Occurrences of relapses among 18 surviving patients in the Oklahoma TTP-HUS Registry who had severe ADAMTS13 deficiency documented at the time of their initial diagnosis.

Patients are presented consecutively, from patient 1 diagnosed in December 1995 to patient 18 diagnosed in December 2003. During this time 4 patients with severe ADAMTS13 deficiency died during their first episode, before achieving remission and are not presented in this figure. Follow-up is complete to the present time on all patients. Patient 3 died of acute myocardial infarction during her first relapse. Patient 7 had a splenectomy after her fourth relapse. Horizontal bars indicate the duration of follow-up; vertical bars indicate the occurrence of a relapse. Median follow-up duration is 3.3 years; one year of follow-up is indicated by the vertical line.