The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.

Blood
Camila Masias, Spero R Cataland

Abstract

ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13) is a metalloprotease responsible for cleavage of ultra-large von Willebrand factor (VWF) multimers. Severely deficient activity of the protease can trigger an acute episode of thrombotic thrombocytopenic purpura (TTP). Our understanding of the pathophysiology of TTP has allowed us to grasp the important role of ADAMTS13 in other thrombotic microangiopathies (TMAs) and thrombotic disorders, such as ischemic stroke and coronary artery disease. Through its action on VWF, ADAMTS13 can have prothrombotic and proinflammatory properties, not only when its activity is severely deficient, but also when it is only moderately low. Here, we will discuss the biology of ADAMTS13 and the different assays developed to evaluate its function in the context of TTP, in the acute setting and during follow-up. We will also discuss the latest evidence regarding the role of ADAMTS13 in other TMAs, stroke, and cardiovascular disease. This information will be useful for clinicians not only when evaluating patients who present with microangiopathic hemolytic anemia and thrombocytopenia, but also when making clinical decisions regarding the follow-up of patients with TTP.

References

Nov 26, 1998·The New England Journal of Medicine·H M Tsai, E C Lian
Dec 16, 2003·Biochemical and Biophysical Research Communications·Misako SuzukiYasuo Ikeda
Apr 2, 2005·British Journal of Haematology·Koichi KokameToshiyuki Miyata
Apr 28, 2005·Blood·Masahito UemuraYoshihiro Fujimura
Aug 17, 2005·Journal of Thrombosis and Haemostasis : JTH·J-F Dong
Sep 24, 2005·Journal of Thrombosis and Haemostasis : JTH·L LiuJ-F Dong
Jan 3, 2006·Seminars in Thrombosis and Hemostasis·Suresh G ShelatX Long Zheng
May 19, 2006·Journal of Thrombosis and Haemostasis : JTH·N TurnerJ Moake
Mar 12, 2008·Hypertension·Bert-Jan H van den BornGert A van Montfrans
Apr 10, 2008·British Journal of Haematology·Ming JinHaifeng M Wu
Aug 1, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Minola ManeaDiana Karpman
Aug 18, 2009·Journal of Thrombosis and Haemostasis : JTH·S FerrariF Scheiflinger
Sep 22, 2009·Journal of Thrombosis and Haemostasis : JTH·H B FeysJ E Sadler
Jul 8, 2010·The Journal of Biological Chemistry·Christopher G SkipwithX Long Zheng
Oct 29, 2010·American Journal of Hematology·Spero R CatalandHaifeng M Wu
Jul 17, 2013·Journal of Thrombosis and Haemostasis : JTH·X L Zheng
May 30, 2014·Blood·Miguel HieUNKNOWN French Thrombotic Microangiopathies Reference Centre

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Citations

Feb 25, 2020·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·Zhenzhen XuJianyu Weng
Jul 17, 2020·International Journal of Laboratory Hematology·Ian MackieRoss Baker
May 3, 2019·The New England Journal of Medicine·Marie ScullyFilip Callewaert
Sep 4, 2020·TH Open : Companion Journal to Thrombosis and Haemostasis·Giovanni L TisciaUNKNOWN CSS COVID-19 Group
Oct 24, 2020·International Journal of Laboratory Hematology·Barbara MontaruliDomenico Cosseddu
Jan 7, 2021·Annals of Hematology·Valéria Sutana LadeiraDanyelle Romana Alves Rios
Feb 3, 2021·Haemophilia : the Official Journal of the World Federation of Hemophilia·Filippo MoriClaudio Farina
Feb 6, 2021·Journal of Clinical Medicine·Senthil SukumarSpero R Cataland
May 1, 2021·International Journal of Molecular Sciences·Shengshi HuangBas de Laat
Sep 4, 2021·PLoS Neglected Tropical Diseases·Camila Martos ThomaziniMarcelo Larami Santoro
Nov 9, 2021·Expert Review of Hematology·Katerina PavenskiChristopher J Patriquin

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