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  • Title: Current management of acquired factor VIII inhibitors.
    Author: Barnett B, Kruse-Jarres R, Leissinger CA.
    Journal: Curr Opin Hematol; 2008 Sep; 15(5):451-5. PubMed ID: 18695367.
    Abstract:
    PURPOSE OF REVIEW: Acquired hemophilia is a rare autoimmune disease that can result in life-threatening bleeding if not treated effectively. Appropriate management requires the urgent treatment of bleeding episodes and prompt institution of immunosuppressive therapy for long-term inhibitor eradication. RECENT FINDINGS: Acute bleeding episodes are generally best controlled with 'bypassing' hemostatic factor concentrates. Corticosteroid-based immunosuppressive therapy is effective in eliminating most acquired inhibitors; additional therapies, such as rituximab, are useful for patients who do not respond to standard immune-suppressing regimens. Up to 20% of patients relapse after immunomodulation and require additional treatment. A lack of controlled clinical data hampers the optimal selection of immunosuppressive therapy. SUMMARY: Patients with acquired hemophilia remain at risk for severe hemorrhage until their inhibitors are permanently eradicated. Concurrent with bleed management, immunomodulation should be initiated with corticosteroid-based therapy in order to eliminate the autoantibody and restore normal hemostasis.
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