What is the prevalence of hemophilia A?

Updated: Jan 14, 2019
  • Author: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Hemophilia A is the most common X-linked genetic disease and the second most common factor deficiency after von Willebrand disease (vWD). The worldwide incidence of hemophilia A is approximately 1 case per 5000 males, with approximately one third of affected individuals not having a family history of the disorder. The prevalence of hemophilia A varies with the reporting country, with a range of 5.4-14.5 cases per 100,000 males.

In the United States, the prevalence of hemophilia A is 20.6 cases per 100,000 males. In 2016, the number of people in the United States with hemophilia was estimated to be about 20,000. [17]

Approximately 50-60% of patients have severe hemophilia A (FVIII < 2% of normal), associated with the severest bleeding manifestations. Approximately 25-30% have moderate hemophilia (FVIII 2-5%) and manifest bleeding after minor trauma. Those with mild hemophilia A (FVIII 6-30%) comprise 15-20% of all people with hemophilia; these patients develop bleeding only after trauma or surgery.

Acquired hemophilia A, caused by the development of an autoantibody to FVIII in a person with previously normal hemostasis, develops with a frequency of 1 case per 1 million population per year. [18] Acquired FVIII deficiency is observed in older populations, generally those older than 60 years.

The inherited, combined deficiency of factors V and VIII is a rare but recognized cause of a bleeding disorder. The prevalence is estimated to be 1 case per million population. [16]


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