Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 170, Issue 2, Pages 223-228Publisher
WILEY-BLACKWELL
DOI: 10.1111/bjh.13423
Keywords
coagulation; haemostasis; haemophilia; factor VIII
Categories
Funding
- NCATS NIH HHS [UL1 TR000445] Funding Source: Medline
- NCI NIH HHS [CA154267, T32 CA154267] Funding Source: Medline
- NCRR NIH HHS [UL1 RR024975, 1 UL1 RR024975] Funding Source: Medline
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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 (825 vs. 134%, P<0001). Carriers reported a higher number of bleeding events, and both condensed MCMDM-1 VWD bleeding scores (5 vs. 1, P<0001) and PBAC scores (423 vs. 1825, P=0018) 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.
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