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Author Notes:

Robert F Sidonio Jr, MD MSC, Department of Pediatrics, Aflac Cancer and Blood Disorders Center, Emory University, 1760 Haygood Drive; Health Sciences Research Building Suite 340; Atlanta, GA 30322,(phone) 404-785-1637; (fax) 404-727-3681. robert.sidonio@choa.org

Allison Paroskie, MD MSCI conceptualized and designed the study, performed data collection, carried out the initial analyses, drafted the initial manuscript, and approved the final manuscript submitted; Dave Gailani, MD, Michael R. DeBaun, MD MPH, and Robert F Sidonio Jr, MD MSC all contributed to the design of the study, reviewed and revised the manuscript and approved the final manuscript as submitted.

We would also like to thank all of the patients and family members who participated in this study.

The authors declare that they have no financial conflicts.

The authors declare that they have no financial conflicts.

Robert Sidonio has received honoraria for participation in advisory boards for Grifols. Baxter, Bayer, Pfizer, Biogen, CSL Behring and Kedrion.

Subject:

Research Funding:

UL1 TR000445 from NCATS/NIH

Vanderbilt CTSA grant 1 UL1 RR024975 from NCRR/NIH

CA154267 from NIH, Conducting Research in Pediatric Hematology/Oncology

Vanderbilt Clinical & Translational Research Scholars Program, KL2

Investigator-initiated grant from Grifols.

Hemostasis and Thrombosis Research Society (HTRS) Mentored Research Award

Hemophilia of Georgia Clinical Scientist Development Grant

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Hematology
  • coagulation
  • haemostasis
  • haemophilia
  • factor VIII
  • PREDICTOR
  • VWD

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

Tools:

Journal Title:

British Journal of Haematology

Volume:

Volume 170, Number 2

Publisher:

, Pages 223-228

Type of Work:

Article | Post-print: After Peer Review

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<sup>TM</sup> 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.

Copyright information:

© 2015 John Wiley & Sons Ltd.

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