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Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay
Published on: September 9, 2012
Factor V Leiden and hemophilia
Massimo Franchini1, Giuseppe Lippi
1Immunohematology and Transfusion Center, Department of Pathology and Laboratory Medicine, University Hospital of Parma, Parma, Italy. mfranchini@ao.pr.it
Thrombosis Research
|November 26, 2009
Summary
Factor V Leiden mutation, a common inherited risk for blood clots, may offer a survival advantage by potentially reducing hemophilia symptoms. However, it can also increase clotting risk in hemophiliacs.
Area of Science:
- Hematology
- Genetics
- Evolutionary Medicine
Background:
- Factor V Leiden (FV Leiden) is the most prevalent inherited prothrombotic risk factor.
- FV Leiden impairs activated protein C's ability to inactivate factor V, increasing thrombosis risk.
- Its high prevalence suggests a potential evolutionary survival advantage.
Purpose of the Study:
- To review the interaction between FV Leiden and hemophilia (Factor VIII or IX deficiency).
- To analyze clinical effects and pathogenic mechanisms of this interaction.
- To explore FV Leiden's role in modulating hemophilia phenotypes.
Main Methods:
- Literature review of existing studies on FV Leiden and hemophilia.
- Analysis of clinical data and proposed pathogenic mechanisms.
- Focus on the interplay between prothrombotic and bleeding disorders.
Main Results:
- FV Leiden may compensate for low Factor VIII/IX levels, enhancing thrombin generation.
- This compensation can lead to attenuated clinical symptoms in hemophiliacs.
- Co-occurrence with other thrombophilic factors may increase thrombotic risk in hemophiliacs.
Conclusions:
- FV Leiden might offer a protective effect against severe bleeding in hemophilia.
- The prothrombotic nature of FV Leiden can paradoxically increase clotting risk in hemophiliacs.
- Understanding this interaction is crucial for managing hemophilia patients with FV Leiden.
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