20.500.12556/DiRROS-28804 Epidemiology and management of malignancies in patients with inborn errors of immunity - an ESID registry study of 19,959 patients Epidemiology and management of malignancies in patients with inborn errors of immun Background Inborn errors of immunity (IEI), or primary immune disorders (PIDs), predispose individuals to infections, autoimmunity, inflammation, allergy, and malignancy. Malignancies are a major cause of morbidity and mortality in patients with IEI/PIDs, with poorer outcomes compared with the general population. Objective We sought to determine the frequency and types of malignancies in patients with IEI/PIDs and to assess clinical management approaches across Europe. Methods Descriptive analyses were performed on malignancy data within each IEI category. In addition, a European Society for Immunodeficiencies Registry survey (05/2022-03/2024) collected data on management strategies and challenges. Results Of 19,959 patients with IEI/PIDs, 1783 (8.9%) developed malignancies, of whom 27.1% presented malignancy as first manifestation of IEI/PIDs. A total of 1210 malignancies were specified; B-cell non-Hodgkin lymphoma was most common (24.2%). Detailed malignancy-IEI/PID association maps are provided. Predominantly antibody deficiencies accounted for 59.1% of malignancy cases, with a higher median age at first malignancy (43.6 years) compared with other IEI/PID categories, for example, combined immunodeficiencies with syndromic or associated features (11.7 years). Survey findings revealed that oncological treatment was modified because of IEI/PIDs in 21.5% of cases, with assumed negative impacts of IEI/PIDs on complications and outcomes (in 27.4% and 30.7%, respectively). IEI/PIDs influenced transplant decisions in 16.5% of cases. Management practices such as interdisciplinary decision finding and guideline availability were recorded. Conclusions This study provides comprehensive epidemiological data on malignancies in IEI/PIDs, highlighting the need for tailored screening and management. Survey results emphasize the real-world challenges and support the development of IEI/PID-specific oncological surveillance guidelines and treatment strategies. inborn errors of immunity IEI primary immunodeficiency PID primary immune disorder malignancy cancer predisposition syndromes tumor predisposition ESID registry prirojene napake imunosti primarne imunske pomanjkljivosti primarne imunske motnje malignomi sindromi nagnjenosti k raku predispozicija za tumorje register ESID true false true Angleški jezik Slovenski jezik Neznano 2026-04-08 10:24:33 2026-04-08 10:24:34 2026-04-09 03:51:17 0000-00-00 00:00:00 2026 0 0 Nasl. z nasl. zaslona; Opis vira z dne 7. 4. 2026; str. 739-753 no. 3 Vol. 157 Mar. 2026 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 616-006:616-056.7 1097-6825 10.1016/j.jaci.2025.10.033 262327299 RAZ_Bogaert_Delfien_J._A._2026.pdf RAZ_Bogaert_Delfien_J._A._2026.pdf 1 99C0A2489D905D662C2757A17DB61415 3b78559f7d9eefa0ebe734aa7274c9ccfaa818041212015c34acc8a81800a441 babdd115-3324-11f1-b941-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=42905 https://www.jacionline.org/article/S0091-6749(25)01126-1/fulltext 1 62a33ea4-3324-11f1-b941-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=42903 Univerzitetni klinični center Ljubljana 0 0 0