{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T01:08:24Z","timestamp":1790644104658,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2020,5,21]],"date-time":"2020-05-21T00:00:00Z","timestamp":1590019200000},"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":["American J Hematol"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Immune\u2010mediated thrombotic thrombocytopenic purpura (iTTP) is a life\u2010threatening immune\u2010mediated thrombotic microangiopathy. Daily therapeutic plasma exchange (TPE) and the optimized use of rituximab have strikingly improved the outcome of this disease, however the rate of disease recurrence remains high. Specific predictors of relapse in patients in remission can be relevant for an optimal patient management. In this study, we aimed to identify predictive variables of disease relapse in a multicenter cohort of 74 out of 153 iTTP patients. They were tested at different time points during remission for the levels of ADAMTS\u201013 activity and autoantibody, and did not receive pre\u2010emptive treatment for ADAMTS\u201013 activity deficiency during remission. The results showed that the association of ADAMTS13 activity \u226420% with a high anti\u2010ADAMTS\u201013 titer at remission, and the time to response to first line treatment \u226513\u2009days, were independent predictive factors of disease relapse. In addition, the use of rituximab in patients with exacerbation or refractoriness to TPE was significantly associated with reduced relapse rate. By Cox regression analysis, patients with ADAMTS\u201013 activity \u226420% plus anti\u2010ADAMTS13 antibody titer \u226515\u2009U\/mL at remission had an increased risk of relapse (HR 1.98, CI 95% 1.087\u20103.614;\n                    <jats:italic>P<\/jats:italic>\n                    &lt;\u2009.02). These findings may help to outline more personalized therapeutic strategies in order to provide faster and sustained responses to first\u2010line iTTP treatment and prevent relapses in these patients.\n                  <\/jats:p>","DOI":"10.1002\/ajh.25845","type":"journal-article","created":{"date-parts":[[2020,4,30]],"date-time":"2020-04-30T02:42:36Z","timestamp":1588214556000},"page":"953-959","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Low levels of\n                    <scp>ADAMTS<\/scp>\n                    \u201013 with high\n                    <scp>anti\u2010ADAMTS<\/scp>\n                    \u201013 antibodies during remission of immune\u2010mediated thrombotic thrombocytopenic purpura highly predict for disease relapse: A multi\u2010institutional study"],"prefix":"10.1002","volume":"95","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0338-2000","authenticated-orcid":false,"given":"Francesca","family":"Schieppati","sequence":"first","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Russo","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Marchetti","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Barcella","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Cefis","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Gomez\u2010Rosas","sequence":"additional","affiliation":[{"name":"Hospital General Regional Tecamac, IMSS  Esdato de Mexico Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gloria","family":"Caldara","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4355-3954","authenticated-orcid":false,"given":"Monica","family":"Carpenedo","sequence":"additional","affiliation":[{"name":"Hematology Hospital San Gerardo  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariella","family":"D'Adda","sequence":"additional","affiliation":[{"name":"Hematology Hospital Spedali Civili  Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Rambaldi","sequence":"additional","affiliation":[{"name":"Hematology Hospital Papa Giovanni XXIII  Bergamo Italy"},{"name":"School of Medicine University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Savignano","sequence":"additional","affiliation":[{"name":"Transfusion Medicine University Hospital  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atto","family":"Billio","sequence":"additional","affiliation":[{"name":"Hematology San Maurizio Regional Hospital  Bolzano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimiliano","family":"Bruno Franco","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine ASL 1  Imperia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Toschi","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital San Carlo Borromeo  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5007-3457","authenticated-orcid":false,"given":"Anna","family":"Falanga","sequence":"additional","affiliation":[{"name":"Immunohematology and Transfusion Medicine Hospital Papa Giovanni XXIII  Bergamo Italy"},{"name":"School of Medicine University of Milano Bicocca  Milan 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