Unless otherwise noted, data are n (%).

Time to normalization of platelet count was defined in line with the HERCULES study as the time from the first IV administration of caplacizumab to normalization of platelet count (ie, ≥150 × 10⁹/L with discontinuation of PEX within 5 days thereafter). Time to normalization of platelet count is based on 81 of 85 patients; the remaining 4 patients did not achieve normalization of their platelet count by 30 days post-PEX discontinuation (1 case because of concomitant diagnosis of immune thrombocytopenia with a history of chronic low-grade thrombocytopenia, 1 case who achieved a normal platelet count by day 43, and 2 cases with multiple organ failure leading to death).

Based on n = 81 patients, excluding 4 who died prior to discharge from the hospital.

Based on available data for 54 of 55 patients who presented with neurological symptoms. The most common neurological symptoms on discharge were depressed mood and anxiety.

Based on available data for 32 of 35 patients who presented with acute kidney injury.

Recurrence of TTP was defined as a new decrease in platelet count after initial normalization of the platelet count, requiring PEX therapy to be reinitiated. A recurrence within 30 days after completion of PEX therapy was defined as an exacerbation, and a recurrence occurring >30 days after completion of PEX therapy was defined as a relapse.

The cause of death was determined by the hematology clinician submitting the data; in all cases the cause was believed to be secondary to severe and/or refractory TTP.