Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients

WiN Study Grp; Maurice Swinkels; Ferdows Atiq; Petra E. Burgisser; Iris van Moort; Karina Meijer; Jeroen Eikenboom; Karin Fijnvandraat; Karin P. M. van Galen; Joke de Meris; Saskia E. M. Schols; Johanna G. van der Bom; Marjon H. Cnossen; Jan Voorberg; Frank W. G. Leebeek; Ruben Bierings; A. J. Gerard Jansen

doi:10.1111/bjh.18145

Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients

WiN Study Grp, Maurice Swinkels, Ferdows Atiq, Petra E. Burgisser, Iris van Moort, Karina Meijer, Jeroen Eikenboom, Karin Fijnvandraat, Karin P. M. van Galen, Joke de Meris, Saskia E. M. Schols, Johanna G. van der Bom, Marjon H. Cnossen, Jan Voorberg, Frank W. G. Leebeek, Ruben Bierings, A. J. Gerard Jansen^*

^*Corresponding author for this work

Real World Studies in PharmacoEpidemiology, -Genetics, -Economics and -Therapy (PEGET)

Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.

Original language	English
Pages (from-to)	497-501
Number of pages	5
Journal	British Journal of Haematology
Volume	197
Issue number	4
DOIs	https://doi.org/10.1111/bjh.18145
Publication status	Published - May-2022

Keywords

bleeding disorders
platelet activation
platelet factor 4
VWD
VWF

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Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patientsFinal publisher's version, 773 KBLicence: CC BY

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WiN Study Grp, Swinkels, M., Atiq, F., Burgisser, P. E., van Moort, I., Meijer, K., Eikenboom, J., Fijnvandraat, K., van Galen, K. P. M., de Meris, J., Schols, S. E. M., van der Bom, J. G., Cnossen, M. H., Voorberg, J., Leebeek, F. W. G., Bierings, R., & Jansen, A. J. G. (2022). Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients. British Journal of Haematology, 197(4), 497-501. https://doi.org/10.1111/bjh.18145

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title = "Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients",

abstract = "Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.",

keywords = "bleeding disorders, platelet activation, platelet factor 4, VWD, VWF",

author = "{WiN Study Grp} and Maurice Swinkels and Ferdows Atiq and Burgisser, {Petra E.} and {van Moort}, Iris and Karina Meijer and Jeroen Eikenboom and Karin Fijnvandraat and {van Galen}, {Karin P. M.} and {de Meris}, Joke and Schols, {Saskia E. M.} and {van der Bom}, {Johanna G.} and Cnossen, {Marjon H.} and Jan Voorberg and Leebeek, {Frank W. G.} and Ruben Bierings and Jansen, {A. J. Gerard}",

year = "2022",

month = may,

doi = "10.1111/bjh.18145",

language = "English",

volume = "197",

pages = "497--501",

journal = "British Journal of Haematology",

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WiN Study Grp, Swinkels, M, Atiq, F, Burgisser, PE, van Moort, I, Meijer, K, Eikenboom, J, Fijnvandraat, K, van Galen, KPM, de Meris, J, Schols, SEM, van der Bom, JG, Cnossen, MH, Voorberg, J, Leebeek, FWG, Bierings, R & Jansen, AJG 2022, 'Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients', British Journal of Haematology, vol. 197, no. 4, pp. 497-501. https://doi.org/10.1111/bjh.18145

TY - JOUR

T1 - Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients

AU - WiN Study Grp

AU - Swinkels, Maurice

AU - Atiq, Ferdows

AU - Burgisser, Petra E.

AU - van Moort, Iris

AU - Meijer, Karina

AU - Eikenboom, Jeroen

AU - Fijnvandraat, Karin

AU - van Galen, Karin P. M.

AU - de Meris, Joke

AU - Schols, Saskia E. M.

AU - van der Bom, Johanna G.

AU - Cnossen, Marjon H.

AU - Voorberg, Jan

AU - Leebeek, Frank W. G.

AU - Bierings, Ruben

AU - Jansen, A. J. Gerard

PY - 2022/5

Y1 - 2022/5

N2 - Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.

AB - Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.

KW - bleeding disorders

KW - platelet activation

KW - platelet factor 4

KW - VWD

KW - VWF

U2 - 10.1111/bjh.18145

DO - 10.1111/bjh.18145

M3 - Article

VL - 197

SP - 497

EP - 501

JO - British Journal of Haematology

JF - British Journal of Haematology

SN - 0007-1048

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