Patients with hemophilia A, factor VIII deficiency, account for 85 percent of patients with hemophilia. This X-linked, recessive disorder occurs in 1 in 10,000 live male births. Hemophilia B, factor IX deficiency, is also an X-linked recessive disorder and occurs in 1 in 25,000 to 30,000 live male births. Thirty percent of affected patients have no family history of hemophilia; spontaneous mutations are believed to be responsible. Females are generally carriers of hemophilia A or B, have 50 percent of normal factor VIII or factor IX activity, and are asymptomatic. On occasion, due to extreme lyonization, a female carrier can have lower factor VIII or factor IX levels and have clinical manifestations of the disease.
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