Patient outcomes
| Time to normalization of platelet count, median (range), mean, d | 5.5 (3-28), 7.4 |
| No. of patients managed with PEX | 15 |
| No. of patients managed with plasma infusion | 1 |
| No. of exchanges, median (range), d | 6 (3-18) |
| No. of PEXs used before platelet count normalization (>150 × 109/L) | 5 (2-9) |
| Duration of PEX treatment before platelet count normalization (>150 × 109/L) | 5.5 (3-28) |
| Length of caplacizumab treatment, median (range), d | 31 (15-74) |
| Duration of hospital admission, median (range), d | 11 (5-26) |
| VWF levels on samples taken while on caplacizumab (n = 10) 10 mg od | VWF:Ag median 86 (43-194 IU/dL) |
| Normal range according to a laboratory (most 50-150 IU/dL) | VWF:Act median 2 (2-57 IU/dL) |
| Sample date taken after caplacizumab initiation: median, 11 d; mean, 19.5 d; minimum, 7; maximum, 63 d | FVIII median, 60 (6-156 IU/dL) |
| VWF levels on samples taken while on caplacizumab (n = 1) 5 mg od | VWF:Ag 94 |
| Normal range, 50-150 IU/dL | VWF:Act 2 |
| Sample date taken at 2 d after caplacizumab initiation | FVIII, 76 |
| ADAMTS13 at cessation of caplacizumab treatment | 63 (23.9- 89.6) |
| Time to normalization of ADAMTS13 (>64 IU/dL), median (range), d | 35 (8-149) |
| Time to normalization of platelet count, median (range), mean, d | 5.5 (3-28), 7.4 |
| No. of patients managed with PEX | 15 |
| No. of patients managed with plasma infusion | 1 |
| No. of exchanges, median (range), d | 6 (3-18) |
| No. of PEXs used before platelet count normalization (>150 × 109/L) | 5 (2-9) |
| Duration of PEX treatment before platelet count normalization (>150 × 109/L) | 5.5 (3-28) |
| Length of caplacizumab treatment, median (range), d | 31 (15-74) |
| Duration of hospital admission, median (range), d | 11 (5-26) |
| VWF levels on samples taken while on caplacizumab (n = 10) 10 mg od | VWF:Ag median 86 (43-194 IU/dL) |
| Normal range according to a laboratory (most 50-150 IU/dL) | VWF:Act median 2 (2-57 IU/dL) |
| Sample date taken after caplacizumab initiation: median, 11 d; mean, 19.5 d; minimum, 7; maximum, 63 d | FVIII median, 60 (6-156 IU/dL) |
| VWF levels on samples taken while on caplacizumab (n = 1) 5 mg od | VWF:Ag 94 |
| Normal range, 50-150 IU/dL | VWF:Act 2 |
| Sample date taken at 2 d after caplacizumab initiation | FVIII, 76 |
| ADAMTS13 at cessation of caplacizumab treatment | 63 (23.9- 89.6) |
| Time to normalization of ADAMTS13 (>64 IU/dL), median (range), d | 35 (8-149) |
MMF, mycophenolate mofetil; od, once daily.