Treatment

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

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Treatment of bleeding if present
Eradication of inhibitor if present
Treatment of underlying medical condition if present

1. Treatment of bleeding

What are the 2 preferred treatment options for bleeding in patients with acquired FX deficiency?

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Human FX concentrate (where available)
Prothrombin complex concentrates (PCC)

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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