Overview of ADAMTS13 Protein in Diagnosis and Patient Management of TTP

Abstract

The accurate diagnosis and effective management of Thrombotic Thrombocytopenic Purpura (TTP), a rare but life-threatening hematologic disorder, rely critically on the assessment of ADAMTS13 activity. ADAMTS13, a von Willebrand factor-cleaving protease, plays a pivotal role in the pathophysiology of TTP. This review synthesizes current understanding and recent advancements in the role of ADAMTS13 in the diagnosis and therapeutic monitoring of TTP, with a specific focus on the implementation of a fluorescence resonance energy transfer (FRET)-based assay for ADAMTS13 activity. Our retrospective analysis of 50 suspected TTP cases, including 19 confirmed diagnoses, revealed that the FRET-based assay significantly improved the sensitivity and specificity of TTP diagnosis compared to traditional qualitative methods. Furthermore, the FRET assay facilitated timely initiation of appropriate therapies and monitoring of disease activity, underscoring its utility in clinical practice. Our findings highlight the FRET-based ADAMTS13 activity assay's contribution to enhancing the diagnostic accuracy and management efficiency of TTP, suggesting its potential as a standard diagnostic tool in clinical settings. The review aims to provide a comprehensive overview for hematologists and laboratory medicine practitioners, encouraging the adoption of the FRET-based assay to improve patient outcomes in TTP management.