2023Article
- Blood Coagulation and Thrombotic Disorders following SARS-CoV-2 Infection and COVID-19 Vaccination.Biomedicines · 2023Review
- NLRP3 inflammasome and interleukin-1 contributions to COVID-19-associated coagulopathy and immunothrombosis.Cardiovascular research · 2023Article
- Status of major hemostatic components in the setting of COVID-19: the effect on endothelium, platelets, coagulation factors, fibrinolytic system, and complement.Annals of hematology · 2023Review
- Missense Variants of von Willebrand Factor in the Background of COVID-19 Associated Coagulopathy.Genes · 2023Article
- Endothelial cell activation, Weibel-Palade body secretion, and enhanced angiogenesis in severe COVID-19.Research and practice in thrombosis and haemostasis · 2023Article
- Ultrasound during the COVID-19 Pandemic: A Global Approach.Journal of clinical medicine · 2023Review
- Effects of convalescent plasma infusion on the ADAMTS13-von Willebrand factor axis and endothelial integrity in patients with severe and critical COVID-19.Research and practice in thrombosis and haemostasis · 2023Article
- Increased von Willebrand Factor Platelet-Binding Capacity Is Related to Poor Prognosis in COVID-19 Patients.Thrombosis and haemostasis · 2023Article
- Altered VWF:ADAMTS13 homeostasis is a target for therapeutic intervention in sickle cell disease.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
Corrections and comments
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Authors and funding
18 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundConsistent with fulminant endothelial cell activation, elevated plasma von Willebrand factor (VWF) antigen levels have been reported in patients with COVID-19. The multimeric size and function of VWF are normally regulated through A Disintegrin And Metalloprotease with ThrombSpondin Motif type 1 motif, member 13 (ADAMTS-13)--mediated proteolysis.
objectivesThis study investigated the hypothesis that ADAMTS-13 regulation of VWF multimer distribution may be impaired in severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection contributing to the observed microvascular thrombosis. PATIENTS AND
methodsPatients with COVID-19 (n = 23) were recruited from the Beaumont Hospital Intensive Care Unit (ICU) in Dublin. Plasma VWF antigen, multimer distribution, ADAMTS-13 activity, and known inhibitors thereof were assessed.
resultsWe observed markedly increased VWF collagen-binding activity in patients with severe COVID-19 compared to controls (median 509.1 versus 94.3 IU/dl). Conversely, plasma ADAMTS-13 activity was significantly reduced (median 68.2 IU/dl). In keeping with an increase in VWF:ADAMTS-13 ratio, abnormalities in VWF multimer distribution were common in patients with COVID-19, with reductions in high molecular weight VWF multimers. Terminal sialylation regulates VWF susceptibility to proteolysis by ADAMTS-13 and other proteases. We observed that both N- and O-linked sialylation were altered in severe COVID-19. Furthermore, plasma levels of the ADAMTS-13 inhibitors interleukin-6, thrombospondin-1, and platelet factor 4 were significantly elevated.
conclusionsThese findings support the hypothesis that SARS-CoV-2 is associated with profound quantitative and qualitative increases in plasma VWF levels, and a multifactorial down-regulation in ADAMTS-13 function. Further studies will be required to determine whether therapeutic interventions to correct ADAMTS-13-VWF multimer dysfunction may be useful in COVID-microvascular thrombosis and angiopathy.
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