Secondary efficacy outcomes and AEs
| Secondary Efficacy Outcomes | n = 25 |
| Baseline ADAMTS13 <20% | n = 21 |
| Partial remission, n (%) | 10 (48) |
| Time to partial remission (mo), median (IQR) | 3.0 (2.8-8.5) |
| Duration of partial remission (mo), median (IQR) | 40 (16-56) |
| Complete remission, n (%) | 7 (33) |
| Time to complete remission (mo), median (IQR) | 8 (3-16) |
| Duration of complete remission (mo), median (IQR) | 16 (0-53) |
| Baseline ADAMTS13 ≥20% | n = 4 |
| ADAMTS13 relapse, n (%) | 3 (75) |
| AEs, n (%) | n = 40 |
| Patients with ≥1 AE | 11 (28) |
| Patients with 1 AE | 6 (15) |
| Patients with 2 AEs | 5 (13) |
| AEs leading to drug discontinuation | 5 (13) |
| List of AEs, n (%) | |
| Gastrointestinal | 8 (44) |
| Hepatopancreatic | 5 (28) |
| Leukopenia | 2 (11) |
| Acute myeloid leukemia | 1 (6) |
| Joint pain | 1 (6) |
| Oral aphthosis | 1 (6) |
| List of AEs leading to azathioprine stop, n (%) | |
| Hepatopancreatic | 4 (50) |
| Gastrointestinal | 3 (38) |
| Acute myeloid leukemia | 1 (13) |
| Time to adverse events (d), median (IQR)* | 50 (27-332) |
| Secondary Efficacy Outcomes | n = 25 |
| Baseline ADAMTS13 <20% | n = 21 |
| Partial remission, n (%) | 10 (48) |
| Time to partial remission (mo), median (IQR) | 3.0 (2.8-8.5) |
| Duration of partial remission (mo), median (IQR) | 40 (16-56) |
| Complete remission, n (%) | 7 (33) |
| Time to complete remission (mo), median (IQR) | 8 (3-16) |
| Duration of complete remission (mo), median (IQR) | 16 (0-53) |
| Baseline ADAMTS13 ≥20% | n = 4 |
| ADAMTS13 relapse, n (%) | 3 (75) |
| AEs, n (%) | n = 40 |
| Patients with ≥1 AE | 11 (28) |
| Patients with 1 AE | 6 (15) |
| Patients with 2 AEs | 5 (13) |
| AEs leading to drug discontinuation | 5 (13) |
| List of AEs, n (%) | |
| Gastrointestinal | 8 (44) |
| Hepatopancreatic | 5 (28) |
| Leukopenia | 2 (11) |
| Acute myeloid leukemia | 1 (6) |
| Joint pain | 1 (6) |
| Oral aphthosis | 1 (6) |
| List of AEs leading to azathioprine stop, n (%) | |
| Hepatopancreatic | 4 (50) |
| Gastrointestinal | 3 (38) |
| Acute myeloid leukemia | 1 (13) |
| Time to adverse events (d), median (IQR)* | 50 (27-332) |
Available for 9 patients.