{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:25:59Z","timestamp":1787063159893,"version":"build-2736575974"},"reference-count":38,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2025,7,15]],"date-time":"2025-07-15T00:00:00Z","timestamp":1752537600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Current triplet regimens associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab have dramatically improved the outcome of immune\u2010mediated thrombotic thrombocytopenic purpura (iTTP). However, nearly half of the patients require extended caplacizumab treatment (i.e., &gt;\u200930\u2009days) due to persistent ADAMTS13 deficiency, raising cost and tolerance concerns. Therefore, we investigated whether anti\u2010ADAMTS13 antibodies titer and their trajectory during the acute phase of the disease could predict ADAMTS13 improvement (i.e., activity \u2265\u200920% before day\u201030 post\u2010TPE). From a cohort of 286 patients receiving the triplet regimen, we identified on diagnosis a cut\u2010off value for anti\u2010ADAMTS13 IgG antibodies of 90.5\u2009U\/mL, with a modest discriminating ability (AUC: 0.57) for predicting long\u2010term response, precluding its use to guide therapeutic strategies. Nonetheless, the analysis of anti\u2010ADAMTS13 IgG antibodies titer trajectory from diagnosis revealed that the proportion of iTTP patients with ADAMTS13 activity improvement was higher in patients who decreased (Dec+) their antibodies titer within the 7\u201314\u2009days interval post\u2010TPE compared to those without decrease (Dec\u2212) (65% vs. 25% of cases, respectively,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001), a finding confirmed in a validation cohort (\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u200951). These results highlight the possibility of intensifying immunosuppression in an early period post\u2010TPE to shorten time to ADAMTS13 activity recovery. Close monitoring of anti\u2010ADAMTS13 antibodies titer may guide immunomodulation strategies, including additional courses of B\u2010cell depleting agents when appropriate.\n                  <\/jats:p>","DOI":"10.1002\/ajh.70005","type":"journal-article","created":{"date-parts":[[2025,7,15]],"date-time":"2025-07-15T11:49:39Z","timestamp":1752580179000},"page":"1736-1746","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Anti\u2010\n                    <scp>ADAMTS13<\/scp>\n                    Antibodies Trajectory is Associated With\n                    <scp>ADAMTS13<\/scp>\n                    Recovery in Immune\u2010Mediated\n                    <scp>TTP<\/scp>"],"prefix":"10.1002","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1374-6210","authenticated-orcid":false,"given":"Marie","family":"Robert","sequence":"first","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2995-767X","authenticated-orcid":false,"given":"Arthur","family":"Mageau","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP)  Paris France"},{"name":"D\u00e9partement de M\u00e9decine Interne H\u00f4pital Bichat\u2010Claude Bernard, HUPNVS, AP\u2010HP. Universit\u00e9 Paris Cit\u00e9. Inserm UMR 1137 IAME, Team DESCID  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8890-7341","authenticated-orcid":false,"given":"Ygal","family":"Benhamou","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne CHU Charles Nicolle  Rouen France"},{"name":"Universit\u00e9 de Normandie, UNIROUEN, INSERM U1096 EnVI  Rouen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0218-9153","authenticated-orcid":false,"given":"Fran\u00e7ois","family":"Prov\u00f4t","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie H\u00f4pital Albert Calmette  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3064-9401","authenticated-orcid":false,"given":"Jehane","family":"Fadlallah","sequence":"additional","affiliation":[{"name":"Service d\u2019Immunologie Clinique H\u00f4pital 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