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Boender J, Nederlof A, Meijer K, et al. ADAMTS-13 and bleeding phenotype in von Willebrand disease. Res Pract Thromb Haemost. 2020;4(8):1331-1339doi: 10.1002/rth2.12442.
Boender, J., Nederlof, A., Meijer, K., Mauser-Bunschoten, E. P., Cnossen, M. H., Fijnvandraat, K., van der Bom, J. G., de Meris, J., Laros-van Gorkom, B. A. P., van Galen, K. P. M., Eikenboom, J., de Maat, M. P. M., Leebeek, F. W. G. (2020). ADAMTS-13 and bleeding phenotype in von Willebrand disease. Research and practice in thrombosis and haemostasis, 4(8), 1331-1339. https://doi.org/10.1002/rth2.12442
Boender, Johan, et al. "ADAMTS-13 and bleeding phenotype in von Willebrand disease." Research and practice in thrombosis and haemostasis vol. 4,8 (2020): 1331-1339. doi: https://doi.org/10.1002/rth2.12442
Boender J, Nederlof A, Meijer K, Mauser-Bunschoten EP, Cnossen MH, Fijnvandraat K, van der Bom JG, de Meris J, Laros-van Gorkom BAP, van Galen KPM, Eikenboom J, de Maat MPM, Leebeek FWG. ADAMTS-13 and bleeding phenotype in von Willebrand disease. Res Pract Thromb Haemost. 2020 Oct 31;4(8):1331-1339. doi: 10.1002/rth2.12442. eCollection 2020 Nov. PMID: 33313472; PMCID: PMC7695560.
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Res Pract Thromb Haemost. 2020 Oct 31;4(8):1331-1339. doi: 10.1002/rth2.12442. eCollection 2020 Nov.

ADAMTS-13 and bleeding phenotype in von Willebrand disease.

Research and practice in thrombosis and haemostasis

Johan Boender, Angelique Nederlof, Karina Meijer, Evelien P Mauser-Bunschoten, Marjon H Cnossen, Karin Fijnvandraat, Johanna G van der Bom, Joke de Meris, Britta A P Laros-van Gorkom, Karin P M van Galen, Jeroen Eikenboom, Moniek P M de Maat, Frank W G Leebeek,

Affiliations

  1. Department of Hematology Erasmus MC, University Medical Center Rotterdam Rotterdam The Netherlands.
  2. Department of Pediatric Hematology Erasmus MC, University Medical Center Rotterdam Sophia Children's Hospital Rotterdam The Netherlands.
  3. Department Hematology University Medical Center Groningen Groningen The Netherlands.
  4. van Creveld kliniek/Department Benign Hematology University Medical Center Utrecht Utrecht The Netherlands.
  5. Department of Pediatric Hematology Emma Children's Hospital Amsterdam UMC University of Amsterdam Amsterdam The Netherlands.
  6. Department of Plasma Proteins Sanquin Research Amsterdam Netherlands.
  7. Department Clinical Epidemiology Leiden University Medical Center Leiden The Netherlands.
  8. Jon J van Rood Center for Clinical Transfusion Medicine Sanquin Research Leiden The Netherlands.
  9. Netherlands Hemophilia Society Nijkerk The Netherlands.
  10. Department Hematology Radboud University Medical Center Nijmegen The Netherlands.
  11. Department Internal Medicine division Thrombosis and Hemostasis Leiden University Medical Center Leiden The Netherlands.
  12. Einthoven Laboratory for Vascular and Regenerative Medicine Leiden University Medical Center Leiden The Netherlands.

PMID: 33313472 PMCID: PMC7695560 DOI: 10.1002/rth2.12442

Abstract

BACKGROUND: The bleeding phenotype of von Willebrand disease (VWD) varies highly between patients and can only partly be explained by von Willebrand factor (VWF) parameters. By cleaving large VWF multimers into smaller, less active multimers, ADAMTS-13 is an important regulator of VWF activity. However, it is unknown what the role of ADAMTS-13 is in individuals with VWD.

OBJECTIVES: We therefore studied how ADAMTS-13 activity is associated with the laboratory and bleeding phenotype in individuals with VWD.

METHODS: We measured ADAMTS-13 activity using the fluorescence resonance energy transfer substrate VWF 73 assay in 638 individuals with VWD in the nationwide cross-sectional Willebrand in the Netherlands study and in 36 healthy controls. The bleeding phenotype was assessed using the Tosetto bleeding score.

RESULTS: ADAMTS-13 activity was similar in individuals with VWD (109% ± 20.6%) and controls (110% ± 19.7%). ADAMTS-13 activity was higher in individuals with VWD with type 3 than those with type 1 (mean difference, 11.8%; 95% confidence interval [CI], 2.9%-20.8%) or type 2 (mean difference, 16.1%; 95% CI, 7.1%-25.1%). ADAMTS-13 activity was not associated with the Tosetto bleeding score (0.1 Tosetto bleeding score increase per 10% ADAMTS-13 increase, 95% CI, -0.2 to 0.3). Furthermore, ADAMTS-13 activity did not differ between individuals with and without a bleeding event during the year preceding blood sampling (mean difference, 1.4%; 95% CI, -2.1% to 4.9%).

CONCLUSION: ADAMTS-13 activity was highest in individuals with type 3 VWD, but it had only minor associations with VWF parameters. ADAMTS-13 activity does not influence the bleeding phenotype in individuals with VWD.

© 2020 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.

Keywords: ADAMTS‐13 protein; blood coagulation disorders; human; von Willebrand diseases; von Willebrand factor

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