Fig. 5.

Mean plasma levels of von Willebrand factor ristocetin cofactor (VWF:RCo) in the safety population. Error bars represent standard error of the mean; D2–3 is the daily TPE period, W1–W10 is the post-daily TPE period; VWF:RCo values of < 20% represent the threshold for pharmacologic activity of caplacizumab. The mean VWF:RCo of 29.2% at Week 4 (slightly above the 20% threshold) was caused by an aberrant data point of 279.6% from one participant who stopped caplacizumab due to physician decision, BL baseline, D day, FU follow-up, TPE therapeutic plasma exchange, W week