Treatment modalities of the TPE-free and TPE cohorts, treated with frontline caplacizumab
| Parameter . | TPE-free cohort (n = 42) . | TPE cohort (n = 59) . | P value . |
|---|---|---|---|
| Caplacizumab treatment | |||
| Median days of caplacizumab treatment (IQR) | 21.5 (13-34) | 31 (14.5-47.5) | .09 |
| Median total doses of caplacizumab (IQR)∗ | 20 (13-30) | 29 (13-41.5) | .05 |
| Immunosuppression | |||
| Patients treated with steroids, n (%) | 42 (100) | 59 (100) | >.99 |
| Patients treated with rituximab, n (%) | 38 (90.5) | 48 (81.4) | .26 |
| Frontline <72 h, n (%) | 18 (42.9) | 14 (23.7) | .12 |
| Additional immunosuppression during first 30 d, n (%) | 1 (2.4) | 6 (10.2) | .23 |
| Azathioprine | 0 (0) | 3 (5.1) | |
| Bortezomib | 0 (0) | 1 (1.7) | |
| Ciclosporin | 0 (0) | 2 (3.4) | |
| Mycophenolate mofetil | 0 (0) | 0 (0) | |
| Obinutuzumab | 1 (2.4) | 0 (0) | |
| TPE† | |||
| Patients receiving TPE, n (%) | 4 (9.5) | 59 (100) | |
| Median start day of TPE after caplacizumab start (range) | 1.5 (1-2) | 0 (0-1) | |
| Median days of plasma exchange (IQR) | 4.5 (3.5-9) | 5 (3-6) |
| Parameter . | TPE-free cohort (n = 42) . | TPE cohort (n = 59) . | P value . |
|---|---|---|---|
| Caplacizumab treatment | |||
| Median days of caplacizumab treatment (IQR) | 21.5 (13-34) | 31 (14.5-47.5) | .09 |
| Median total doses of caplacizumab (IQR)∗ | 20 (13-30) | 29 (13-41.5) | .05 |
| Immunosuppression | |||
| Patients treated with steroids, n (%) | 42 (100) | 59 (100) | >.99 |
| Patients treated with rituximab, n (%) | 38 (90.5) | 48 (81.4) | .26 |
| Frontline <72 h, n (%) | 18 (42.9) | 14 (23.7) | .12 |
| Additional immunosuppression during first 30 d, n (%) | 1 (2.4) | 6 (10.2) | .23 |
| Azathioprine | 0 (0) | 3 (5.1) | |
| Bortezomib | 0 (0) | 1 (1.7) | |
| Ciclosporin | 0 (0) | 2 (3.4) | |
| Mycophenolate mofetil | 0 (0) | 0 (0) | |
| Obinutuzumab | 1 (2.4) | 0 (0) | |
| TPE† | |||
| Patients receiving TPE, n (%) | 4 (9.5) | 59 (100) | |
| Median start day of TPE after caplacizumab start (range) | 1.5 (1-2) | 0 (0-1) | |
| Median days of plasma exchange (IQR) | 4.5 (3.5-9) | 5 (3-6) |
All parameters are presented per episode. The TPE parameters were not evaluated for statistical significance. The TPE-free cohort comprised 42 acute iTTP episodes in 41 patients. The TPE cohort comprised 59 acute iTTP episodes in 59 patients.
Differences between total days of caplacizumab treatment and total doses of caplacizumab derive from application of alternate-day dosing regimen, as reported by our group.16 Alternate-day dosing was implemented in some patients in both cohorts and did not influence patient outcomes.
In the TPE-free cohort, application of TPE is reported for patients without an adequate response to the TPE-free treatment approach.