{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T16:51:02Z","timestamp":1789750262125,"version":"4.0.1"},"reference-count":60,"publisher":"Georg Thieme Verlag KG","issue":"02","license":[{"start":{"date-parts":[[2022,1,21]],"date-time":"2022-01-21T00:00:00Z","timestamp":1642723200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2022,2]]},"abstract":"<jats:p>\n            Background\u2003Endothelial and complement activation were both associated with immunothrombosis, a key determinant of COVID-19 severity, but their interrelation has not yet been investigated.<\/jats:p><jats:p>\n            Objectives\u2003We aimed to determine von Willebrand factor (VWF) antigen (VWF:Ag) concentration, VWF collagen binding activity (VWF:CBA), a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity (ADAMTS13:Ac), and their ratios in hospitalized COVID-19 patients, and to investigate how these parameters and their constellation with complement activation relate to disease severity and in-hospital mortality in COVID-19.<\/jats:p><jats:p>\n            Methods\u2003Samples of 102 hospitalized patients with polymerase chain reaction-confirmed severe acute respiratory syndrome coronavirus 2 positivity were included in our observational cohort study. Patients were stratified according to the peak severity of COVID-19 disease in agreement with the World Health Organization ordinal scale. Twenty-six convalescent plasma donors with previous COVID-19 disease formed the control group. VWF:Ag concentration and VWF:CBA were determined by enzyme-linked immunosorbent assay (ELISA); ADAMTS13:Ac was determined by fluorescence resonance energy transfer. Complement C3 and C3a were measured by turbidimetry and ELISA, respectively. Clinical covariates and markers of inflammation were extracted from hospital records.<\/jats:p><jats:p>\n            Results\u2003VWF:Ag and VWF:CBA were elevated in all groups of hospitalized COVID-19 patients and increased in parallel with disease severity. ADAMTS13:Ac was decreased in patients with severe COVID-19, with the lowest values in nonsurvivors. High (&gt; 300%) VWF:Ag concentrations or decreased (&lt; 67%) ADAMTS13:Ac were associated with higher risk of severe COVID-19 disease or in-hospital mortality. The concomitant presence of decreased ADAMTS13:Ac and increased C3a\/C3 ratio\u2014indicating complement overactivation and consumption\u2014was a strong independent predictor of in-hospital mortality.<\/jats:p><jats:p>\n            Conclusion\u2003Our results suggest that an interaction between the VWF-ADAMTS13 axis and complement overactivation and consumption plays an important role in the pathogenesis of COVID-19.<\/jats:p>","DOI":"10.1055\/s-0041-1740182","type":"journal-article","created":{"date-parts":[[2022,1,21]],"date-time":"2022-01-21T23:37:36Z","timestamp":1642808256000},"page":"240-256","source":"Crossref","is-referenced-by-count":21,"title":["Associations between the von Willebrand Factor\u2014ADAMTS13 Axis, Complement Activation, and COVID-19 Severity and Mortality"],"prefix":"10.1055","volume":"122","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5355-7318","authenticated-orcid":false,"given":"Gy\u00f6rgy","family":"Sinkovits","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marienn","family":"R\u00e9ti","sequence":"additional","affiliation":[{"name":"Department of Haematology and Stem Cell Transplantation, Central Hospital of Southern Pest, Institute of Haematology and Infectious Diseases, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronika","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zsolt","family":"Iv\u00e1nyi","sequence":"additional","affiliation":[{"name":"Department of 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Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Be\u00e1ta","family":"Szathm\u00e1ry","sequence":"additional","affiliation":[{"name":"Department of Infectology, Central Hospital of Southern Pest, Institute of Haematology and Infectious Diseases, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Botond","family":"Lakatos","sequence":"additional","affiliation":[{"name":"Department of Infectology, Central Hospital of Southern Pest, Institute of Haematology and Infectious Diseases, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e1nos","family":"Szl\u00e1vik","sequence":"additional","affiliation":[{"name":"Department of Infectology, Central Hospital of Southern Pest, Institute of Haematology and Infectious Diseases, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilona","family":"Bobek","sequence":"additional","affiliation":[{"name":"Department of Anaesthesiology and Intensive Therapy, Central Hospital of Southern Pest, Institute of Haematology and Infectious Diseases, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zita Z.","family":"Proh\u00e1szka","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zsolt","family":"F\u00f6rh\u00e9cz","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blanka","family":"Mez\u0151","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"},{"name":"Research Group for Immunology and Haematology, Semmelweis University \u2013 E\u00f6tv\u00f6s Lor\u00e1nd Research Network (Office for Supported Research Groups), Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3610-9852","authenticated-orcid":false,"given":"Dorottya","family":"Csuka","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8156-266X","authenticated-orcid":false,"given":"Lisa","family":"Hurler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika","family":"Kajd\u00e1csi","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00e1szl\u00f3","family":"Cervenak","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Kiszel","sequence":"additional","affiliation":[{"name":"Research Group for Immunology and Haematology, Semmelweis University \u2013 E\u00f6tv\u00f6s Lor\u00e1nd Research Network (Office for Supported Research Groups), Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tam\u00e1s","family":"Masszi","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Istv\u00e1n","family":"V\u00e1lyi-Nagy","sequence":"additional","affiliation":[{"name":"Department of Haematology and Stem Cell 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