Platelet degranulation and glycoprotein IIbIIIa opening are not related to bleeding phenotype in severe haemophilia A patients

Esther R. van Bladel; Roger E. G. Schutgens; Kathelijn Fischer; Philip G. de Groot; Mark Roest

doi:10.1160/TH13-07-0546

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2014; 111(06): 1022-1030
DOI: 10.1160/TH13-07-0546

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

Platelet degranulation and glycoprotein IIbIIIa opening are not related to bleeding phenotype in severe haemophilia A patients

Authors

Esther R. van Bladel

¹Department of Clinical Chemistry and Hematology, University Medical Center Utrecht, Utrecht, the Netherlands

²Van Creveld Laboratory, University Medical Center Utrecht, Utrecht, the Netherlands
Roger E. G. Schutgens

²Van Creveld Laboratory, University Medical Center Utrecht, Utrecht, the Netherlands

³Van Creveldkliniek/Department of Hematology, University Medical Center Utrecht, Utrecht, the Netherlands
Kathelijn Fischer

³Van Creveldkliniek/Department of Hematology, University Medical Center Utrecht, Utrecht, the Netherlands
Philip G. de Groot

¹Department of Clinical Chemistry and Hematology, University Medical Center Utrecht, Utrecht, the Netherlands
Mark Roest

¹Department of Clinical Chemistry and Hematology, University Medical Center Utrecht, Utrecht, the Netherlands

Abstract

Summary

Recently we reported data suggesting that platelets could compensate for the bleeding phenotype in severe haemophilia A (HA). The aim of this study was to confirm these results in a larger population with a detailed characterisation of clinical phenotype. Patients with diagnostic severe HA (FVIII:C <1%) were scored for clinical phenotype by integrating data on age at first joint bleed, joint damage, bleeding frequency and FVIII consumption. Phenotype was defined as onset of joint bleeding-score + arthropathy-score + joint bleeding-score + (2* treatment intensity-score). After a washout period of three days, blood was collected for measurement of basal level of platelet activation, platelet reactivity, endothelial cell activation and presence of procoagulant phospholipids in plasma. Thirty-three patients with severe HA were included, 13 patients with a mild, 12 patients with an average and eight patients with a severe clinical phenotype. No relevant differences in basal level of platelet activation, platelet reactivity, endothelial cell activation and procoagulant phospholipids between all three groups were observed. The mean annual FVIII consumption per kg did not correlate with the platelet P-selectin expression and glycoprotein (GP)IIbIIIa activation on platelets. In conclusion, variability in clinical phenotype in patients with diagnostic severe HA is not related to platelet activation or reactivity, measured as platelet degranulation and platelet GPIIbIIIa opening.

Keywords

Haemophilia A - clinical phenotype - platelet reactivity - P-selectin - GPIIbIIIa

Full Text

References

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