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Automated and Rapid ADAMTS13 Testing Using Chemiluminescence: Utility for Identification or Exclusion of TTP and Beyond

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Hemostasis and Thrombosis

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

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a prothrombotic condition caused by a significant deficiency of the enzyme, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). In the absence of adequate levels of ADAMTS13 (i.e., in TTP), plasma VWF accumulates, in particular as “ultra-large” VWF multimers, and this leads to pathological platelet aggregation and thrombosis. In addition to TTP, ADAMTS13 may be mildly to moderately reduced in a range of other conditions, including secondary thrombotic microangiopathies (TMA) such as those caused by infections (e.g., hemolytic uremic syndrome (HUS)), liver disease, disseminated intravascular coagulation (DIC), and sepsis, during acute/chronic inflammatory conditions, and sometimes also in COVID-19 (coronavirus disease 2019)). ADAMTS13 can be detected by a variety of techniques, including ELISA (enzyme-linked immunosorbent assay), FRET (fluorescence resonance energy transfer) and by chemiluminescence immunoassay (CLIA). The current report describes a protocol for assessment of ADAMTS13 by CLIA. This protocol reflects a rapid test able to be performed within 35 min on the AcuStar instrument (Werfen/Instrumentation Laboratory), although certain regional approvals may also permit this testing to be performed on a BioFlash instrument from the same manufacturer.

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Abbreviations

ADAMTS13:

a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13

CLIA:

chemiluminescence immunoassay

COVID-19:

coronavirus disease 2019

FRET:

fluorescence resonance energy transfer

TMA:

thrombotic microangiopathies

TTP:

thrombotic thrombocytopenic purpura

VWF:

von Willebrand factor

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Acknowledgments

The authors thank current and past laboratory staff for performance of this assay in the Westmead laboratory. We also thank Werfen/IL for past assistance with CLIA-based assay evaluations performed in our laboratory.

Author information

Authors and Affiliations

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

    Soma Mohammed, Ronny Vong & 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, NSW, Australia

    Leonardo Pasalic

  5. Haematology Department, John Hunter Hospital, NSW Health Pathology, Newcastle, NSW, Australia

    Kent Chapman

  6. Westmead Clinical School, Sydney University, Westmead, NSW, Australia

    Leonardo Pasalic

Authors
  1. Emmanuel J. Favaloro
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  2. Kent Chapman
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  3. Soma Mohammed
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  4. Ronny Vong
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  5. 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., Chapman, K., Mohammed, S., Vong, R., Pasalic, L. (2023). Automated and Rapid ADAMTS13 Testing Using Chemiluminescence: Utility for Identification or Exclusion of TTP and Beyond. 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_32

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  • DOI: https://doi.org/10.1007/978-1-0716-3175-1_32

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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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