Hemophilia is a genetic disorder characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII or IX deficiency. Annual incidence is estimated at 1/5,000 male births and the prevalence is estimated at 1/12,000 which causes increased bleeding and usually affects males. About 70% of the time it is inherited as an X-linked recessive trait, but around 30% of cases arise from spontaneous mutations. Haemophilia A (incidence) occurs in approximately 1 in 5,000 males.The incidence of haemophilia B is 1 in 30,000 in male population.Of these, 85% have haemophilia A and 15% have hemophilia B.
Signs and symptoms
Characteristic symptoms vary with severity. In general symptoms are internal or external bleeding episodes, which are called "bleeds".Patients with more severe haemophilia suffer more severe and more frequent bleeds, while patients with mild haemophilia typically suffer more minor symptoms except after surgery or serious trauma. Moderate haemophiliacs have variable symptoms which manifest along a spectrum between severe and mild forms.
Most severe haemophilia patients require regular supplementation with intravenous recombinant or plasma concentrate Factor VIII.The prevalence by race/ethnicity was 13.2 cases/100,000 among white, 11.0 among African American, and 11.5 among Hispanic males. Application of age-specific prevalence rates from the six surveillance states to the U.S. population resulted in an estimated national population of 13,320 cases of hemophilia A and 3,640 cases of hemophilia B. For the 10-year period 1982-1991, the average incidence of hemophilia A and B in the hemophilia surveillance system (HSS) states was estimated to be 1 in 5,032 live male births. Death rate extrapolations for USA for Hemophilia: 1,681 per year, 140 per month, 32 per week, 4 per day, 0 per hour, 0 per minute, 0 per second.