Flowchart diagram of the study. ∗One patient died of a CNS thrombosis 30 days after iTTP diagnosis, after having achieved clinical remission with PEX, CS, and RTX. ^†There were 2 deaths: 1 male patient died 10 days after diagnosis due to iTTP evolution, no information about ADAMTS13 levels other than those reported at diagnosis were available, and thus he was considered only for statistics regarding baseline clinical features, treatment response, and remission; 1 female patient died >3 months after diagnosis (cause not reported) and was enrolled for the whole statistical study. ^‡There were 3 deaths: 2 male patients died in the first 48 hours after diagnosis due to iTTP evolution and were considered only for baseline statistics and calculations of rates of response, remission, and death; 2 male patient died >4 months after diagnosis due to iTTP evolution and was enrolled for the whole statistical study. ^§Relapse is clinical relapse according to the revised definitions of Cuker et al.¹⁵ Capla, caplacizumab; CS, corticosteroids; CNS, central nervous system; RTX, rituximab.