Epidemiology and management of malignancies in patients with inborn errors of immunity—An ESID registry study of 19,959 patients

Publikation: Beitrag in Fachzeitschrift › Forschungsartikel › Beigetragen › Begutachtung

Beitragende

  • ESID Registry Working Party - (Autor:in)

Abstract

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.

Details

OriginalspracheEnglisch
Seiten (von - bis)739-753
Seitenumfang15
FachzeitschriftJournal of allergy and clinical immunology
Jahrgang157
Ausgabenummer3
Frühes Online-Datum14 Nov. 2025
PublikationsstatusVeröffentlicht - März 2026
Peer-Review-StatusJa

Externe IDs

PubMed 41242640
ORCID /0009-0003-6519-0482/work/203813553
ORCID /0000-0001-6313-4434/work/203814407

Schlagworte

Ziele für nachhaltige Entwicklung

  • SDG 3 – Gute Gesundheit und Wohlergehen - cancer predisposition syndromes, ESID registry, Inborn errors of immunity (IEI), malignancy, primary immune disorder (PID), primary immunodeficiency (PID), tumor predisposition

ASJC Scopus Sachgebiete

  • Immunologie und Allergologie - cancer predisposition syndromes, ESID registry, Inborn errors of immunity (IEI), malignancy, primary immune disorder (PID), primary immunodeficiency (PID), tumor predisposition
  • Immunologie - cancer predisposition syndromes, ESID registry, Inborn errors of immunity (IEI), malignancy, primary immune disorder (PID), primary immunodeficiency (PID), tumor predisposition

Schlagwörter

  • cancer predisposition syndromes, ESID registry, Inborn errors of immunity (IEI), malignancy, primary immune disorder (PID), primary immunodeficiency (PID), tumor predisposition