Treatment modalities
| Caplacizumab dosing | 10 mg od n = 13 |
| 5 mg od n = 3 | |
| Immunosuppressive/adjuvant treatment started during admission | |
| Glucocorticoids | 16 (100%) |
| Methylprednisolone 1 gm od (n = 9) | |
| Methylprednisolone 500 mg (n = 2) | |
| Prednisolone 1 mg/kg (n = 5, including the 2 children <40 kg in weight) | |
| Anti-CD20 | 16 (100%) |
| Rituximab or biosimilar | 375 mg/m2, median 4 doses (range, 4-8) |
| MMF | 1 (6%) |
| Caplacizumab dosing | 10 mg od n = 13 |
| 5 mg od n = 3 | |
| Immunosuppressive/adjuvant treatment started during admission | |
| Glucocorticoids | 16 (100%) |
| Methylprednisolone 1 gm od (n = 9) | |
| Methylprednisolone 500 mg (n = 2) | |
| Prednisolone 1 mg/kg (n = 5, including the 2 children <40 kg in weight) | |
| Anti-CD20 | 16 (100%) |
| Rituximab or biosimilar | 375 mg/m2, median 4 doses (range, 4-8) |
| MMF | 1 (6%) |
MMF, mycophenolate mofetil; od, once daily.