Table 3.
Immunosuppressant Therapies for Acquired Hemophilia A
| Medication | Dosing | Cautions |
|---|---|---|
| First-line Therapies | ||
| Corticosteroids | Prednisone 1 mg/kg PO daily, tapered as factor VIII increases | -Immunosuppressive, consider PCP prophylaxis with prolonged use -Agitation/mood disorders -Elevated blood glucose |
| Cyclophosphamide* | 1.5–2 mg/kg/day PO daily for maximum of 6 weeks (alternative IV pulse every 3–4 weeks)15 | -Increased risk of secondary malignancies with prolonged use (eg, bladder and myelodysplastic syndrome) - Myelosuppressive, monitor CBC closely - Renal toxicity, ensure adequate hydration with oral dosing -Gonadal toxicity with prolonged use |
| Rituximab* | 375 mg/m2 IV weekly x 4 | -Immunosuppression, increased risk of viral infections -Hepatitis B reactivation |
| Second-line Therapies# | ||
| Mycophenolate mofetil | 1 gram per day in divided doses increased to 2 grams per day after 1 week61 | -Myelosuppression, most commonly neutropenia, which typically resolved with dose-reduction |
| Cyclosporine* | Initial dosing 5 mg/kg per day, adjusted to trough 200–400 ug/dL60 | -Renal toxicity -Delirium -Hypertension |
| Tacrolimus* | Initial dosing 0.3 mg/kg per day, adjusted to trough 1.5 μg/dL60 | -Renal toxicity -Delirium -Hypertension |
| Cyclophosphamide, vincristine, prednisone | Cyclophosphamide 7 mg/kg IV and vincristine 2 mg IV on the first day followed by cyclophosphamide 3 mg/kg daily P.O. on days 25. Cycles repeated every 3–4 weeks if inhibitor persisted62 | -Myelosuppression -Renal toxicity -Neuropathy -Constipation (vincristine) |
| Bortezomib | 1.3 mg/m2 on days 1,4,8 and 11 on 21-day cycles64 | -Antiviral prophylaxis recommended -Neuropathy -Myelosuppressive |
Notes: *Used in combination with corticosteroids. #Off-label use of medications. Limited data available for treatment options in patients who are refractory to steroids, cyclophosphamide, and rituximab. Adapted from Kruse-Jarres R, Kempton CL, Baudo F, et al. Acquired hemophilia A: updated review of evidence and treatment guidance. Am J Hematol. 2017;92(7):695–705. with permission from © 2017 Wiley Periodicals, Inc.42