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Risk assessment for coronary heart disease in patients with haemophilia: a single centre study in the United States

Authors

  • A. S. Sait,

    1. Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, CA, USA
    2. King Fahd Specialist Hospital, Dammam, Saudi Arabia
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  • A. Kuo,

    1. Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, CA, USA
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  • R. Bettencourt,

    1. Department of Family and Preventive Medicine, Division of Epidemiology, University of California San Diego, San Diego, CA
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  • J. Bergstrom,

    1. Department of Family and Preventive Medicine, Division of Epidemiology, University of California San Diego, San Diego, CA
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  • M. Allison,

    1. Department of Family and Preventive Medicine, Division of Epidemiology, University of California San Diego, San Diego, CA
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  • A. von Drygalski

    Corresponding author
    1. Department of Medicine, Division of Hematology/Oncology, University of California San Diego, San Diego, CA, USA
    2. Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA, USA
    • Correspondence: Annette von Drygalski, MD, Pharm D, Department of Medicine, Division of Hematology/Oncology, University of California San Diego, Hemophilia and Thrombosis Treatment Center, Medical Offices South, 4168 Front Street, San Diego, CA 92103-8651, USA.

      Tel.: +(619) 471 0335; fax: +(619) 471 0338;

      e-mail: avondrygalski@ucsd.edu

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Summary

In haemophilia, coronary heart disease (CHD) occurs at a similar frequency as in the general population, but the contributing risk factors in haemophilia are incompletely understood. To investigate risk factors and 10-year CHD risk in a single centre cohort of patients with haemophilia (PWH) ≥20 years old (n = 89). We retrospectively applied the modified Framingham National Cholesterol Education Program/Adult Treatment Panel (NCEP/ATP) III risk prediction equation. Three risk levels were defined: <10% (low), 10–20% (intermediate) and >20% (high). Results were compared to the National Health and Nutrition Examination Survey (NHANES). Mean age in both cohorts was similar. Compared to NHANES, systolic blood pressures were significantly higher in PWH, but current smoking and cholesterol were lower. CHD risk differed significantly between PWH and NHANES (P = 0.005) with a higher proportion of PWH classified at low risk (77.5% vs. 61.0%). The proportion of low risk patients was also significantly higher for severe haemophilia patients compared to non-severe haemophilia patients (88.6% vs. 66.7%, P = 0.02). Among PWH, and compared to PWH who were hepatitis C (HepC) negative, HepC positive patients had significantly lower cholesterol, LDL and triglycerides. The CHD risk of HepC positive patients differed significantly from NHANES (P = 0.03) with a lower proportion of HepC positives being classified as high risk (5.7% vs. 17.3%). Favourable CHD risk classification in PWH may be influenced by low cholesterol associated with HepC infection. Estimates of CHD risk in PWH by composite scoring may not be accurate and will require studies correlating risk factors with incident CHD.

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