Clinical laboratory diagnostics and monitoring of ADAMTS13 deficiency and of related diseases: an integrated approach

INTRODUCTION ADAMTS13, a crucial enzyme in hemostasis, that specifically cleaves von Willebrand factor (VWF), plays a pivotal role in the pathogenesis and diagnosis of thrombotic thrombocytopenic purpura (TTP), a microcirculatory form of thrombotic microangiopathy (TMA). Its deficiency leads to the accumulation of ultra-large VWF multimers, causing microvascular thrombosis. Understanding and measuring ADAMTS13 activity are essential for diagnosis and management of TTP and other thrombotic diseases associated with ADAMTS13 deficiency.AREAS COVERED This review explores the evolution of ADAMTS13 assays from labor-intensive methods to rapid, automated platforms. It examines congenital and acquired ADAMTS13 deficiencies, highlighting diagnostic strategies and therapeutic interventions. A comprehensive literature search was conducted between September 2025 and June 2026 using PubMed and GoogleScholar, focusing on recent advancements and extending to foundational studies when necessary.EXPERT OPINION The integration of rapid ADAMTS13 testing has the potential to improve clinical decision-making by shortening diagnostic uncertainty and supporting earlier differentiation of TTP from other TMAs. Despite progress, challenges remain in assay standardization, sensitivity, and interpretation. Future developments may include point-of-care technologies and conformation-sensitive assays to enhance relapse prediction and monitoring. Collaboration between clinicians and laboratories is crucial to optimize patient outcomes and advance the field of ADAMTS13 diagnostics.