Venous thrombosis in von Willebrand disease as observed in one centre and as reported in the literature

Girolami, Antonio; Tasinato, Valentina; Sambado, Luisa; Peroni, Edoardo; Casonato, Allesandra

doi:10.1097/mbc.0000000000000179

Cited by 16 publications

(13 citation statements)

References 28 publications

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“…This strong association of high FVIII or VWF levels with VT is in line with the findings that, after a first VT, patients are more prone to develop a second event with higher FVIII levels , and that patients with hemophilia A and von Willebrand disease, who have (very) low levels of FVIII or VWF, rarely suffer from VT. When episodes occur, they are mainly catheter‐associated or occur shortly after therapy with FVIII, activated FVII, FEIBA or VWF concentrates . In a recent study, FVIII levels in hemophilia A patients were elevated after gene therapy .…”

Section: Discussionmentioning

confidence: 99%

High levels of coagulation factors and venous thrombosis risk: strongest association for factor VIII and von Willebrand factor

Rietveld

Lijfering

Cessie

et al. 2019

Journal of Thrombosis and Haemostasis

104

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Elevated procoagulant levels are associated with an increased risk of venous thrombosis (VT). The dependency on concurrent increased factor levels and VT was analyzed in a large study. Factor VIII (FVIII) and von Willebrand factor (VWF) were associated with the highest VT risk. The risks for other procoagulant factor levels were largely explained by FVIII and VWF. Summary BackgroundCoagulation factors are essential for robust clot formation. However, elevated levels of procoagulant factors are associated with an increased risk of venous thrombosis (VT). The precise contribution of these factors to the development of VT is not yet understood. ObjectivesWe determined the thrombosis risk for the highest levels of eight selected coagulation factors. Furthermore, we analyzed which of these coagulation factors had the strongest impact on the supposed association. MethodsWe used data of 2377 patients with a first VT and 2940 control subjects in whom fibrinogen, von Willebrand factor (VWF), factor II, FVII, FVIII, FIX, FX and FXI levels were measured. ResultsThe odds ratios (ORs) for the various coagulation factor levels (> 99th percentile versus ≤ 25th percentile) varied between 1.8 and 4, except for FVIII (OR 23.0; 95% confidence interval [CI] 14.7–36.0) and VWF (OR 24.0; 95% CI 15.3–37.3). Adjustment for FVIII and VWF in a mediation analysis reduced the risks of the other factors to unity, with the exception of FIX and FXI (remaining ORs between 1.7 and 1.9). Conversely, the ORs for FVIII and VWF levels remained high after adjustment for all other procoagulant factors (FVIII: 16.0; 95% CI 9.7–26.3; VWF: 17.6; 95% CI 10.7–28.8). ConclusionsOur results imply that the observed relationship between VT and coagulation factor levels can be largely explained by FVIII and VWF. FVIII and VWF levels were also associated with the highest VT risk.

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Section: Discussionmentioning

confidence: 99%

High levels of coagulation factors and venous thrombosis risk: strongest association for factor VIII and von Willebrand factor

Rietveld

Lijfering

Cessie

et al. 2019

Journal of Thrombosis and Haemostasis

104

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“…577 VWF concentrates contain factor VIII, so carry a potential thromboembolic risk. 598,599 Maintaining levels less than 250% for factor VIII : C and less than 200% for VWF : RCo may reduce thrombogenicity. 557 Antithrombotic prophylaxis should be considered when other risk factors exist, particularly during periods when VWF and factor VIII levels are in the normal or supranormal range.…”

Section: Von Willebrand Disease Recommendationsmentioning

confidence: 99%

Management of severe perioperative bleeding

Kozek‐Langenecker

Ahmed

Afshari

et al. 2017

European Journal of Anaesthesiology

694

316

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: The management of perioperative bleeding involves multiple assessments and strategies to ensure appropriate patient care. Initially, it is important to identify those patients with an increased risk of perioperative bleeding. Next, strategies should be employed to correct preoperative anaemia and to stabilise macrocirculation and microcirculation to optimise the patient's tolerance to bleeding. Finally, targeted interventions should be used to reduce intraoperative and postoperative bleeding, and so prevent subsequent morbidity and mortality. The objective of these updated guidelines is to provide healthcare professionals with an overview of the most recent evidence to help ensure improved clinical management of patients. For this update, electronic databases were searched without language restrictions from 2011 or 2012 (depending on the search) until 2015. These searches produced 18 334 articles. All articles were assessed and the existing 2013 guidelines were revised to take account of new evidence. This update includes revisions to existing recommendations with respect to the wording, or changes in the grade of recommendation, and also the addition of new recommendations. The final draft guideline was posted on the European Society of Anaesthesiology website for four weeks for review. All comments were collated and the guidelines were amended as appropriate. This publication reflects the output of this work.

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“…22,23 Spontaneous VTE is also rare in patients with VWD: in a recently published review of 486 VWD patients at a single institution, for instance, there were no cases of venous thrombosis. 24 A literature review within the same publication reported 33 cases of VWD and venous thrombosis, with all but 7 cases having identifiable risk factors; the most common risk factor (N 5 14) was factor replacement therapy consisting of VWF with FVIII. The association between VTE and factor replacement is thought to be a consequence of high FVIII levels achieved during infusion.…”

Section: Vte In Bleeding Disordersmentioning

confidence: 99%

How I treat patients with inherited bleeding disorders who need anticoagulant therapy

Martin

Key

2016

Blood

106

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Situations that ordinarily necessitate consideration of anticoagulation, such as arterial and venous thrombotic events and prevention of stroke in atrial fibrillation, become challenging in patients with inherited bleeding disorders such as hemophilia A, hemophilia B, and von Willebrand disease. There are no evidence-based guidelines to direct therapy in these patients, and management strategies that incorporate anticoagulation must weigh a treatment that carries a risk of hemorrhage in a patient who is already at heightened risk against the potential consequences of not treating the thrombotic event. In this paper, we review atherothrombotic disease, venous thrombotic disease, and atrial fibrillation in patients with inherited bleeding disorders, and discuss strategies for using anticoagulants in this population using cases to illustrate these considerations.

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Venous thrombosis in von Willebrand disease as observed in one centre and as reported in the literature

Cited by 16 publications

References 28 publications

High levels of coagulation factors and venous thrombosis risk: strongest association for factor VIII and von Willebrand factor

High levels of coagulation factors and venous thrombosis risk: strongest association for factor VIII and von Willebrand factor

Management of severe perioperative bleeding

How I treat patients with inherited bleeding disorders who need anticoagulant therapy

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