Introduction

Hemophilia A and B are hereditary genetic disorders caused by clotting factor deficiency, factors VIII and IX respectively. In the era of effective therapy with factor concentrates life expectancy of patients with hemophilia has significantly increased. With increasing age, these patients face the same medical problems as other aging groups, especially atherosclerosis and cardiovascular disease. Medications which affect the clotting process, like aspirin, clopidogrel or warfarin are usually central to the treatment of these conditions. Obviously, antiplatelet therapy and anticoagulants could be potentially dangerous for patients with baseline increased risk of bleeding. This is why emphasis on prevention of cardiovascular disease is that much more important in this patient group. Data about prevalence of atherosclerotic cardiovascular risk factors in this group of patients are still suboptimal. The aim of our study was to assess prevalence of modifiable cardiovascular risk factors like hypertension, diabetes, obesity and smoking in patients with hemophilia A and B at out treatment center.

Methods

We performed retrospective chart review of patients followed at a single hemophilia treatment center in the United States. We included 59 patients with factor VIII or IX deficiency, age 30 and older, followed in clinic between 2005 and 2014. Patients with acquired hemophilia were excluded from the study. Hypertension was defined as blood pressure over 140/90 mmHg and/or the use of antihypertensive medications; diabetes mellitus as HbA1c at least 6.5% and/or use of medications for treatment of diabetes; overweight as body mass index between 25 kg/m2 and 29.9 kg/m2 and obesity as body mass index (BMI) of 30 kg/m2 or more. Patient was considered a smoker if he smoked tobacco during the month preceding last clinic visit. We chose to address the other significant risk factor namely hyperlipidemia, in more detail in a separate abstract as the newest 2013 ACC/AHA Guideline have proposed a stepwise approach to hyperlipidemia treatment.

Results

34 (57.6%) patients had hypertension [95% CI 0.44-0.70], 6 (10.2%) patients had diabetes treated with oral medications only [95% CI 0.04-0.21] , 3 (5.1%) patients had diabetes treated with insulin [95% CI 0.01-0.14], 20 (33.9%) patients were overweight [95% CI 0.22-0.47], 24 (40.7%) patients had obesity [95% CI 0.28-0.54], 23 (39%) patients were smokers [95% CI 0.27-0.53]. 3 (5.1%) patients experienced myocardial infarction and 1 (1.7%) of them died due to intracerebral hemorrhage during treatment with antiplatelet therapy.

Conclusions

Cardiovascular disease is the leading cause of death in developed countries. With increasing life expectancy of patients with hemophilia greater emphasis is needed on modifiable risk factors for prevention as treatment of cardiovascular events carries greater risk of adverse outcomes due to bleeding in these patients. Our study indicated that the prevalence of cardiovascular risk factors in hemophilia patients is high. There are only few existing publications about prevalence of hypertension, diabetes, obesity and smoking in hemophilia patients. This is an area that warrants more systematic study and a cooperative effort to generate such data at a national and even international level that in turn would assist with strategic planning and mitigation.

Disclosures

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.

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