Prevalent inhibitors in haemophilia B subjects enrolled in the Universal Data Collection database

Authors

  • J. Puetz,

    Corresponding author
    1. Division of Hematology/Oncology, Department of Pediatrics, Saint Louis University, St. Louis, MO, USA
    • Correspondence: John Puetz, MD, Clinical Professor, Division of Hematology/Oncology, Department of Pediatrics, Saint Louis University, 1465 S. Grand, St. Louis, MO 63104, USA.

      Tel.: +314 577 5332; fax: +314 577 5309;

      e-mail: puetzjj@slu.edu

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  • J. M. Soucie,

    1. Division of Blood Disorders, National Center for Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA
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  • C. L. Kempton,

    1. Departments of Pediatrics and Hematology/Oncology, Emory University, Atlanta, GA, USA
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  • P. E. Monahan,

    1. Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
    2. Division of Blood Disorders, National Center for Birth Defects and Developmental Disabilities, Atlanta, GA, USA
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  • Hemophilia Treatment Center Network (HTCN) Investigators


Summary

Several risk factors for inhibitors have recently been described for haemophilia A. It has been assumed that similar risk factors are also relevant for haemophilia B, but there is limited data to confirm this notion. The aim of this study was to determine the prevalence of and risk factors associated with inhibitors in haemophilia B. The database of the Universal Data Collection (UDC) project of the Centers for Disease Control for the years 1998–2011 was queried to determine the prevalence of inhibitors in haemophilia B subjects. In addition, disease severity, race/ethnicity, age, factor exposure and prophylaxis usage were evaluated to determine their impact on inhibitor prevalence. Of the 3785 male subjects with haemophilia B enrolled in the UDC database, 75 (2%) were determined to have an inhibitor at some point during the study period. Severe disease (OR 13.1, 95% CI 6.2–27.7), black race (OR 2.2, 95% CI 1.2–4.1), and age <11 years (OR 2.5, 95% CI 1.5–4.0) were found to be significantly associated with having an inhibitor. There was insufficient data to determine if type of factor used and prophylaxis were associated with inhibitors. Inhibitors in haemophilia B are much less prevalent than haemophilia A, especially in patients with mild disease. Similar factors associated with inhibitors in haemophilia A also seem to be present for haemophilia B. The information collected by this large surveillance project did not permit evaluation of potential risk factors related to treatment approaches and exposures, and additional studies will be required.

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