What are the three pillars of treatment for a patent with acquired FX deficiency?Click for Answer
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.