Table 3.

Summary of clinical outcomes of historical non-caplacizumab cohort vs caplacizumab cohort

Non-caplacizumab cohort (n = 16),
median (range)
Caplacizumab cohort (n = 17),
median (range)
P value
Days to platelet count normalization 6 (3-29) 4 (2-10) .2 
Days to ADAMTS13 activity ≥10% IU/dL  10.5 (2-929) 5 (1-72) .3 
Days to ADAMTS13 activity ≥20% IU/dL  14 (2-929) 5 (1-72) .1 
Days to ADAMTS13 activity ≥30% IU/dL  18 (3-929) 9 (1-230) .1 
Platelet count at time of caplacizumab discontinuation, × 109/L — 191 (92-467) — 
Platelet count at time of PLEX discontinuation, × 109/L 249 (166-466) 247 (107-375) .7 
ADAMTS13 activity at time of caplacizumab discontinuation, IU/dL — 50 (1-97) — 
ADAMTS13 activity at time of PLEX discontinuation, IU/dL 55 (1-70) 58 (1-97) .6 
Length of stay, d 13 (6-30) 15 (5-25) .8 
ADAMTS13 activity 30 d after PLEX, IU/dL 84 (50-103; n = 12) 70 (1-95; n = 15) .02 
Patients with iTTP exacerbation, n (%) 1 (6) .3 
Patients with iTTP relapse, n (%) 3 (18.8) 5 (29) .3 
Days after PLEX to iTTP relapse 175 (151-203) 234 (102-1053) .79 
Days after caplacizumab to iTTP relapse — 237 (102-1053) — 
Caplacizumab-associated bleeding complications, n (%) — 1 (6) — 
Mortality, n (%) — 
Non-caplacizumab cohort (n = 16),
median (range)
Caplacizumab cohort (n = 17),
median (range)
P value
Days to platelet count normalization 6 (3-29) 4 (2-10) .2 
Days to ADAMTS13 activity ≥10% IU/dL  10.5 (2-929) 5 (1-72) .3 
Days to ADAMTS13 activity ≥20% IU/dL  14 (2-929) 5 (1-72) .1 
Days to ADAMTS13 activity ≥30% IU/dL  18 (3-929) 9 (1-230) .1 
Platelet count at time of caplacizumab discontinuation, × 109/L — 191 (92-467) — 
Platelet count at time of PLEX discontinuation, × 109/L 249 (166-466) 247 (107-375) .7 
ADAMTS13 activity at time of caplacizumab discontinuation, IU/dL — 50 (1-97) — 
ADAMTS13 activity at time of PLEX discontinuation, IU/dL 55 (1-70) 58 (1-97) .6 
Length of stay, d 13 (6-30) 15 (5-25) .8 
ADAMTS13 activity 30 d after PLEX, IU/dL 84 (50-103; n = 12) 70 (1-95; n = 15) .02 
Patients with iTTP exacerbation, n (%) 1 (6) .3 
Patients with iTTP relapse, n (%) 3 (18.8) 5 (29) .3 
Days after PLEX to iTTP relapse 175 (151-203) 234 (102-1053) .79 
Days after caplacizumab to iTTP relapse — 237 (102-1053) — 
Caplacizumab-associated bleeding complications, n (%) — 1 (6) — 
Mortality, n (%) — 

During an acute presentation of iTTP.

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