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. 2015 Jul;170(2):223-8.
doi: 10.1111/bjh.13423. Epub 2015 Apr 1.

A cross-sectional study of bleeding phenotype in haemophilia A carriers

Allison Paroskie  1   2 , Dave Gailani  1 , Michael R DeBaun  2 , Robert F Sidonio Jr  3
Affiliations

A cross-sectional study of bleeding phenotype in haemophilia A carriers

Allison Paroskie et al. Br J Haematol. 2015 Jul.

Abstract

Haemophilia A carriers have historically been thought to exhibit normal haemostasis. However, recent data demonstrates that, despite normal factor VIII (FVIII), haemophilia A carriers demonstrate an increased bleeding tendency. We tested the hypothesis that obligate haemophilia carriers exhibit an increase in clinically relevant bleeding. A cross-sectional study was performed comparing haemophilia A carriers to normal women. Questionnaire assessment included a general bleeding questionnaire, condensed MCMDM-1VWD bleeding assessment tool and Pictorial Bleeding Assessment Chart (PBAC). Laboratory assessment included complete blood count, prothrombin time, activated partial thromboplastin time, fibrinogen activity, FVIII activity (

Fviii: C), von Willebrand factor antigen level, ristocetin cofactor, platelet function analyser-100(TM) and ABO blood type. Forty-four haemophilia A carriers and 43 controls were included. Demographic features were similar. Laboratory results demonstrated a statistically significant difference only in

Fviii: C (82·5 vs. 134%, P < 0·001). Carriers reported a higher number of bleeding events, and both condensed MCMDM-1 VWD bleeding scores (5 vs. 1, P < 0·001) and PBAC scores (423 vs. 182·5, P = 0·018) were significantly higher in carriers. Haemophilia A carriers exhibit increased bleeding symptoms when compared to normal women. Further studies are necessary to fully understand the bleeding phenotype in this population and optimize clinical management.

Keywords: coagulation; factor VIII; haemophilia; haemostasis.

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Conflict of interest statement

Conflict of Interest: The authors declare that they have no conflicts of interest relevant to the manuscript. Robert Sidonio has received honoraria for participation in advisory boards for Grifols. Baxter, Bayer, Pfizer, Biogen, CSL Behring and Kedrion.

Figures

Figure 1
Figure 1
Comparison of reported bleeding history between haemophilia A carriers and normal women. Statistical analysis performed with Fisher’s Exact Chi Square test.
Figure 2
Figure 2
Condensed MCMDM-1 VWD score for Haemophilia A carriers versus normal women. Statistical analysis performed with Mann Whitney U test.
Figure 3
Figure 3
Comparison of individual components of the condensed MCMDM-1VWD bleeding assessment tool between Haemophilia A carriers and normal women. Statistical analysis performed with Fisher’s Exact Chi Square test.
Figure 4
Figure 4
Historical pictorial bleeding assessment chart score for Haemophilia A carriers versus normal women. Statistical analysis performed with Mann Whitney U test.
See this image and copyright information in PMC

References

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