Vascular Disease in the Gradually Aging Population of Hemophiliacs: An Underestimated Problem?
The aging population of individuals with hemophilia brings new experiences and challenges, leading to the search for the safest and most effective treatments and prevention strategies for diseases typical of older age, particularly cardiovascular and cerebrovascular diseases. It is important to recognize that these complications pose a real risk for individuals with hemophilia, a risk that has thus far been somewhat underestimated.
Risk of Vascular Diseases in Hemophiliacs
Ischemic heart disease (IHD) and ischemic stroke (IS) are major causes of death, especially in developed countries. Patients with hemophilia are generally considered a population at low risk for these diseases due to the hypocoagulable state caused by hemophilia. However, some recent data even show a higher risk of IHD, myocardial infarction, and IS in patients with hemophilia A and B compared to the general population.
Prevention and Treatment of Vascular Diseases in Individuals with Hemophilia A and B
Traditional cardiovascular (CV) risk factors (obesity, hypertension, hyperlipidemia, smoking) are present in hemophiliacs as well as in the general population. For instance, obesity may be even more common related to impaired mobility in individuals with hemophilia, and hypertension also appears to be more frequent. Thus, the prevention and treatment of known CV risk factors are essential in this patient group, as recommended by the 2015 consensus on the treatment of CV diseases in individuals with hemophilia.
According to international guidelines, in the case of acute IS and subsequent prophylaxis in hemophiliacs, low doses of antithrombotic drugs should be administered depending on the current levels of coagulation factors. Thromboprophylaxis in individuals with hemophilia who have atrial fibrillation also depends on the levels of FVIII or FIX. For patients with severe hemophilia, thromboprophylaxis is administered only if they have previously been given a concentrate of the missing coagulation factor. The decision to treat with acetylsalicylic acid or oral anticoagulants should be based on the presence of risk factors for stroke in the individual.
Interdisciplinary Collaboration is Key
In the case of CV and cerebrovascular diseases in individuals with hemophilia, close cooperation between cardiologists, neurologists, and hematologists is absolutely essential to establish the most effective and safest treatment possible.
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Source: Zano E., Milan M., Sarolo L., Pasca S. Cerebrovascular diseases in hemophiliacs: a real, but underestimated risk. Haemophilia 2018; 24 (1): e3−e5, doi: 10.1111/hae.13285.
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