Classification

The clinical classification of the hemophilias is based on the severity of deficiency. Patients classified as mildly deficient have 6 to 60 percent of normal factor VIII or factor IX activity; moderate disease is 1 to 5 percent of normal; severe hemophilia describes patients with less than 1 percent of normal factor activity and accounts for 60 percent of all patients. The most common bleeding manifestations are outlined in T.a.b.ie 2.1.2r.l.. Unless there is other underlying disease, patients with hemophilia do not have problems with minor cuts and abrasions. Bleeding in hemophiliacs is characterized by deep hematomas and hemathroses that occur spontaneously or with minimal trauma; the bleeding is often delayed several hours after minor injury.

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