Final outcome of acquired hemophilia A by first-line immunosuppressive therapy
Regimen . | n . | Alive at final follow-up, n (%) . | Alive and inhibitor-free at final follow-up, n (%) . |
---|---|---|---|
Steroids alone | 142 | 95/135 (70) | 90/135 (67) |
Steroids + cyclophosphamide | 83 | 44/69 (64) | 43/69 (62) |
Rituximab-based regimens | 51 | 38/49 (78) | 35/49 (71) |
Regimen . | n . | Alive at final follow-up, n (%) . | Alive and inhibitor-free at final follow-up, n (%) . |
---|---|---|---|
Steroids alone | 142 | 95/135 (70) | 90/135 (67) |
Steroids + cyclophosphamide | 83 | 44/69 (64) | 43/69 (62) |
Rituximab-based regimens | 51 | 38/49 (78) | 35/49 (71) |
Final follow-up was reported in 177 patients (64%) after a median of 262 days (IQR, 66-666 days). The proportion of patients alive and inhibitor-free was similar for each first-line treatment regimen, and there were no statistically significant differences. In the steroid-alone group, 11 patients required immunosuppressive therapy to maintain remission, 1 patient required immunosuppressive therapy to maintain remission in the steroid and cyclophosphamide group, whereas 4 patients required immunosuppressive therapy to maintain remission in the rituximab-based regimen group.