Response to first-line immunosuppression
| Regimen . | n . | CR, n (%) . | Days from start of immunosuppression, median (IQR) . | Relapse, n (%) . | Stable CR, n (%) . | ||
|---|---|---|---|---|---|---|---|
| Inhibitor negative . | FVIII > 70 IU/dL . | IS stopped . | |||||
| Steroids alone | 142 | 83 (58) | 34 (17-76) | 32 (15-51) | 108 (55-208) | 15 (18) | 68 (48) |
| Steroids + cyclophosphamide | 83 | 66 (80) | 32 (12-77) | 40 (18-81) | 74 (52-151) | 8 (12) | 58 (70) |
| Steroids + rituximab | 28 | 18 (64) | 46 (28-109) | 35 (26-189) | 62 (31-113) | 0 (0) | 18 (64) |
| Cytotoxic + rituximab | 3 | 2 (67) | ND | ND | ND | 0 (0) | 2 (67) |
| Steroids + cytotoxic + rituximab | 8 | 6 (75) | 50 (20-122) | 67 (45-113) | 67 (29-129) | 1 (17) | 5 (63) |
| Rituximab alone | 12 | 5 (42) | 53, 145, 209, 334* | 145, 209, 252, 334* | 21, 21, 21, 21, 22* | 0 (0) | 5 (42) |
| Rituximab + any other agent | 39 | 26 (67) | 49 (28-93) | 42 (28-138) | 67 (31-109) | 1 (3) | 25 (64) |
| All rituximab-based regimens | 51 | 31 (61) | 65 (29-144) | 64 (28-206) | 43 (22-96) | 1 (3) | 30 (59) |
| Regimen . | n . | CR, n (%) . | Days from start of immunosuppression, median (IQR) . | Relapse, n (%) . | Stable CR, n (%) . | ||
|---|---|---|---|---|---|---|---|
| Inhibitor negative . | FVIII > 70 IU/dL . | IS stopped . | |||||
| Steroids alone | 142 | 83 (58) | 34 (17-76) | 32 (15-51) | 108 (55-208) | 15 (18) | 68 (48) |
| Steroids + cyclophosphamide | 83 | 66 (80) | 32 (12-77) | 40 (18-81) | 74 (52-151) | 8 (12) | 58 (70) |
| Steroids + rituximab | 28 | 18 (64) | 46 (28-109) | 35 (26-189) | 62 (31-113) | 0 (0) | 18 (64) |
| Cytotoxic + rituximab | 3 | 2 (67) | ND | ND | ND | 0 (0) | 2 (67) |
| Steroids + cytotoxic + rituximab | 8 | 6 (75) | 50 (20-122) | 67 (45-113) | 67 (29-129) | 1 (17) | 5 (63) |
| Rituximab alone | 12 | 5 (42) | 53, 145, 209, 334* | 145, 209, 252, 334* | 21, 21, 21, 21, 22* | 0 (0) | 5 (42) |
| Rituximab + any other agent | 39 | 26 (67) | 49 (28-93) | 42 (28-138) | 67 (31-109) | 1 (3) | 25 (64) |
| All rituximab-based regimens | 51 | 31 (61) | 65 (29-144) | 64 (28-206) | 43 (22-96) | 1 (3) | 30 (59) |
The outcome of first-line immunosuppressive therapy (IS) is shown. Complete remission (CR) was defined as inhibitor-negative, FVIII > 70 IU/dL, and immunosuppressive therapy stopped. Stable CR was defined as achieving CR with no relapse during follow-up. Because the groups are not matched, it is not appropriate to make statistical comparisons between the treatment arms.
Actual days given resulting from limited data.