{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T04:46:09Z","timestamp":1784263569246,"version":"3.55.0"},"reference-count":0,"publisher":"Hematology Section, Dept. of Radiological Science and Hematology, Catholic University, Rome, Italy","issue":"1","license":[{"start":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T00:00:00Z","timestamp":1772236800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Mediterr J Hematol Infect Dis"],"abstract":"<jats:p>Background: In the ICU, distinguishing immune\u2011mediated thrombotic thrombocytopenic purpura (iTTP) from sepsis\u2011associated thrombotic microangiopathy (TMA) is time\u2011critical. We tested whether serial ADAMTS\u201113 combined with targeted coagulation and inflammation markers improves iTTP risk stratification in a Sepsis\u20113 ICU cohort and whether a pragmatic rule\u2011out is feasible.\r\nMethods: Prospective single\u2011center study of adults meeting Sepsis\u20113 with thrombocytopenia and schistocytes \u2265\u202f1% or LDH\u202f&gt;\u202f2\u00d7\u202fULN within 24\u202fh of ICU admission. ADAMTS\u201113 activity and VWF: Ag were assayed at 0\/24\/48\/72\u202fh alongside a thrombo\u2011inflammatory panel. We derived a Dynamic ADAMTS\u201113 Index (DAI), a Coagulation Consumption Index (CCI) anchored to ISTH DIC and fibrinogen\/antithrombin III, and an Inflammation Index (IL\u20116\/HBP). The prespecified main rule\u2011out required a \u2265\u202f15% ADAMTS\u201113 rise by 48\u202fh plus low CCI. A prespecified RCV\u2011anchored sensitivity analysis required \u2265\u202f35% relative rise or \u2265\u202f10 absolute %-points plus low consumption. For decision\u2011making, pre\u2011treatment (pre\u2011plasma exchange, PEx) analyses are emphasized. Intent\u2011to\u2011diagnose (care\u2011embedded) analyses are exploratory, and internal validation used bootstrap optimism correction.\r\nResults: Of 1,274 screened, 330 were included (iTTP\u202f=\u202f34). Discrimination improved from baseline ADAMTS\u201113 (AUROC 0.78) to DAI (0.93), with smaller gains after adding CCI (0.95) and the Inflammation Index (0.96). With the main rule\u2011out (\u2265\u202f15% + low CCI) in the intent\u2011to\u2011diagnose analysis, sensitivity was 97.1%, specificity was 86.1%, and NPV was 99.6%. the RCV\u2011anchored sensitivity analysis preserved 100.0% sensitivity and NPV with 76.0% specificity. A 72\u2011h phenotype (ADAMTS\u201113\u202f&lt;\u202f10% with high IL\u20116\/HBP) was associated with higher 28\u2011day mortality (adjusted HR\u202f2.6).\r\nConclusions: In Sepsis\u20113 ICU patients with TMA features, serial ADAMTS\u201113 plus targeted coagulation\/inflammation markers enhance early iTTP risk stratification and supports a pragmatic rule\u2011out framework. The \u2265\u202f15% + low CCI rule\u2011out achieved high sensitivity and NPV, while an RCV\u2011anchored threshold ensured 100% sensitivity\/NPV at reduced specificity. External validation and implementation studies remain essential.<\/jats:p>","DOI":"10.4084\/mjhid.2026.019","type":"journal-article","created":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T07:38:46Z","timestamp":1772350726000},"page":"e2026019","source":"Crossref","is-referenced-by-count":1,"title":["DYNAMIC MONITORING OF ADAMTS 13 ACTIVITY FOR DIFFERENTIAL DIAGNOSIS ACROSS THE SPECTRUM OF SEPSIS ASSOCIATED WITH THROMBOTIC MICROANGIOPATIES"],"prefix":"10.4084","volume":"18","author":[{"given":"Yi","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fan","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Binbin","family":"Ji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingjie","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bining","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2554","published-online":{"date-parts":[[2026,2,28]]},"container-title":["Mediterranean Journal of Hematology and Infectious Diseases"],"original-title":[],"link":[{"URL":"https:\/\/www.mjhid.org\/mjhid\/article\/download\/6170\/4825","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.mjhid.org\/mjhid\/article\/download\/6170\/4832","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.mjhid.org\/mjhid\/article\/download\/6170\/4825","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,2]],"date-time":"2026-03-02T07:23:17Z","timestamp":1772436197000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mjhid.org\/mjhid\/article\/view\/6170"}},"subtitle":["Adamts 13 and Sepsis Associated with Thrombotic Thrombocytopenic Purpura"],"short-title":[],"issued":{"date-parts":[[2026,2,28]]},"references-count":0,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1,1]]}},"URL":"https:\/\/doi.org\/10.4084\/mjhid.2026.019","relation":{},"ISSN":["2035-3006"],"issn-type":[{"value":"2035-3006","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,28]]}}}