Guided precision management of iTTP: optimizing treatment, monitoring, and relapse prevention

INTRODUCTION: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is caused by severe deficiency of plasma ADAMTS13 (A Disintegrin And Metalloprotease with Thrombospondin type 1 repeats, 13) activity resulting from acquired autoantibodies against ADAMTS13. Significant progress has been made in recent decades in our understandings of pathogenesis, diagnosis, and management of iTTP. AREAS COVERED: This review summarizes the key literatures available in PubMed (1925-2026) concerning the diagnosis and management of iTTP, particularly focusing on ADAMTS13-guided therapeutic strategy for prevention of the disease exacerbation or relapse. EXPERT OPINION: The management of iTTP is moving from a uniform protocol toward guided precision strategies based on dynamic assessment of the disease activity and individual relapse risk. ADAMTS13 monitoring should serve as a central component of short- and long-term cares, enabling timely modification of therapy in acute setting and preemptive intervention before clinical relapse occurs. Multicenter and prospective studies are needed to define the best therapeutic targets, the monitoring intervals, and the intervention thresholds. Integration of the rapid diagnostics, monitoring, and mechanism-based therapies may further reduce mortality, prevent relapse, minimize cumulative toxicity, and improve the long-term outcomes.