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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Bogaert, DJA; Wolfsberger, CH; Attarbaschi, A; Gathmann, J; Warnatz, K; Mueller, G; Mukhina, A; Rusch, S; Kindle, G; van, Montfrans, JM; Seidel, MG, , ESID, Registry, Working, Party.
Epidemiology and Management of Malignancies in Patients with Inborn Errors of Immunity - An ESID Registry Study of 19,959 Patients.
J Allergy Clin Immunol. 2025; Doi: 10.1016/j.jaci.2025.10.033
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Führende Autor*innen der Med Uni Graz

Seidel Markus

Co-Autor*innen der Med Uni Graz

Wolfsberger Christina Helene

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Abstract:

BACKGROUND: Inborn errors of immunity (IEI), or primary immune disorders (PID), predispose individuals to infections, autoimmunity, inflammation, allergy, and malignancy. Malignancies are a major cause of morbidity and mortality in IEI/PID patients, with poorer outcomes compared to the general population. OBJECTIVES: This European Society for Immunodeficiencies Registry (ESID-R) study aimed to determine the frequency and types of malignancies in IEI/PID patients and to assess clinical management approaches across Europe. METHODS: Descriptive analyses were performed on malignancy data within each IEI category. Additionally, an ESID-R survey (05/2022-03/2024) collected data on management strategies and challenges. RESULTS: Of 19,959 IEI/PID patients, 1,783 (8.9%) developed malignancies, of whom 27.1% presented malignancy as first manifestation of IEI/PID. A total of 1,210 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 to other IEI/PID categories, e.g., combined immunodeficiencies with syndromic or associated features (11.7 years). Survey findings revealed oncological treatment was modified due to IEI/PID in 21.5% of cases, with assumed negative impacts of IEI/PID on complications and outcomes (in 27.4% and 30.7%, respectively). IEI/PID influenced transplant decisions in 16.5% of cases. Management practices like interdisciplinary decision finding and guideline availability were recorded. CONCLUSION: This study provides comprehensive epidemiological data on malignancies in IEI/PID, highlighting the need for tailored screening and management. Survey results emphasize the real-world challenges and support the development of IEI/PID-specific oncologic surveillance guidelines and treatment strategies.

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