Laboratory presentation and remission status of patients with AHA with at least 12 weeks of follow-up on IST regimen (n = 55)
| . | None, n = 4 . | Glucocorticoids monotherapy, n = 6 . | Glucocorticoids & cyclophosphamide, n = 3 . | Rituximab & glucocorticoids, n = 10 . | Rituximab monotherapy, n = 14 . | Rituximab and other IST, n = 17 . | MMF monotherapy n = 1 . | Total, n = 55 . |
|---|---|---|---|---|---|---|---|---|
| Lowest FVIII activity level,∗ median (range) | 2% (<1%-3%) | <1% (<1%-23%) | <1% (<1%-<1%) | <1% (<1%-9%) | <1% (<1%-7%) | <1% (<0.3%-5%) | <1% | <1% (<0.3%-23%) |
| Maximum FVIII inhibitor titer,† median (range) | 19 (0.8-243) | 16 (4-749) | 100 (69-368) | 30 (3.8-660.5) | 44 (9.2-169) | 140 (10-1100) | 33 | 69 (0.8-1100) |
| Remission status‡ | ||||||||
| CR | 2 (50%) | 3 (50%) | 0 (0%) | 6 (60%) | 9 (64%) | 11 (65%) | 0 (0%) | 31 (56%) |
| PR | 0 (0%) | 0 (0%) | 0 (0%) | 0 (0%) | 1 (7%) | 1 (6%) | 0 (0%) | 2 (4%) |
| Not in remission/relapse | 1 (25%) | 3 (50%) | 2 (67%) | 3 (30%) | 2 (14%) | 2 (12%) | 1 (100%) | 14 (25%) |
| Deceased | 1 (25%) | 0 (0%) | 0 (0%) | 1 (10%) | 0 (0%) | 3 (18%) | 0 (0%) | 5 (9%) |
| Unknown | 0 (0%) | 0 (0%) | 1 (33%) | 0 (0%) | 2 (14%) | 0 (0%) | 0 (0%) | 3 (5%) |
| . | None, n = 4 . | Glucocorticoids monotherapy, n = 6 . | Glucocorticoids & cyclophosphamide, n = 3 . | Rituximab & glucocorticoids, n = 10 . | Rituximab monotherapy, n = 14 . | Rituximab and other IST, n = 17 . | MMF monotherapy n = 1 . | Total, n = 55 . |
|---|---|---|---|---|---|---|---|---|
| Lowest FVIII activity level,∗ median (range) | 2% (<1%-3%) | <1% (<1%-23%) | <1% (<1%-<1%) | <1% (<1%-9%) | <1% (<1%-7%) | <1% (<0.3%-5%) | <1% | <1% (<0.3%-23%) |
| Maximum FVIII inhibitor titer,† median (range) | 19 (0.8-243) | 16 (4-749) | 100 (69-368) | 30 (3.8-660.5) | 44 (9.2-169) | 140 (10-1100) | 33 | 69 (0.8-1100) |
| Remission status‡ | ||||||||
| CR | 2 (50%) | 3 (50%) | 0 (0%) | 6 (60%) | 9 (64%) | 11 (65%) | 0 (0%) | 31 (56%) |
| PR | 0 (0%) | 0 (0%) | 0 (0%) | 0 (0%) | 1 (7%) | 1 (6%) | 0 (0%) | 2 (4%) |
| Not in remission/relapse | 1 (25%) | 3 (50%) | 2 (67%) | 3 (30%) | 2 (14%) | 2 (12%) | 1 (100%) | 14 (25%) |
| Deceased | 1 (25%) | 0 (0%) | 0 (0%) | 1 (10%) | 0 (0%) | 3 (18%) | 0 (0%) | 5 (9%) |
| Unknown | 0 (0%) | 0 (0%) | 1 (33%) | 0 (0%) | 2 (14%) | 0 (0%) | 0 (0%) | 3 (5%) |
FVIII activity level was measured with either a 1-stage or chromogenic assay.
FVIII inhibitor titer units are BU/mL.
CR was defined as a normalized FVIII without an inhibitor while off IST. Patients were considered in a PR with a FVIII of at least 50% while on IST and/or with a persistent inhibitor.