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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. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-09-3879v1 108/1/52    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Plug, I. Articles by Rosendaal, F. R. Search for Related Content PubMed PubMed Citation Articles by Plug, I. Articles by Rosendaal, F. R. Related Collections Hemostasis, Thrombosis, and Vascular Biology Free Research Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Bleeding in carriers of hemophilia Iris Plug, Eveline P. Mauser-Bunschoten, Annette H. J. T. Bröcker-Vriends, Hans Kristian Ploos van Amstel, Johanna G. van der Bom, Joanna E. M. van Diemen-Homan, José Willemse, and Frits R. Rosendaal From the Leiden University Medical Center, Clinical Epidemiology, the Netherlands; University Medical Center Utrecht, Van Creveldkliniek, the Netherlands; Leiden University Medical Center, Center for Human and Clinical Genetics, the Netherlands; University Medical Center Utrecht, Department of Medical Genetics, the Netherlands; Netherlands Hemophilia Society, Badhoevedorp, the Netherlands; and Leiden University Medical Center, Thrombosis and Hemostasis Research Center, the Netherlands. A wide range of factor VIII and IX levels is observed in heterozygous carriers of hemophilia as well as in noncarriers. In female carriers, extreme lyonization may lead to low clotting factor levels. We studied the effect of heterozygous hemophilia carriership on the occurrence of bleeding symptoms. A postal survey was performed among most of the women who were tested for carriership of hemophilia in the Netherlands before 2001. The questionnaire included items on personal characteristics, characteristics of hemophilia in the affected family members, and carrier testing and history of bleeding problems such as bleeding after tooth extraction, bleeding after tonsillectomy, and other operations. Information on clotting factor levels was obtained from the hospital charts. Logistic regression was used to assess the relation of carrier status and clotting factor levels with the occurrence of hemorrhagic events. In 2004, 766 questionnaires were sent, and 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, 0.05-2.19 IU/mL) compared with 1.02 IU/mL (range, 0.45-3.28 IU/mL) in noncarriers. Clotting factor levels from 0.60 to 0.05 IU/mL were increasingly associated with prolonged bleeding from small wounds and 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. (Blood. 2006;108:52-56) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Do hemophilia carriers bleed? Yes. Louis M. Aledort Blood 2006 108: 6. [Full Text] [PDF] This article has been cited by other articles: C. Chi and R. A Kadir Management of women with inherited bleeding disorders in pregnancy Obstet Gynaecol (Lond), January 1, 2007; 9(1): 27 - 33. [Abstract] [Full Text] [PDF]

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