Unfortunately, bleeding is not the only problem facing patients with hemophilia. Many hemophiliacs also have chronic hepatitis and are infected with the human immunodeficiency virus type 1 (HIV-1) as a result of their exposure to blood products. In hemophilic patients who received plasma products prior to the mid-1980s, 90 percent have serologic evidence of hepatitis B, 85 to 100 percent have hepatitis C antibodies, and 60 to 90 percent have HIV-1 infection. Those most likely to be infected with HIV-1 are patients with severe hemophilia A without an inhibitor. Hemophilia B patients are less likely to be infected with HIV-1 as a result of the products they receive for treatment—about 50 percent are infected. Since 1985, acquired immunodeficiency syndrome (AIDS) has become the leading cause of death in patients with hemophilia. Hemophilic patients with AIDS account for 1 percent of the total U.S. AIDS population. Since 1986, as a result of new viral inactivation procedures, there have been few seroconversions resulting from the use of currently available factor replacement products.
Patients with hemophilia are usually identified in childhood or adolescence; however, those with a mild deficiency may go undetected until there is a major hemostatic challenge. Occasionally, an emergency physician will encounter a patient with acute bleeding who may have an undiagnosed coagulopathy. Tab.l„e...2.12-2i lists some historical and clinical findings that might be indicative of the presence of a bleeding disorder. Hematologic consultation should be obtained for these patients.
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