Platelets as Modifiers of Clinical Phenotype in Hemophilia

Platelets occupy a central role in the maintenance of hemostasis by adhering to sites of vascular injury and facilitating thrombin generation, which leads to the formation of a fibrin clot. Patients with hemophilia exhibit defective thrombin generation secondary to reduced plasma factor concentratio...

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Main Author: Donald L. Yee
Format: Article
Language:English
Published: Wiley 2006-01-01
Series:The Scientific World Journal
Online Access:http://dx.doi.org/10.1100/tsw.2006.133
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author Donald L. Yee
author_facet Donald L. Yee
author_sort Donald L. Yee
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description Platelets occupy a central role in the maintenance of hemostasis by adhering to sites of vascular injury and facilitating thrombin generation, which leads to the formation of a fibrin clot. Patients with hemophilia exhibit defective thrombin generation secondary to reduced plasma factor concentrations, which can lead to excessive and sometimes life-threatening bleeding. Individuals differ greatly with respect to platelet function and platelets from different individuals differ inherently in their ability to enact thrombin generation, the key coagulative process that is deficient in hemophilia. Similarly, some patients with hemophilia seem to bleed less often than others despite exhibiting similar plasma factor levels. The biologic factors that underlie this phenotypic variability remain poorly understood, but evidence is reviewed supporting a role for platelets and platelet-related factors in modifying bleeding tendency in patients with hemophilia and potential directions for further clinical research in this area are discussed.
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spelling doaj-art-8a67e3f68ebb4efda4066463ae5167f72025-08-20T02:09:20ZengWileyThe Scientific World Journal1537-744X2006-01-01666166810.1100/tsw.2006.133Platelets as Modifiers of Clinical Phenotype in HemophiliaDonald L. Yee0Department of Pediatrics, Hematology-Oncology Section and Department of Medicine, Thrombosis Research Section, Baylor College of Medicine, Houston, TX, USAPlatelets occupy a central role in the maintenance of hemostasis by adhering to sites of vascular injury and facilitating thrombin generation, which leads to the formation of a fibrin clot. Patients with hemophilia exhibit defective thrombin generation secondary to reduced plasma factor concentrations, which can lead to excessive and sometimes life-threatening bleeding. Individuals differ greatly with respect to platelet function and platelets from different individuals differ inherently in their ability to enact thrombin generation, the key coagulative process that is deficient in hemophilia. Similarly, some patients with hemophilia seem to bleed less often than others despite exhibiting similar plasma factor levels. The biologic factors that underlie this phenotypic variability remain poorly understood, but evidence is reviewed supporting a role for platelets and platelet-related factors in modifying bleeding tendency in patients with hemophilia and potential directions for further clinical research in this area are discussed.http://dx.doi.org/10.1100/tsw.2006.133
spellingShingle Donald L. Yee
Platelets as Modifiers of Clinical Phenotype in Hemophilia
The Scientific World Journal
title Platelets as Modifiers of Clinical Phenotype in Hemophilia
title_full Platelets as Modifiers of Clinical Phenotype in Hemophilia
title_fullStr Platelets as Modifiers of Clinical Phenotype in Hemophilia
title_full_unstemmed Platelets as Modifiers of Clinical Phenotype in Hemophilia
title_short Platelets as Modifiers of Clinical Phenotype in Hemophilia
title_sort platelets as modifiers of clinical phenotype in hemophilia
url http://dx.doi.org/10.1100/tsw.2006.133
work_keys_str_mv AT donaldlyee plateletsasmodifiersofclinicalphenotypeinhemophilia