Treatment

1. Treatment of bleeding

2. Eradication of inhibitor if present

  • Typically with corticosteroids and cyclophosphamide or rituximab.

3. Treatment of underlying condition

  • Treat amyloidosis if present.

In this case, the patient was treated with prothrombin complex concentrate (PCC) 20-30 factor IX units/kg with additional PCC 10-20 units/kg at 24 hour intervals, when needed, adjusted to maintain factor X activity at > 20%. She also received the antifibrinolytic agent, tranexamic acid. Finally, her amyloidosis was treated with Velcade, dexamethasone and cyclophosphamide. Her PT and aPTT normalized over the next 6 months, and she did not have another bleeding episode.

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