The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Evaluation of thrombin generating capacity in plasma from patients with haemophilia A and B
Open accessAbstract
Summary: In haemophilia patients, a relationship is usually observed between the clinical expression of the disease and plasmatic factor VIII/factor IX (FVIII/FIX) activity. However, it is known from clinical experience, that some haemophilia patients, despite similar FVIII/FIX plasma levels, could exhibit different bleeding phenotype. After determining preanalytical test conditions, we evaluated the thrombin generation capacity from haemophilia plasma samples in various conditions and the potential usefulness of thrombin generation test (TGT) in haemophilia patients. In a series of 46 haemophilia patients (34 haemophilia A and 12 haemophilia B patients), we found a significant correlation between plasmatic FVIII/FIX levels and endogenous thrombin potential (ETP), peak and time to peak obtained by thrombin generation measurement. In addition, a correlation was found between severe clinical bleeding phenotype and ETP. Our results suggest that TGT could be a promising tool to evaluate haemostasis capacity in patients with haemophilia. Our ex vivo results, obtained 24 hours after FVIII concentrate administration, showed that in patients presenting similar plasmatic FVIIII levels, thrombin generation capacity may be significantly different. These results suggest that in patients with haemophilia, TGT could be useful for individually tailoring prophylactic regimens as well as for adapting clotting factors infusions in surgical situations, in addition to FVIII/FIX plasma clotting activities.
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Cites
The hemophilias--from royal genes to gene therapy
Mannucci, P. M., & Tuddenham, E. G. D. (2001) · New England Journal of Medicine · doi:10.1056/nejm200106073442307 · Cited by 1,110 · cited 1×
“These mutations result in absent or decreased FVIII/FIX procoagulant functions and markedly delayed thrombin generation, despite significant vascular injury (1).”
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Cited by
Thrombin generation and fibrin clot structure
Wolberg, A. S. (2007) · Blood Reviews · doi:10.1016/j.blre.2006.11.001 · Cited by 747
Targeting of Antithrombin in Hemophilia A or B with RNAi Therapy
Pasi, K. J., Rangarajan, S., Georgiev, P., Mant, T., Creagh, M. D., Lissitchkov, T., Bevan, D., Austin, S., Hay, C. R., Hegemann, I., et al. (2017) · New England Journal of Medicine · doi:10.1056/nejmoa1616569 · Cited by 367
Thrombin generation, fibrin clot formation and hemostasis
Wolberg, A. S., & Campbell, R. A. (2008) · Transfusion and Apheresis Science · doi:10.1016/j.transci.2007.12.005 · Cited by 355
Metadata
- Authors
- Béguin, S., Lienhart, A., Al Dieri, R., Trzeciak, C., Bordet, J., Hemker, C., Negrier, C., & Dargaud, Y.
- Venue
- Thrombosis and Haemostasis
- Publisher
- Georg Thieme Verlag KG
- Published
- 2005
- Volume
- 93
- Issue
- 03
- Pages
- 475-480
- ISSN
- 0340-6245 (ISSN-L)
- 2567-689X
- OpenAlex
- W2033408014
- Cited by
- 336
- References
- 1
- FWCI
- 9.28
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Béguin, S., Lienhart, A., Al Dieri, R., Trzeciak, C., Bordet, J., Hemker, C., Negrier, C., & Dargaud, Y. (2005). Evaluation of thrombin generating capacity in plasma from patients with haemophilia A and B. Thrombosis and Haemostasis. https://doi.org/10.1160/th04-10-0706
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