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Curr Opin Hematol. 2008 Sep;15(5):451-5. doi: 10.1097/MOH.0b013e328309ecab.

Current management of acquired factor VIII inhibitors.

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  • 1Tulane University School of Medicine, New Orleans, Louisiana, USA.

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.

[PubMed - indexed for MEDLINE]
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