Factor VIII antibodies: Rx

Clinical - Hematologic

Also known as acquired Hemophillia A

  • Treat bleeding and its complications

  • If titer < 5 BU, try human FVIII infusions, aiming for plasma levels of 30– 50%

  • If titer > 5 BU, begin with rVIIa at a dose of 90–120 μg/kg IV q 3 hours. Follow clinical response.

  • If still bleeding, should use activated prothrombin complex concentrate (aPCC) or recombinant factor VIIa (rVIIa). These agents bypass Factor VIII

  • Removal or eradication of inhibitors

  • If associated with pregnancy, wait 4-8 weeks and follow titers

  • rituximab, 375 mg/m2 q week × 4. Follow inhibitor titers. If Bethesda titers have not begun to fall by week 4, institute therapy with prednisone 1 mg/kg and cyclophosphamide, 100 mg po qd.

  • If no benefit from above therapies, consider other immunosuppressive agents, including mycophenolate mofetil, cyclosporine A, azathioprine, vincristine, and 2-chlorodeoxyadenosine.

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2012

Year asked