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

Paroskie A, Gailani D, DeBaun MR, Sidonio RF
Br J Haematol. 2015 170 (2): 223-8

PMID: 25832012 · PMCID: PMC4490107 · DOI:10.1111/bjh.13423

UNLABELLED - 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.

© 2015 John Wiley & Sons Ltd.

MeSH Terms (14)

Adult Blood Coagulation Blood Coagulation Tests Case-Control Studies Cross-Sectional Studies Female Hemophilia A Hemorrhage Heterozygote Humans Middle Aged Phenotype Risk Factors Young Adult

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