Treatment

1. Treatment of bleeding

Recombinant factor VII (rFVIIa) and activated prothrombin complex (aPCC, also known as FEIBA) are called bypassing agents. If the first bypassing agent fails, consider switching treatment to the other bypassing agent.

Other treatment options include:

  • Porcine factor VIII (FVIII)
  • FVIII concentrate
  • Plasmapheresis and immunoadsorption 
  • Tranexamic acid:
    • Particularly for mucosal bleeding
    • Contraindicated in setting of urinary tract bleeding

2. Eradication of inhibitor

Combined immunosuppressive therapy (IST) is usually initiated in patients with acquired hemophilia A irrespective of presence of bleeding, inhibitor titer, or identification of underlying disease, and includes both:

  • Prednisone, and
  • Cyclophosphamide or rituximab

3. Treatment of underlying condition, for example:

  • Treat systemic lupus erythematosus (SLE)
  • Treat malignancy
  • Stop causative medication

In this case, investigations (including CT torso and antinuclear antibody [ANA]) failed to reveal an underlying cause of the acquired hemophilia. For the bleeding, the patient was treated with aPCC (FEIBA) 100 units/kg IV boluses every 8 hours for 3 days, and then with tapering doses for the following 4 days. The arm hematomas stabilized and he did not develop any other bleeding source. For inhibitor eradication, he was started on prednisone 1 mg/kg/day and cyclophosphamide 2 mg/kg/day. He was not started on rituximab because of concerns of rendering him more susceptible to severe COVID infection.

The following is the time course of aPTT and FVIII relative to the day of admission (day 1):

DayaPTTFVIII
Day 2035.3102
Day 19 (discharged home)37.954
Day 1841.335
Day 1745.634
Day 1651.229
Day 1552.121
Day 1459.913
Day 1360.215
Day 1288.111
Day 1194.519
Day 10998
Day 9103.67.0
Day 181.6<1