{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T04:28:57Z","timestamp":1790051337572,"version":"4.0.1"},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,5,11]],"date-time":"2013-05-11T00:00:00Z","timestamp":1368230400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Vox Sanguinis"],"published-print":{"date-parts":[[2013,11]]},"abstract":"<jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>Idiopathic thrombotic thrombocytopenic purpura (<jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content>) is a rare, clinically diagnosed disorder characterized by widespread intravascular platelet thrombosis. The pathophysiology involves acquired deficiency of <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 (A disintegrin and metalloprotease with thrombospondin type 1 repeats), the enzyme responsible for cleavage of high molecular weight von<jats:styled-content style=\"fixed-case\">W<\/jats:styled-content>illebrand factor multimers. Disease mortality is high, although prompt treatment with plasma exchange is generally effective. A readily available and highly reliable method of identifying <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13\u2010deficient patients for appropriate plasma exchange is therefore of interest.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods<\/jats:title><jats:p>Our initial study involved the assessment of multiple clinical and laboratory variables in patients with clinically suspected <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> for whom <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 assay was performed. Five variables were found to be of significant predictive power. This enabled the development of a point\u2010based scoring system to efficiently determine the likelihood of <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> and response to plasma exchange in a given patient. This current study involved a separate validation cohort of patients with clinically suspected <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> who underwent <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 testing within two large healthcare systems in <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>tah between 2009 and 2011. The previously derived score was applied to this cohort and its performance was analysed. Additionally, the original and validation cohorts were combined to revisit the predictive power of individual variables and the five\u2010variable prediction score.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 84 (11 paediatric cases excluded) patients comprised the validation population. The percentage of <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> diagnoses in this group (10%) was identical to that in the initial cohort. Using an <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 activity of &lt;10% of normal, our original score correctly predicted or excluded severe <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 deficiency in all patients in the second cohort when data for all variables was available. Individual variables retained predictive power and the performance of a three\u2010variable parsimonious model, as well as the ultimate diagnoses for patients in the second cohort are described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This work confirms the predictive power of a simple point\u2010based score to exclude <jats:styled-content style=\"fixed-case\">TTP<\/jats:styled-content> as evidenced by severe <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 deficiency in appropriately selected patients. It may enable clinicians to rapidly begin plasma exchange or to pursue an alternative cause of thrombotic microangiopathy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/vox.12050","type":"journal-article","created":{"date-parts":[[2013,5,11]],"date-time":"2013-05-11T09:02:50Z","timestamp":1368262970000},"page":"313-318","source":"Crossref","is-referenced-by-count":37,"title":["Performance of a clinical prediction score for thrombotic thrombocytopenic purpura in an independent cohort"],"prefix":"10.1111","volume":"105","author":[{"given":"M. J.","family":"Bentley","sequence":"first","affiliation":[{"name":"Internal Medicine University of Utah Salt Lake City UT USA"},{"name":"Ogden Internal Medicine Ogden UT USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. R.","family":"Wilson","sequence":"additional","affiliation":[{"name":"Statistics ARUP Laboratories Salt Lake City UT USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. M.","family":"Rodgers","sequence":"additional","affiliation":[{"name":"Internal Medicine and Pathology University of Utah Health Sciences Center Salt Lake City UT USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,5,11]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra020528"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2007.06.003"},{"key":"e_1_2_6_4_1","first-page":"21","article-title":"Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease","volume":"24","author":"Moschcowitz E","year":"1924","journal-title":"Proc NY Pathol Soc"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.3949\/ccjm.75.5.369"},{"key":"e_1_2_6_6_1","first-page":"178","article-title":"The treatment of thrombotic thrombocytopenic purpura with exchange transfusions, plasma infusions, and plasma exchange","volume":"24","author":"Shephard KV","year":"1987","journal-title":"Semin Hematol"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(99)00286-7"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/jca.20134"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-02-078170"},{"key":"e_1_2_6_10_1","doi-asserted-by":"crossref","first-page":"4235","DOI":"10.1182\/blood.V87.10.4235.bloodjournal87104235","article-title":"Physiologic cleavage of vonWillebrand factor by a plasma protease is dependent on its conformation and requires calcium ion","volume":"87","author":"Tsai HM","year":"1996","journal-title":"Blood"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.10.4223.bloodjournal87104223"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198212023072306"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2010.02653.x"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1309\/LM95OQN0GLZPYHBP"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01767.x"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613830"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1159\/000180580"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-130-6-199903160-00002"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)61145-8"},{"key":"e_1_2_6_20_1","article-title":"Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura (Review)","author":"Michael M","year":"2009","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_6_21_1","first-page":"5","article-title":"Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome","volume":"3","author":"Ohanian M","year":"2011","journal-title":"Clin Pharmacol"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e3181ccd80d"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.01090209"}],"container-title":["Vox Sanguinis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fvox.12050","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/vox.12050","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T17:43:36Z","timestamp":1696009416000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/vox.12050"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,5,11]]},"references-count":22,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2013,11]]}},"alternative-id":["10.1111\/vox.12050"],"URL":"https:\/\/doi.org\/10.1111\/vox.12050","archive":["Portico"],"relation":{},"ISSN":["0042-9007","1423-0410"],"issn-type":[{"value":"0042-9007","type":"print"},{"value":"1423-0410","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,5,11]]}}}