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Blood, 1 July 2006, Vol. 108, No. 1, pp. 52-56. Prepublished online as a Blood First Edition Paper on March 21, 2006; DOI 10.1182/blood-2005-09-3879.
Submitted September 28, 2005
Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands * Corresponding author; email: F.R.Rosendaal{at}lumc.nl.
A wide range of factor VIII and IX levels is observed in heterozygous carriers of hemophilia, as well as in non-carriers. We studied the effect of heterozygous hemophilia carriership on the occurrence of bleeding symptoms.
A postal survey was performed among women who were tested for carriership of hemophilia in the Netherlands before 2001. The questionnaire included items on characteristics of hemophilia in the affected family members, carrier testing and bleeding problems such as bleeding after tooth extraction, bleeding after tonsillectomy and other operations.
In 2004, 766 questionnaires were sent, 546 women responded (80%). Of these 274 were carriers of hemophilia A or B. The median clotting factor level of carriers was 0.60 IU/ml (range 0.05-2.20 IU/ml) compared to 1.02 IU/ml (range 0.45-3.28 IU/ ml) in non-carriers. Clotting factor levels from 0.60 to 0.05 IU/ml were increasingly associated with prolonged bleeding from small wounds, prolonged bleeding after tooth extraction, tonsillectomy and operations.
Carriers of hemophilia bleed more than other women, especially after medical interventions. Our findings suggest that not only clotting factor levels at the extreme of the distribution, resembling mild hemophilia, but also mildly reduced clotting factor levels between 0.41 and 0.60 IU/ml are associated with bleeding.
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