Abstract | BACKGROUND: OBJECTIVE: To assess the hemostatic balance in patients with multiple coagulation disorders by using a thrombin generation assay. METHOD: RESULTS: A total of 35 patients were included: 71% and 57% had low antithrombin and factor XI levels, respectively. Protein C and protein S levels were abnormally low in 29% and 26% of the patients, respectively, whereas only 11% displayed low factor IX levels. Under baseline conditions, the thrombin generation assay revealed a significantly higher endogenous thrombin potential and thrombin peak in patients, relative to controls. After spiking with thrombomodulin, we observed impaired involvement of the protein C system. Hence, 54% of patients displayed a hypercoagulant phenotype in vitro. All the patients with a history of stroke-like episodes or thrombosis displayed this hypercoagulant phenotype. CONCLUSION: A thrombin generation assay revealed a hypercoagulant in vitro phenotype under baseline condition; this was accentuated by impaired involvement of the protein C system. This procoagulant phenotype may thus reflect the risk of severe vascular complications. Further research will have to determine whether the thrombin generation assay is predictive of vascular events.
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Authors | Tiffany Pascreau, Maria E de la Morena-Barrio, Dominique Lasne, Mercedes Serrano, Elsa Bianchini, Manoelle Kossorotoff, Nathalie Boddaert, Arnaud Bruneel, Nathalie Seta, Vicente Vicente, Pascale de Lonlay, Javier Corral, Delphine Borgel |
Journal | Journal of thrombosis and haemostasis : JTH
(J Thromb Haemost)
Vol. 17
Issue 11
Pg. 1798-1807
(11 2019)
ISSN: 1538-7836 [Electronic] England |
PMID | 31271700
(Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
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Copyright | © 2019 French National Institute of Health and Medical Research. © 2019 International Society on Thrombosis and Haemostasis. |
Chemical References |
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Topics |
- Adolescent
- Blood Coagulation
(genetics)
- Blood Coagulation Disorders, Inherited
(blood, diagnosis, genetics)
- Child
- Child, Preschool
- Coagulation Protein Disorders
(blood, diagnosis, genetics)
- Congenital Disorders of Glycosylation
(blood, diagnosis, genetics)
- Female
- Genetic Predisposition to Disease
- Humans
- Male
- Paris
- Phenotype
- Retrospective Studies
- Spain
- Thrombin
(metabolism)
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