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. 2019 Nov;25(6):1083-1091.
doi: 10.1111/hae.13860. Epub 2019 Oct 14.

Measurement of plasma and platelet tissue factor pathway inhibitor, factor V and Protein S in people with haemophilia

Paul E R Ellery  1   2 , Ida Hilden  3 , Peter Thyregod  4 , Nicholas D Martinez  1 , Susan A Maroney  1 , Joan C Gill  1 , Alan E Mast  1   5
Affiliations

Measurement of plasma and platelet tissue factor pathway inhibitor, factor V and Protein S in people with haemophilia

Paul E R Ellery et al. Haemophilia. 2019 Nov.

Abstract

Introduction: Tissue factor pathway inhibitor (TFPI) is a naturally occurring anticoagulant found in plasma, where it circulates bound to lipoproteins, factor V (FV) or Protein S (PS), and in platelets. Therapeutic agents targeting TFPI are under development for the treatment of haemophilia A and haemophilia B.

Aim: To begin to understand how TFPI, FV and PS interact to modulate haemophilia bleeding.

Methods: Plasma and platelet antigen concentrations of these factors were determined in 73 people with haemophilia A and 18 with haemophilia B. Using multiple regression models, these were compared to the same analytes measured in 224 male blood donors.

Results: There were no differences in plasma or platelet TFPI, FV or PS concentrations between haemophilia types or severities. However, compared to blood donors, people with haemophilia had approximately one-third lower plasma PS, 9% lower plasma TFPIα, 50% higher platelet FV and 26% lower platelet Protein S.

Conclusion: Together, the presented data suggest that individuals with haemophilia may have a compensatory procoagulant response of both plasma and platelet proteins to the decreased concentrations of FVIII or FIX.

Keywords: Protein S; factor V; haemophilia; tissue factor pathway inhibitor.

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Figures

FIGURE 1
FIGURE 1
Dot plots of Plasma Total TFPI (A), TFPIα (B), FV (C) and PS (D) in blood donors and people with haemophilia A or haemophilia B. Each dot represents an individual sample
FIGURE 2
FIGURE 2
Dot plots of Platelet TFPI (A), FV (B) and PS (C) in blood donors and people with haemophilia A or haemophilia B. Each dot represents an individual sample
See this image and copyright information in PMC

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