Patient characteristics and first-line immunosuppressive regimens
| Study group . | n . | Mean age, y (IQR) . | Female/male (ratio) . | Etiology, n (%) . | FVIII at diagnosis, IU/dL (IQR) . | Inhibitor titer at diagnosis, BU/mL (IQR) . | |||
|---|---|---|---|---|---|---|---|---|---|
| Idiopathic . | Autoimmune . | Malignancy . | Pregnancy . | ||||||
| All patients | 331 | 75 (63-81) | 152/179 (1:1.2) | 184 (64) | 46 (16) | 34 (12) | 23 (8) | 2 (1-6) | 15 (5-54) |
| Steroids alone | 142 | 75 (65-82) | 72/79 (1:1.1) | 82 (61) | 21 (16) | 15 (11) | 16 (12) | 3 (1-6) | 13 (5-43) |
| Steroids + cyclophosphamide | 83 | 76 (63-80) | 43/51 (1:1.2) | 55 (68) | 14 (17) | 8 (10) | 4 (5) | 1 (1-4) | 22 (8-67) |
| Rituximab-based regimens | 51 | 74 (5-78) | 23/30 (1:1.3) | 27 (64) | 7 (17) | 7 (17) | 1 (2) | 2 (0-7) | 16 (6-62) |
| Cyclosporine + steroids | 5 | 74 (42-74) | 2/3 (1:1.5) | ID | ID | ID | ID | 1 (0-9) | 22 (1-137) |
| Cytotoxics alone | 6 | 78 (58-85) | 3/3 (1:1) | ID | ID | ID | ID | 4 (1-9) | 7 (4-26) |
| FVIII-based regimens | 7 | 73 (69-80) | 5/2 (1:0.4) | ID | ID | ID | ID | 2 (0-10) | 10 (3-38) |
| Data not recorded | 37 | 77 (69-83) | 15/22 (1:1.5) | ID | ID | ID | ID | 2 (1-5) | 14 (5-55) |
| Study group . | n . | Mean age, y (IQR) . | Female/male (ratio) . | Etiology, n (%) . | FVIII at diagnosis, IU/dL (IQR) . | Inhibitor titer at diagnosis, BU/mL (IQR) . | |||
|---|---|---|---|---|---|---|---|---|---|
| Idiopathic . | Autoimmune . | Malignancy . | Pregnancy . | ||||||
| All patients | 331 | 75 (63-81) | 152/179 (1:1.2) | 184 (64) | 46 (16) | 34 (12) | 23 (8) | 2 (1-6) | 15 (5-54) |
| Steroids alone | 142 | 75 (65-82) | 72/79 (1:1.1) | 82 (61) | 21 (16) | 15 (11) | 16 (12) | 3 (1-6) | 13 (5-43) |
| Steroids + cyclophosphamide | 83 | 76 (63-80) | 43/51 (1:1.2) | 55 (68) | 14 (17) | 8 (10) | 4 (5) | 1 (1-4) | 22 (8-67) |
| Rituximab-based regimens | 51 | 74 (5-78) | 23/30 (1:1.3) | 27 (64) | 7 (17) | 7 (17) | 1 (2) | 2 (0-7) | 16 (6-62) |
| Cyclosporine + steroids | 5 | 74 (42-74) | 2/3 (1:1.5) | ID | ID | ID | ID | 1 (0-9) | 22 (1-137) |
| Cytotoxics alone | 6 | 78 (58-85) | 3/3 (1:1) | ID | ID | ID | ID | 4 (1-9) | 7 (4-26) |
| FVIII-based regimens | 7 | 73 (69-80) | 5/2 (1:0.4) | ID | ID | ID | ID | 2 (0-10) | 10 (3-38) |
| Data not recorded | 37 | 77 (69-83) | 15/22 (1:1.5) | ID | ID | ID | ID | 2 (1-5) | 14 (5-55) |
The presenting characteristics of patients treated with the various first-line therapies. There were no significant differences between the groups.
ID indicates insufficient data.