{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:59:00Z","timestamp":1787065140054,"version":"build-2736575974"},"reference-count":22,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Clin Appl Thromb Hemost"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:p>The introduction Caplacizumab in the management of Immune thrombotic thrombocytopenic purpura (iTTP) has raised different questions, considering its cost-efficacy and the optimal immunosuppressive treatment (IST) to associate. A retrospective multicenter collection of 42 first iTTP cases was conducted to identify variables associated with a higher burden of care and necessity of an implemented IST with early Rituximab (RTX) rescue. A significant correlation resulted between ADAMTS13 inhibitors (ADAMTS13inh) at diagnosis with total plasma exchange (PEXtot) and PEX needed to achieve clinical response (PEXtoCR, r\u2009=\u20090.46; r\u2009=\u20090.48), along with age (r\u2009=\u2009- 0.31; r\u2009=\u2009-0.35), platelet count (r\u2009=\u2009-0.30; r\u2009=\u2009-0.30), LDH (r\u2009=\u20090.44; r\u2009=\u20090.41) and total bilirubin (r\u2009=\u20090.54; r\u2009=\u20090.35). ADAMTS13inh also correlated with number of days of hospitalization (DoH, r\u2009=\u20090.44). A significant difference was observed in terms of median ADAMTS13inh titer at diagnosis in patient treated with RTX rescue and those responding to only steroid treatment. Thus, ADAMTS13inh titer resulted a marker of iTTP burden of care, associated with higher number of PEXtot, PEXtoCR, DoH and higher probability of needing RTX rescue to achieve clinical response and could be a useful tool for management of new iTTP cases and an interesting variable to optimize iTTP cases stratification in future Caplacizumab cost-efficacy analysis.<\/jats:p>","DOI":"10.1177\/10760296221125785","type":"journal-article","created":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T02:17:43Z","timestamp":1663640263000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["ADAMTS13 Autoantibodies and Burden of Care in Immune Thrombotic Thrombocytopenic purpura: New Evidence and Future Implications"],"prefix":"10.1177","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8672-1372","authenticated-orcid":false,"given":"Cristina","family":"Dainese","sequence":"first","affiliation":[{"name":"Regional Reference Center for Thrombotic and Haemorrhagic disorders of the adult, Department of Hematology and Oncology, Azienda Ospedaliera Citt\u00e0 della Salute e della Scienza di Torino - Molinette, 10126, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Valeri","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Thrombotic and Haemorrhagic disorders of the adult, Department of Hematology and Oncology, Azienda Ospedaliera Citt\u00e0 della Salute e della Scienza di Torino - Molinette, 10126, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Pizzo","sequence":"additional","affiliation":[{"name":"School of Medicine, Study University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Valpreda","sequence":"additional","affiliation":[{"name":"School of Medicine, Study University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piera","family":"Sivera","sequence":"additional","affiliation":[{"name":"Hematology Unit, Ordine Mauriziano Hospital, Turin, 10128, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Montaruli","sequence":"additional","affiliation":[{"name":"Laboratory Analysis, Ordine Mauriziano Hospital, 10128, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Porreca","sequence":"additional","affiliation":[{"name":"Department of Medical, Oral and Biotechnologies Sciences, University of Chieti-Pescara, 66100, Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Massaia","sequence":"additional","affiliation":[{"name":"Division of Hematology, \u201cSanta Croce e Carle di Cuneo\u201d Hospital, 12100, Cuneo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedetto","family":"Bruno","sequence":"additional","affiliation":[{"name":"Hematology Unit, Department of Hematology and Oncology, Azienda Ospedaliera Citt\u00e0 della Salute e della Scienza di Torino - Molinette, 10126, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Borchiellini","sequence":"additional","affiliation":[{"name":"Regional Reference Center for Thrombotic and Haemorrhagic disorders of the adult, Department of Hematology and Oncology, Azienda Ospedaliera Citt\u00e0 della Salute e della Scienza di Torino - Molinette, 10126, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2022,9,19]]},"reference":[{"key":"bibr1-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006052"},{"key":"bibr2-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15194"},{"key":"bibr3-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1016\/S2352-3026(17)30026-1"},{"key":"bibr4-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0010208"},{"key":"bibr5-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1016\/S0268-960X(05)80019-0"},{"key":"bibr6-10760296221125785","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2011.049676"},{"key":"bibr7-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05857.x"},{"key":"bibr8-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/trf.13586"},{"key":"bibr9-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9681(87)90171-8"},{"key":"bibr10-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/jth.13571"},{"key":"bibr11-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15010"},{"key":"bibr12-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15006"},{"key":"bibr13-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-1679907"},{"key":"bibr14-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07276.x"},{"key":"bibr15-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-011-0771-5"},{"key":"bibr16-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/imj.12935"},{"key":"bibr17-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2017005124"},{"key":"bibr18-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1505533"},{"key":"bibr19-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1806311"},{"key":"bibr20-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-11-4035"},{"key":"bibr21-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-12-758656"},{"key":"bibr22-10760296221125785","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05837.x"}],"container-title":["Clinical and Applied Thrombosis\/Hemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10760296221125785","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/10760296221125785","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/10760296221125785","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T14:03:33Z","timestamp":1777385013000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/10760296221125785"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1]]},"references-count":22,"alternative-id":["10.1177\/10760296221125785"],"URL":"https:\/\/doi.org\/10.1177\/10760296221125785","relation":{},"ISSN":["1076-0296","1938-2723"],"issn-type":[{"value":"1076-0296","type":"print"},{"value":"1938-2723","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1]]},"article-number":"10760296221125785"}}