Current management of acquired factor VIII inhibitors.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Barnett
Brian
B
Tulane University School of Medicine, New Orleans, Louisiana, USA.
Kruse-Jarres
Rebecca
R
Leissinger
Cindy A
CA
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000187:drug effects*; D005169:Factor VIII / Q000276:immunology*; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D006470:Hemorrhage / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069283:Rituximab
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D000069283:Rituximab; D005169:Factor VIII