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An Overview of Laboratory Testing for ADAMTS13
Emmanuel J Favaloro1,2, Leonardo Pasalic3,4,5
1School of Medical Sciences, Faculty of Medicine and Health University of Sydney, Westmead Hospital, Westmead, NSW, Australia. Emmanuel.Favaloro@health.nsw.gov.au.
Methods in Molecular Biology (Clifton, N.J.)
|May 19, 2023
Summary
ADAMTS13 deficiency causes von Willebrand factor accumulation, leading to thrombosis in conditions like TTP and COVID-19. Laboratory testing aids diagnosis and management of these ADAMTS13-related disorders.
Area of Science:
- Biochemistry
- Hematology
- Molecular Biology
Background:
- ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) cleaves von Willebrand factor (VWF) multimers, regulating plasma VWF activity.
- Deficiency in ADAMTS13 activity can lead to the accumulation of ultra-large VWF multimers, promoting thrombosis.
- Conditions such as thrombotic thrombocytopenia purpura (TTP) and COVID-19 are associated with reduced ADAMTS13 levels and increased thrombosis risk.
Purpose of the Study:
- To provide an overview of laboratory testing for ADAMTS13.
- To highlight the diagnostic and management value of ADAMTS13 testing in related disorders.
- To discuss the role of ADAMTS13 in TTP, secondary thrombotic microangiopathies (TMA), and COVID-19.
Main Methods:
- Review of current literature on ADAMTS13.
- Discussion of various laboratory assay methodologies for ADAMTS13.
- Analysis of clinical implications of ADAMTS13 testing.
Main Results:
- Laboratory testing for ADAMTS13 is crucial for diagnosing TTP and secondary TMAs.
- Reduced ADAMTS13 activity is implicated in the thrombotic complications of COVID-19.
- Various assays are available for assessing ADAMTS13 activity and levels.
Conclusions:
- ADAMTS13 testing is essential for the diagnosis and management of thrombotic microangiopathies.
- Understanding ADAMTS13 function is critical for managing patients with VWF-related thrombotic disorders.
- Further research into ADAMTS13's role in COVID-19 may reveal new therapeutic strategies.
