Low Normal Levels of Factor V Enhance Thrombin Generation in Hemophilia a in a Tissue Factor Pathway Inhibitor Independent Manner

Background: Low normal levels (50%-75%) of factor V (FV) enhance thrombin generation in hemophilia A (Link, JTH, 2020). A mathematical model of coagulation predicts this observation that can be explained by substrate competition for FXa between FV and FVIII. Alternatively, this observation could be...

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Bibliographic Details
Published in:Blood 2024-11, Vol.144, p.2579-2579
Main Authors: Jewell, Megan P, Bernal, Alexandra, Baird, Christine, Manco-Johnson, Marilyn, Monroe, Dougald M., Neeves, Keith B.
Format: Article
Language:English
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Summary:Background: Low normal levels (50%-75%) of factor V (FV) enhance thrombin generation in hemophilia A (Link, JTH, 2020). A mathematical model of coagulation predicts this observation that can be explained by substrate competition for FXa between FV and FVIII. Alternatively, this observation could be explained by reduced inhibition of TF:FVIIa by TFPIα bound to FV-short. Aims: The objective of this study was to determine whether FXa substrate competition between FV and FVIII explains enhanced thrombin generation with decreasing FV levels independent of TFPIα. Methods: FV and FVIII were immunodepleted to
ISSN:0006-4971
DOI:10.1182/blood-2024-210722