Current management of acquired factor VIII inhibitors.

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.