First-line immunosuppressive regimens
| Agent . | n . | Route of administration . | Median dose, mg/kg (IQR) . |
|---|---|---|---|
| Steroid alone | |||
| Prednisone | 124 | Oral | 1.0 (0.92-1.07) |
| Dexamethasone | 3 | Not known | ND |
| Other unspecified | 15 | Not known | 1.0 (0.92-1.2) |
| Steroid + cyclophosphamide | |||
| Prednisone | 81 | Oral | 1.03 (0.97-1.03) |
| Dexamethasone | 1 | Not known | ND |
| Other steroid | 1 | Not known | 0.91 |
| Cyclophosphamide | 73 | Oral | 1.67 (1.3-2.1) |
| Cyclophosphamide | 9 | Intravenous | 5.2 (2.9-10.6) |
| Cyclophosphamide | 1 | Unknown | 1.6 |
| Rituximab-based regimens* | |||
| Rituximab alone | 12 | Intravenous | ND |
| Rituximab + steroid | |||
| Prednisone | 27 | Oral | 1.0 (0.83-1.02) |
| Other steroid | 1 | Not known | 1.1 |
| Rituximab + cytotoxic | |||
| Cyclophosphamide | Oral | 1.1 | |
| Unknown | 2 | Intravenous | 12.0, 18.0* |
| Rituximab + steroid + cytotoxic | |||
| Prednisone | 5 | Oral | 0.9 (0.94-0.96) |
| Dexamethasone | 1 | Not known | ND |
| Other steroid | 2 | Not known | 0.95, 3.3* |
| Cyclophosphamide | 3 | Oral | 1.2, 1.5, 1.8* |
| Cyclophosphamide | 3 | Intravenous | 8.8, 9.3, 17.9* |
| Azathioprine | 1 | Oral | 0.96 |
| Azathioprine | 1 | Not known | 3.1 |
| Cyclosporine + steroid | |||
| Prednisone | 3 | Oral | 1.14 (0.98-1.84) |
| Unknown steroid | 2 | Not known | |
| Cytotoxic alone | |||
| Cyclophosphamide | 3 | Oral | 1.0, 1.2, 1.8* |
| Unknown cytotoxic | 3 | Oral | 13.9, 22.2, 29.4* |
| FVIII-based regimen (n = 7) | |||
| Prednisone | 6 | Oral | 1.2 (0.9-1.5) |
| Dexamethasone | 1 | Not known | ND |
| Cyclophosphamide | 4 | Oral | 1.3, 1.7, 2.0, 2.2* |
| Other cytotoxic | 1 | Oral | 11.9 |
| No outcome data recorded | |||
| ND | 37 | ND | ND |
| Agent . | n . | Route of administration . | Median dose, mg/kg (IQR) . |
|---|---|---|---|
| Steroid alone | |||
| Prednisone | 124 | Oral | 1.0 (0.92-1.07) |
| Dexamethasone | 3 | Not known | ND |
| Other unspecified | 15 | Not known | 1.0 (0.92-1.2) |
| Steroid + cyclophosphamide | |||
| Prednisone | 81 | Oral | 1.03 (0.97-1.03) |
| Dexamethasone | 1 | Not known | ND |
| Other steroid | 1 | Not known | 0.91 |
| Cyclophosphamide | 73 | Oral | 1.67 (1.3-2.1) |
| Cyclophosphamide | 9 | Intravenous | 5.2 (2.9-10.6) |
| Cyclophosphamide | 1 | Unknown | 1.6 |
| Rituximab-based regimens* | |||
| Rituximab alone | 12 | Intravenous | ND |
| Rituximab + steroid | |||
| Prednisone | 27 | Oral | 1.0 (0.83-1.02) |
| Other steroid | 1 | Not known | 1.1 |
| Rituximab + cytotoxic | |||
| Cyclophosphamide | Oral | 1.1 | |
| Unknown | 2 | Intravenous | 12.0, 18.0* |
| Rituximab + steroid + cytotoxic | |||
| Prednisone | 5 | Oral | 0.9 (0.94-0.96) |
| Dexamethasone | 1 | Not known | ND |
| Other steroid | 2 | Not known | 0.95, 3.3* |
| Cyclophosphamide | 3 | Oral | 1.2, 1.5, 1.8* |
| Cyclophosphamide | 3 | Intravenous | 8.8, 9.3, 17.9* |
| Azathioprine | 1 | Oral | 0.96 |
| Azathioprine | 1 | Not known | 3.1 |
| Cyclosporine + steroid | |||
| Prednisone | 3 | Oral | 1.14 (0.98-1.84) |
| Unknown steroid | 2 | Not known | |
| Cytotoxic alone | |||
| Cyclophosphamide | 3 | Oral | 1.0, 1.2, 1.8* |
| Unknown cytotoxic | 3 | Oral | 13.9, 22.2, 29.4* |
| FVIII-based regimen (n = 7) | |||
| Prednisone | 6 | Oral | 1.2 (0.9-1.5) |
| Dexamethasone | 1 | Not known | ND |
| Cyclophosphamide | 4 | Oral | 1.3, 1.7, 2.0, 2.2* |
| Other cytotoxic | 1 | Oral | 11.9 |
| No outcome data recorded | |||
| ND | 37 | ND | ND |
Data are immunosuppressive regimens, route of administration, and dose, if known, in patients with acquired hemophilia A. Rituximab was administered intravenously, but dose and frequency were not recorded, although the median treatment time of 21 days suggests 4 infusions. The FVIII-based regimens were reported as immune tolerance and were not considered hemostatic treatment by the reporting investigator.
IQR indicates interquartile range; and ND, no data.
Actual doses given resulting from the small number of data points.