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An Overview of Laboratory Testing for ADAMTS13

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Hemostasis and Thrombosis

Part of the book series: Methods in Molecular Biology ((MIMB,volume 2663))

Abstract

ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) is also called von Willebrand factor (VWF) cleaving protease (VWFCP). ADAMTS13 acts to cleave VWF multimers and thus reduce plasma VWF activity. In the absence of ADAMTS13 (i.e., in thrombotic thrombocytopenia purpura, TTP), plasma VWF can accumulate, in particular as “ultra-large” VWF multimers, and this can lead to thrombosis. Relative deficiencies in ADAMTS13 can also occur in a variety of other conditions, including secondary thrombotic microangiopathies (TMA). Of contemporary interest, COVID-19 (coronavirus disease 2019) may also be associated with relative reduction of ADAMTS13 and also pathological accumulation of VWF, with this likely contributing to the thrombosis risk seen in affected patients. Laboratory testing for ADAMTS13 can assist in the diagnosis of these disorders (i.e., TTP, TMA), as well as in their management, and can be achieved using a variety of assays. This chapter therefore provides an overview of laboratory testing for ADAMTS13 and the value of such testing to assist the diagnosis and management of associated disorders.

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Author information

Authors and Affiliations

  1. Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia

    Leonardo Pasalic

  2. School of Medical Sciences, Faculty of Medicine and Health University of Sydney, Westmead Hospital, Westmead, NSW, Australia

    Emmanuel J. Favaloro

  3. School of Dentistry and Medical Sciences, Faculty of Science and Health, Charles Sturt University, Wagga, Wagga, NSW, Australia

    Emmanuel J. Favaloro

  4. Sydney Centres for Thrombosis and Haemostasis, Westmead Hospital, Westmead, NSW, Australia

    Leonardo Pasalic

  5. Westmead Clinical School, University of Sydney, Westmead Hospital, Westmead, NSW, Australia

    Leonardo Pasalic

Authors
  1. Emmanuel J. Favaloro
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  2. Leonardo Pasalic
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Corresponding author

Correspondence to Emmanuel J. Favaloro .

Editor information

Editors and Affiliations

  1. School of Medical Sciences, Faculty of Medicine and Health University of Sydney, Westmead Hospital, Westmead, NSW, Australia

    Emmanuel J. Favaloro

  2. Hemostasis & Thrombosis Center, University of California, Davis, Sacramento, CA, USA

    Robert C. Gosselin

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© 2023 The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature

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Favaloro, E.J., Pasalic, L. (2023). An Overview of Laboratory Testing for ADAMTS13. In: Favaloro, E.J., Gosselin, R.C. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 2663. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-3175-1_31

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  • DOI: https://doi.org/10.1007/978-1-0716-3175-1_31

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  • Publisher Name: Humana, New York, NY

  • Print ISBN: 978-1-0716-3174-4

  • Online ISBN: 978-1-0716-3175-1

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