ArticleJournal of thrombosis and haemostasis : JTH2021
Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID-19 patients.
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 25 papers, 2 of them syntheses that pooled it.
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Who cites it
25 citing papers in PubMed, 2 syntheses or guidelines pooled it, 44 citations in OpenAlex.
- Endotheliopathy in Acute COVID-19 and Long COVID.International journal of molecular sciences · 2023Pooled it
- Prognostic value of von Willebrand factor and ADAMTS13 in patients with COVID-19: A systematic review and meta-analysis.Thrombosis research · 2022Pooled it
- Increased impact of GPIb receptors on platelet adhesion under flow in severe COVID-19.Biophysics reports · 2026Article
- Can COVID-19 Lead to Refractory Thrombotic Thrombocytopenic Purpura (TTP) During Pregnancy and Postpartum? A Case Report and a Review of the Literature.Clinical case reports · 2025Article
- The effects of coagulation factors on the risk of lung cancer: a Mendelian randomization study.Discover oncology · 2025Article
- SARS-CoV-2 ORF7a activates the endothelium to release von Willebrand factor that promotes thrombosis.Research and practice in thrombosis and haemostasis · 2025Article
- 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
- Combined coagulation and inflammation markers as predictors of venous thrombo-embolism and death in COVID-19.Frontiers in medicine · 2024Article
- Traumatic Brain Injury in the Long-COVID Era.Neurotrauma reports · 2024Review
- Hemophagocytic lymphohistiocytosis is associated with deficiency and closed conformation of ADAMTS-13.Research and practice in thrombosis and haemostasis · 2024Article
- A Long-Term Follow-Up Study in Immune-Mediated Thrombotic Thrombocytopenic Purpura: What Are the Outcomes?Journal of clinical medicine · 2023Article
- Dynamic Assessment of Plasma von Willebrand Factor and ADAMTS13 Predicts Mortality in Hospitalized Patients with SARS-CoV-2 Infection.Journal of clinical medicine · 2023Article
- ADAMTS13 conformation is closed in non-immune acquired thrombotic thrombocytopenic purpura of unidentified pathophysiology.Haematologica · 2023Article
- Case report: COVID-19-associated refractory thrombotic thrombocytopenic purpura complicated with Guillain-Barré syndrome.Frontiers in neurology · 2023Article
- 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
- Hereditary thrombotic thrombocytopenic purpura and COVID-19: Impacts of vaccination and infection in this rare disease.Research and practice in thrombosis and haemostasis · 2022Article
- Carriers ofViruses · 2022Article
- COVID-19 microthrombosis: unusually large VWF multimers are a platform for activation of the alternative complement pathway under cytokine storm.International journal of hematology · 2022Review
- Coagulopathy and Fibrinolytic Pathophysiology in COVID-19 and SARS-CoV-2 Vaccination.International journal of molecular sciences · 2022Review
- Routine and Advanced Laboratory Tests for Hemostasis Disorders in COVID-19 Patients: A Prospective Cohort Study.Journal of clinical medicine · 2022Article
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundCritically ill patients with coronavirus disease 2019 (COVID-19) are prone to developing macrothrombosis and microthrombosis. COVID-19 has been reported to be rarely associated with thrombotic microangiopathies. A disintegrin and metalloprotease with thrombospondin type I repeats, member 13 (ADAMTS13) severe deficiency, the hallmark of thrombotic thrombocytopenic purpura (TTP), induces the formation of platelet, unusually large von Willebrand factor (VWF) multimer microthrombi. In immune-mediated TTP, ADAMTS13 adopts specifically an open conformation. The VWF/ADAMTS13 couple may contribute to the microthrombi formation in pulmonary alveolar capillaries in COVID-19.
objectiveTo investigate clinical features, hemostatic laboratory parameters, VWF/ADAMTS13 axis, and ADAMTS13 conformation in critically ill COVID-19 patients at admission.
methodsFifty three critically ill COVID-19 patients were enrolled between March 18 and May 9 2020 in a monocentric hospital.
resultsThe median age was 59 years and the male-to-female ratio was 2.8/1. We reported seven pulmonary embolisms and 15 deaths. Biological investigations showed increased fibrinogen and factor V levels, and strongly increased D-dimers correlated with mortality. No patient presented severe thrombocytopenia nor microangiopathic hemolytic anemia. An imbalance between high VWF antigen levels and normal or slightly decreased ADAMTS13 activity levels (strongly elevated VWF/ADAMTS13 ratio) was correlated with mortality. Three patients had a partial quantitative deficiency in ADAMTS13. We also reported a closed conformation of ADAMTS13 in all patients, reinforcing the specificity of an open conformation of ADAMTS13 as a hallmark of TTP.
conclusionWe suggest that slightly decreased or normal ADAMTS13 activity and highly elevated VWF are rather biomarkers reflecting both the strong inflammation and the endothelial damage rather than drivers of the thrombotic process of COVID-19.
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Registered trials
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