{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T17:18:36Z","timestamp":1767374316526},"reference-count":32,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,9,20]],"date-time":"2013-09-20T00:00:00Z","timestamp":1379635200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2013,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The clinical presentation of thrombotic thrombocytopenia purpura (<jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>) and other thrombotic microangiopathies (<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>s) can often be similar. The role of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (<jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13) in diagnosing <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> is accepted by most researchers but continues to be debated in a few studies. We report the experience of our single\u2010centre academic institution, where <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 is used to diagnose <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> and guide plasma exchange (<jats:styled-content style=\"fixed-case\">PLEX<\/jats:styled-content>). Patients presenting to our institution with thrombotic microangiopathy (60 patients) between January 2006 and December 2012 were divided into two groups based on <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 activity and clinical history. Patients with <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 activity &lt;10% were included in the <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a030) cohort while patients with activity &gt;11% were classified as \u2018other microangiopathies\u2019 (<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>,<jats:italic> n<\/jats:italic>\u00a0=\u00a030). <jats:styled-content style=\"fixed-case\">PLEX<\/jats:styled-content> was only initiated in patients with a high likelihood of <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> and discontinued when the baseline <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 activity was &gt;11%. Patients with severe <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 deficiency (<jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> group) showed significant presenting differences: lower platelet counts, less renal dysfunction, higher presence of neurological abnormalities, and greater haemolysis markers as compared to non\u2010deficient patients (<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> group). Most importantly, patients without severe <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 deficiency were safely managed without increased mortality despite receiving no <jats:styled-content style=\"fixed-case\">PLEX<\/jats:styled-content> or discontinuing <jats:styled-content style=\"fixed-case\">PLEX<\/jats:styled-content> after a short course (upon availability of <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 results). In conclusion, <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 can be used to diagnose <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> and guide appropriate <jats:styled-content style=\"fixed-case\">PLEX<\/jats:styled-content> therapy.<\/jats:p>","DOI":"10.1111\/bjh.12569","type":"journal-article","created":{"date-parts":[[2013,10,11]],"date-time":"2013-10-11T22:00:10Z","timestamp":1381528810000},"page":"514-519","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["Role of <scp>ADAMTS<\/scp>13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (<scp>TTP<\/scp>)"],"prefix":"10.1111","volume":"163","author":[{"given":"Neil","family":"Shah","sequence":"first","affiliation":[{"name":"Department of Pathology Division of Transfusion Medicine and Hemostasis Dallas TX 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