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Version 1
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The Highs and Lows of ADAMTS13 Activity
Version 1
: Received: 25 June 2024 / Approved: 26 June 2024 / Online: 27 June 2024 (06:10:38 CEST)
How to cite: Shaw, R. J.; Abrams, S. T.; Badu, S.; Toh, C.-H.; Dutt, T. The Highs and Lows of ADAMTS13 Activity. Preprints 2024, 2024061832. https://doi.org/10.20944/preprints202406.1832.v1 Shaw, R. J.; Abrams, S. T.; Badu, S.; Toh, C.-H.; Dutt, T. The Highs and Lows of ADAMTS13 Activity. Preprints 2024, 2024061832. https://doi.org/10.20944/preprints202406.1832.v1
Abstract
Severe deficiency of ADAMTS13 (<10 iu/dL) is diagnostic of thrombotic thrombocytopenic purpura (TTP) and leads to accumulation of ultra-large VWF multimers, platelet aggregation and widespread microthrombi, which can be life-threatening. However, the clinical implications of a low ADAMTS13 activity level are not only important in an acute episode of TTP. In this article, we discuss the effects of a low ADAMTS13 activity in congenital and immune-mediated TTP patients not only at presentation, but once in a clinical remission. Evidence is emerging of the clinical effects of a low ADAMTS13 activity in other disease areas outside of TTP and here we explore the wider impact of low ADAMTS13 activity on the vascular endothelium and the potential for recombinant ADAMTS13 therapy in other thrombotic disease states.
Keywords
ADAMTS13; TTP; VWF
Subject
Medicine and Pharmacology, Hematology
Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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