{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T11:27:30Z","timestamp":1772191650709,"version":"3.50.1"},"reference-count":37,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,9,24]],"date-time":"2022-09-24T00:00:00Z","timestamp":1663977600000},"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":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Thrombotic thrombocytopenic purpura (TTP) is a potentially life\u2010threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and severely reduced or absent ADAMTS13 (A disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) activity, with varying degrees of organ dysfunction. As TTP is rare in pediatrics, most of the medical and scientific literature has largely reported on adult patients. As a result, limited data exist regarding the clinical features, comorbidities, treatment response, and long\u2010term outcomes in pediatric patients with immune\u2010mediated TTP.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A single\u2010center retrospective cohort study was conducted of all children and adolescents presenting to Children's Healthcare of Atlanta, Atlanta, Georgia, between the years 2001 and 2021 with immune\u2010mediated TTP (iTTP). Clinical features, treatments, and outcomes, including long\u2010term neurocognitive function, were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighteen individuals were identified, six of whom had a total of 10 relapses, amounting to 28 episodes overall. Thirty\u2010eight percent of the patients experienced exacerbations but, ultimately, 85% achieved a clinical response and clinical remission. Only one in\u2010hospital death occurred (mortality rate 5.5%). Seventy\u2010three percent of analyzed patients demonstrated long\u2010term neurocognitive abnormalities, including cognitive delay, learning difficulties, and severe depression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Children and adolescents recovering from iTTP are at high risk for neurocognitive deficits from initial and possibly ongoing microvascular disease. Due to risk for long\u2010term neurological deficits, we recommend neuropsychological testing in addition to monitoring of other organ functions in all children with TTP, as well as long\u2010term surveillance of ADAMTS13 activity during remission to detect and promptly treat early relapse.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.29992","type":"journal-article","created":{"date-parts":[[2022,9,24]],"date-time":"2022-09-24T13:13:47Z","timestamp":1664025227000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical features and neurological outcomes in pediatric immune\u2010mediated thrombotic thrombocytopenic purpura: A report from a large pediatric hematology center"],"prefix":"10.1002","volume":"69","author":[{"given":"Sara","family":"Graciaa","sequence":"first","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia 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E.","family":"Zerra","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Center for Transfusion and Cellular Therapy Department of Pathology and Laboratory Medicine Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0866-3090","authenticated-orcid":false,"given":"Carolyn","family":"Bennett","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cassandra D.","family":"Josephson","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Center for Transfusion and Cellular Therapy Department of Pathology and Laboratory Medicine Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Briones","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ross M.","family":"Fasano","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Center for Transfusion and Cellular Therapy Department of Pathology and Laboratory Medicine Emory University School of Medicine  Atlanta Georgia 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