Demographic data of the caplacizumab group and non-caplacizumab group
| . | Caplacizumab group (n = 14) . | Non-caplacizumab group (n = 16) . | P value . |
|---|---|---|---|
| Baseline patient characteristics | |||
| Follow-up period, d | 55.0 (49.3-72.8) | 44 (35.3-61.3) | .197 |
| Age, y | 71 (62.8-80.0) | 66.5 (52.0-84.3) | .983 |
| Female sex, n/n total (%) | 6/14 (42.9%) | 9/16 (56.3%) | .715 |
| Initial episode, n/n total (%) | 13/14 (92.9%) | 15/16 (93.8%) | 1 |
| Hemoglobin, initial, g/dL | 8.1 (7.5-9.1) | 7.9 (6.7-10.0) | .901 |
| Platelet, initial, ×109/L | 11 (8-12) | 11 (7-13) | .754 |
| Lactate dehydrogenase, initial, IU/L | 1086 (931-1282) | 1038 (857-1272) | .854 |
| Serum creatinine, initial, mg/dL | 1.01 (0.68-1.51) | 1.15 (0.82-1.39) | .739 |
| Total bilirubin, initial, mg/dL | 3.9 (2.6-4.8) | 3.1 (2.5-4.2) | .394 |
| Neuropsychiatric symptoms, n/n total (%) | 11/14 (78.6%) | 9/16 (56.3%) | .26 |
| ADAMTS13 activity of <0.5%, initial, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| ADAMTS13 inhibitor, initial, BU/mL | 8.0 (4.6-12.7) | 3.3 (1.2-6.4) | .058 |
| Anti ADAMTS13 IgG antibody level, initial, U/mL | 100.7 (59.6-215.9) | 65.1 (24.6-76.0) | .212 |
| Plasma BAFF level, initial, pg/mL | 861.1 (685.3-1228.4) | 824.2 (704.9-1075) | .697 |
| Treatment | |||
| TPE, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| Number of TPE treatment, n | 8 (7-8.8) | 15 (8-20) | .025 |
| Date of final TPE, d | 8 (7-16.5) | 22 (9-27.3) | .049 |
| Glucocorticoids, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| Rituximab, n/n total (%) | 11/14 (78.6%) | 13/16 (81.4%) | 1 |
| Time from first TPE to first rituximab dose, d | 12 (4.5-15.5) | 10 (7-13) | .954 |
| Duration of caplacizumab treatment, d | 49 (39.5-59.5) | - | |
| Patient outcomes | |||
| Time from first TPE to final ADAMTS13 activity of ≥10%, d | 42 (31.0-55.0) | 23 (14.5-33.3) | .014 |
| Time from first TPE to final ADAMTS13 activity of ≥20%, d | 52 (33.0-55.0) | 25 (20.5-43.5) | .134 |
| Time after final TPE to final ADAMTS13 activity of ≥10%, d | 35 (11.3-41.3) | 2 (2.0-3.0) | <.001 |
| Time after final TPE to final ADAMTS13 activity of ≥20%, d | 38.5 (17.5-48.5) | 11.0 (3.0-18.8) | .008 |
| Time to achieve initial platelet counts of ≥100 × 109/L, d | 5 (4.0-5.0) | 8.5 (5.8-15) | <.001 |
| Time to achieve initial platelet counts of ≥150 × 109/L, d | 6 (5.0-7.0) | 14.5 (7-22.8) | .002 |
| . | Caplacizumab group (n = 14) . | Non-caplacizumab group (n = 16) . | P value . |
|---|---|---|---|
| Baseline patient characteristics | |||
| Follow-up period, d | 55.0 (49.3-72.8) | 44 (35.3-61.3) | .197 |
| Age, y | 71 (62.8-80.0) | 66.5 (52.0-84.3) | .983 |
| Female sex, n/n total (%) | 6/14 (42.9%) | 9/16 (56.3%) | .715 |
| Initial episode, n/n total (%) | 13/14 (92.9%) | 15/16 (93.8%) | 1 |
| Hemoglobin, initial, g/dL | 8.1 (7.5-9.1) | 7.9 (6.7-10.0) | .901 |
| Platelet, initial, ×109/L | 11 (8-12) | 11 (7-13) | .754 |
| Lactate dehydrogenase, initial, IU/L | 1086 (931-1282) | 1038 (857-1272) | .854 |
| Serum creatinine, initial, mg/dL | 1.01 (0.68-1.51) | 1.15 (0.82-1.39) | .739 |
| Total bilirubin, initial, mg/dL | 3.9 (2.6-4.8) | 3.1 (2.5-4.2) | .394 |
| Neuropsychiatric symptoms, n/n total (%) | 11/14 (78.6%) | 9/16 (56.3%) | .26 |
| ADAMTS13 activity of <0.5%, initial, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| ADAMTS13 inhibitor, initial, BU/mL | 8.0 (4.6-12.7) | 3.3 (1.2-6.4) | .058 |
| Anti ADAMTS13 IgG antibody level, initial, U/mL | 100.7 (59.6-215.9) | 65.1 (24.6-76.0) | .212 |
| Plasma BAFF level, initial, pg/mL | 861.1 (685.3-1228.4) | 824.2 (704.9-1075) | .697 |
| Treatment | |||
| TPE, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| Number of TPE treatment, n | 8 (7-8.8) | 15 (8-20) | .025 |
| Date of final TPE, d | 8 (7-16.5) | 22 (9-27.3) | .049 |
| Glucocorticoids, n/n total (%) | 14/14 (100%) | 16/16 (100%) | - |
| Rituximab, n/n total (%) | 11/14 (78.6%) | 13/16 (81.4%) | 1 |
| Time from first TPE to first rituximab dose, d | 12 (4.5-15.5) | 10 (7-13) | .954 |
| Duration of caplacizumab treatment, d | 49 (39.5-59.5) | - | |
| Patient outcomes | |||
| Time from first TPE to final ADAMTS13 activity of ≥10%, d | 42 (31.0-55.0) | 23 (14.5-33.3) | .014 |
| Time from first TPE to final ADAMTS13 activity of ≥20%, d | 52 (33.0-55.0) | 25 (20.5-43.5) | .134 |
| Time after final TPE to final ADAMTS13 activity of ≥10%, d | 35 (11.3-41.3) | 2 (2.0-3.0) | <.001 |
| Time after final TPE to final ADAMTS13 activity of ≥20%, d | 38.5 (17.5-48.5) | 11.0 (3.0-18.8) | .008 |
| Time to achieve initial platelet counts of ≥100 × 109/L, d | 5 (4.0-5.0) | 8.5 (5.8-15) | <.001 |
| Time to achieve initial platelet counts of ≥150 × 109/L, d | 6 (5.0-7.0) | 14.5 (7-22.8) | .002 |
Continuous data are presented as medians (first to third quartiles), whereas categorical data are presented as percentages.