Figure 1.
Patient cohorts for analysis. The primary outcome analyzed in the USTMA retrospective registry cohort (2004-2019) (A) was clinical relapse (CR) defined as a decrease in platelets of ≤150 × 109/L with or without clinical evidence of ischemic organ injury, confirmed by documentation of severe ADAMTS13 deficiency and occurring after having achieved clinical remission.19 The prospective Johns Hopkins and University of Minnesota cohort (2004-2023) (B) also included data on serial ADAMTS13 assessments and the primary outcome analyzed in this cohort was overall relapse defined as a composite end point of CR, ADAMTS13 relapse, and preemptive rituximab (RTX) therapy. OR, overall relapse.