Rapid automated ADAMTS13 assays streamline TTP diagnosis and monitoring
The review examines the evolution of ADAMTS13 testing from labor-intensive methods to rapid, automated platforms for diagnosing thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. Investigators note that integrating fast ADAMTS13 assays reduces diagnostic uncertainty, supports earlier differentiation from other TMAs, and facilitates better therapeutic decision-making. Despite these advances, the authors highlight persistent challenges in assay standardization, sensitivity, and result interpretation. Future developments, including point-of-care formats and conformation-sensitive assays, are expected to improve relapse prediction and long-term monitoring, underscoring the need for closer clinician and laboratory collaboration.
The original study
Clinical laboratory diagnostics and monitoring of ADAMTS13 deficiency and of related diseases: an integrated approach.
- Authors
- Binder NB, Depasse F, Spannagl M, Patzke J, Schwers S, Turecek PL
- Journal
- Expert review of hematology
- Type
- Journal Article, Review
- PMID
- 42461718
Original abstract
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.