Involvement of ADAMTS13 and von Willebrand factor in thromboembolic events in patients infected with SARS‐CoV‐2

Huisman, Albert; Beun, Robert; Sikma, Maaike A.; Westerink, Jan; Kuşadasi, Nuray

doi:10.1111/ijlh.13244

Cited by 69 publications

(92 citation statements)

References 13 publications

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“…In COVID-19, signs of localized pulmonary TMA were observed with typical microvascular platelet-rich thrombi identified in small vessels of the lungs and other organs. Von Willebrand factor antigen and activity were reported to be elevated and ADAMTS13 levels to be decreased, although remaining higher than 10% [27,36,37]. However, schistocytes were not identified on blood smears, signs of hemolysis were absent and platelet count was higher than expected in this condition [27].…”

Section: Introductionmentioning

confidence: 65%

“…Previous studies identified possible direct infection of endothelial cells by SARS-CoV-2 with endothelialitis [34] and increased circulatory endothelial cells, an indicator of endothelial damage [35]. As in many severe inflammatory states, thrombotic microangiopathy (TMA) may develop secondary to acquired deficiency of ADAMTS13 and subsequent increase in large von Willebrand factor multimers [27,36]. In COVID-19, signs of localized pulmonary TMA were observed with typical microvascular platelet-rich thrombi identified in small vessels of the lungs and other organs.…”

Section: Introductionmentioning

confidence: 99%

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Management of the thrombotic risk associated with COVID-19: guidance for the hemostasis laboratory

Hardy¹,

Lecompte

Douxfils

et al. 2020

Thrombosis J

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Coronavirus disease 2019 (COVID-19) is associated with extreme inflammatory response, disordered hemostasis and high thrombotic risk. A high incidence of thromboembolic events has been reported despite thromboprophylaxis, raising the question of a more effective anticoagulation. First-line hemostasis tests such as activated partial thromboplastin time, prothrombin time, fibrinogen and D-dimers are proposed for assessing thrombotic risk and monitoring hemostasis, but are vulnerable to many drawbacks affecting their reliability and clinical relevance. Specialized hemostasis-related tests (soluble fibrin complexes, tests assessing fibrinolytic capacity, viscoelastic tests, thrombin generation) may have an interest to assess the thrombotic risk associated with COVID-19. Another challenge for the hemostasis laboratory is the monitoring of heparin treatment, especially unfractionated heparin in the setting of an extreme inflammatory response. This review aimed at evaluating the role of hemostasis tests in the management of COVID-19 and discussing their main limitations.

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Section: Introductionmentioning

confidence: 65%

Section: Introductionmentioning

confidence: 99%

Management of the thrombotic risk associated with COVID-19: guidance for the hemostasis laboratory

Hardy¹,

Lecompte

Douxfils

et al. 2020

Thrombosis J

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show abstract

“…2). The increase in VWF suggests a possible similarity to thrombotic thrombocytopenic purpura, however, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels in COVID-19 although reported to be decreased, may not be severely depleted as in thrombotic thrombocytopenic purpura [26]. The increased VWF levels to 3-4 times normal values seen in patients with COVID-19 may overwhelm the ADAMTS13 activity to degrade the ultra large VWF multimers.…”

Section: The Endothelial Damage-derived Hypercoagulabilitymentioning

confidence: 96%

The coagulopathy, endotheliopathy, and vasculitis of COVID-19

2020

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Background COVID-19-associated coagulopathy (CAC) characterized by the elevated D-dimer without remarkable changes of other global coagulation markers is associated with various thrombotic complications and disease severity. The purpose of this review is to elucidate the pathophysiology of this unique coagulopathy. Methods The authors performed online search of published medical literature through PubMed using the MeSH (Medical Subject Headings) term "COVID-19," "SARS-CoV-2," "coronavirus," "coagulopathy," and "thrombus." Then, selected 51 articles that closely relevant to coagulopathy in COVID-19. Results The primary targets of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are the pneumocytes, immune cells, and vascular endothelial cells. The alveolar damage and the pulmonary microvascular thrombosis are the major causes of acute lung injury in COVID-19. The endotheliopathy that occurs is due to direct SARS-CoV-2 infection and activation of other pathways that include the immune system and thromboinflammatory responses leading to what is termed CAC. As a result, both microvascular and macrovascular thrombotic events occur in arterial, capillary, venule, and large vein vascular beds to produce multiorgan dysfunction and thrombotic complications. In addition to the endothelial damage, SARS-CoV-2 also can cause vasculitis and presents as a systemic inflammatory vascular disease. Clinical management of COVID-19 includes anticoagulation but novel therapies for endotheliopathy, hypercoagulability, and vasculitis are needed. Conclusion The endotheliopathy due to direct endothelial infection with SARS-COV-2 and the indirect damage caused by inflammation play the predominant role in the development of CAC. The intensive control of thromboinflammation is necessary to improve the outcome of this highly detrimental contagious disease.

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“…Although there have been some COVID-19 data involving ADAMTS13 levels, the small sample size in these reports precluded any major conclusions. [11][12][13] variables, two-sample Student t tests, and one-way ANOVA for three group comparisons. Youden's J statistics was used to find the optimal cutpoint for ADAMTS13 for mortality.…”

Section: Introductionmentioning

confidence: 99%

Evidence for secondary thrombotic microangiopathy in COVID-19

Sweeney

Barouqa

Krause

et al. 2020

Preprint

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The causes of coagulopathy associated with COVID-19 disease are poorly understood. We aimed to investigate the relationship between markers of endothelial activation, intravascular hemolysis, coagulation, and organ damage in COVID-19 patients and study their association with disease severity and mortality. We conducted a retrospective study of 181 hospitalized COVID-19 patients randomly selected with equal distribution of survivors and non-survivors. Patients who died had significantly lower ADAMTS13 activity, significantly higher LDH, schistocytes and von Willebrand Factor levels compared to patients discharged alive. Only 30% of patients with an initial ADAMTS13 activity <43% survived vs. 60% with ADAMTS13 >43% who survived. In conclusion, COVID-19 may manifest as a TMA-like illness in a subset of hospitalized patients. Presence of schistocytes on admission may warrant a work-up for TMA. These findings indicate the need for future investigation to study the relationship between endothelial and coagulation activation and the efficacy of TMA treatments in COVID-19.

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Involvement of ADAMTS13 and von Willebrand factor in thromboembolic events in patients infected with SARS‐CoV‐2

Cited by 69 publications

References 13 publications

Management of the thrombotic risk associated with COVID-19: guidance for the hemostasis laboratory

Management of the thrombotic risk associated with COVID-19: guidance for the hemostasis laboratory

The coagulopathy, endotheliopathy, and vasculitis of COVID-19

Evidence for secondary thrombotic microangiopathy in COVID-19

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