{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T15:13:44Z","timestamp":1793373224747,"version":"4.5.0"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T00:00:00Z","timestamp":1786665600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Inborn errors of immunity (IEI) predispose patients to malignancy, particularly lymphoma, but data on diagnostic sequence and outcomes are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed IEI-associated malignancies diagnosed at a single center between January 2004 and December 2025.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 1,668 patients with IEI, 67 (4.0%) developed malignancy. Median ages at IEI and malignancy diagnosis were 8 and 10 years, respectively. Malignancy-first presentation occurred in 45 patients (67%), especially adolescents, and was associated with advanced-stage disease (p = 0.007). Lymphoid malignancies accounted for 90% of cases; non-Hodgkin lymphoma and Hodgkin lymphoma comprised 54% and 36%, respectively, and 84.7% presented with stage III-IV disease. Median event-free and overall survival were 66 and 155 months, respectively. In a clinically selected multivariable Cox model, NHL, solid\/plasma cell tumors, older age at malignancy diagnosis, and DNA repair defect status were associated with worse OS. Twelve patients underwent allogeneic HSCT; one died early, and no post-transplant relapses occurred.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Early immunologic evaluation and timely, individualized consideration of HSCT may support optimized management in children and adolescents, in whom cancer may be the first manifestation of IEI.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1904173","type":"journal-article","created":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T04:23:01Z","timestamp":1786681381000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical spectrum and survival outcomes of malignancies in pediatric patients with inborn errors of immunity"],"prefix":"10.3389","volume":"17","author":[{"given":"Nazli","family":"Deveci Demirbas","sequence":"first","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Department of Pediatrics, Ankara University Faculty of Medicine","place":["Ankara, T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zehra Sule","family":"Haskologlu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Department of Pediatrics, Ankara University Faculty of Medicine","place":["Ankara, T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tutku","family":"Baylan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Department of Pediatrics, Ankara University Faculty of Medicine","place":["Ankara, T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Candan","family":"Islamoglu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Department of Pediatrics, Ankara University 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