Combined Elevated Anti-Adamts-13 Antibody Level and Low Adamts-13 Activity during Remission Highly Predicts Disease Relapse in a Prospective Cohort of Patients with Autoimmune Thrombotic Thrombocytopenic Purpura (TTP)

Introduction Autoimmune TTP (aTTP) is a rare life-threatening microangiopathy characterized by hemolytic anemia, thrombocytopenia, and severe ADAMTS-13 deficiency. Daily therapeutic plasma exchange (TPE) and preemptive use of rituximab have considerably improved patient outcome. However, a high proportion of patients still experience relapse and several studies are evaluating the role of ADAMTS-13 activity and its inhibitors to predict disease...