Management of Acquired Hemophilia in the Emergency Department
Acquired hemophilia A is a rare but potentially life-threatening autoimmune bleeding disorder that results from the development of autoantibodies that are directed against, and interfere with, the activity of clotting Factor VIII. Inhibition of Factor VIII, which functions as a cofactor for Factor IXa in the enzymatic activation of Factor X, leads to a reduction in the generation of thrombin on the surface of activated platelets.
Patients who develop autoantibodies against Factor VIII may present with severe and sometimes catastrophic bleeding episodes, despite having no previous history of a bleeding diathesis. Spontaneous ecchymoses, retroperitoneal and cerebral hemorrhages, gross hematuria, muscle bleeding, and intractable epistaxis are common in the patients with acquired hemophilia. This pattern of bleeding is in contrast to patients with hemophilia A, which is more often characterized by bleeding into the joints or soft tissues. Retrospective studies of patients with acquired hemophilia have reported mortality rates between 8% and 22%. A recent 2-year study of all patients with acquired hemophilia A in the United Kingdom (n=172) found that bleeding was the cause of death in 9% of the patients.
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A 54-Year-Old Woman With Rheumatoid Arthritis, Bruising, Swelling, and Pain
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A 69-Year-Old Man With Prostate Cancer and Extensive Spontaneous Ecchymoses
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Contact this provider with questions about CME (Jennifer Jones: jjones@pimed.com)
