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A cross‐sectional study of bleeding phenotype in haemophilia A carriers
Author(s) -
Paroskie Allison,
Gailani Dave,
DeBaun Michael R.,
Sidonio Robert F.
Publication year - 2015
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13423
Subject(s) - haemophilia a , haemophilia , medicine , hemarthrosis , partial thromboplastin time , thromboelastometry , population , platelet , bleeding time , von willebrand factor , bleeding diathesis , coagulopathy , von willebrand disease , prothrombin time , gastroenterology , immunology , pediatrics , surgery , platelet aggregation , environmental health
Summary 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 ‐1 VWD 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.