Academic Journal

Multidisciplinary Delphi consensus on malignancy screening in patients with common variable immunodeficiency.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος: Multidisciplinary Delphi consensus on malignancy screening in patients with common variable immunodeficiency.
Συγγραφείς: Cabanero-Navalon MD; Primary Immunodeficiency Unit, Department of Internal Medicine, La Fe University and Polytechnic Hospital, Valencia, Spain., de Andrés-Martín A; Department of Immunology, Ramón y Cajal University Hospital, Madrid, Spain., Gil AA; Department of Internal Medicine, Bellvitge University Hospital, L'Hospitalet de Llobregat, Barcelona, Spain.; Adult Primary Immunodeficiency Unit, Bellvitge University Hospital, L'Hospitalet de Llobregat, Barcelona, Spain.; Bellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat, Barcelona, Spain., Bracke C; Fundació Lluita contra les Infeccions, Germans Trias i Pujol University Hospital, Badalona, Spain.; Department of Infectious Diseases, Germans Trias i Pujol University Hospital, Badalona, Spain., Carrillo-Linares JL; Department of Internal Medicine, Virgen de la Victoria University Hospital, Málaga, Spain.; Instituto de Investigación Biomédica de Málaga y Plataforma en Nanomedicina, IBIMA Plataforma BIONAND, Málaga, Spain., Cos ML; Primary Immunodeficiencies Functional Unit, Hospital del Mar, Barcelona, Spain.; Department of Internal Medicine, Hospital del Mar, Barcelona, Spain., Gasior M; Department of Hematology, La Paz University Hospital, Madrid, Spain., Gil-Niño J; Department of Internal Medicine, 12 de Octubre University Hospital, Madrid, Spain., González-Granado LI; Department of Pediatrics, Primary Immunodeficiencies Unit, 12 de Octubre University Hospital, Institute for Health Research (imas12), Madrid, Spain.; Complutense University School of Medicine, Madrid, Spain., López Osle N; Internal Medicine, Biobizkaia Health Research Institute, Barakaldo, Bizkaia, Spain.; Osakidetza, Cruces University Hospital, Barakaldo, Bizkaia, Spain., Robles-Marhuenda Á; Department of Internal Medicine, La Paz University Hospital, Madrid, Spain.; Working Group on Rare Diseases, Spanish Society of Internal Medicine (GTEM-SEMI), Madrid, Spain., Sánchez Martínez R; Internal medicine department - General Hospital Health Department, Alicante Institute for Health and Biomedical Research (ISABIAL), Alicante, Spain., Sánchez-Ramón S; Department of Clinical Immunology, San Carlos University Clinical Hospital, Madrid, Spain., Soler-Palacin P; Pediatric Infectious Diseases and Immunodeficiencies Unit, Hospital Universitari Vall d'Hebron, Vall d'Hebron Barcelona Hospital Campus, Barcelona, Catalonia, Spain.; Infection and Immunity in Pediatric Patients Research Group, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Barcelona Hospital Campus, Barcelona, Catalonia, Spain., Solanich X; Department of Internal Medicine, Bellvitge University Hospital, L'Hospitalet de Llobregat, Barcelona, Spain.; Adult Primary Immunodeficiency Unit, Bellvitge University Hospital, L'Hospitalet de Llobregat, Barcelona, Spain.; Bellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat, Barcelona, Spain.; Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain., Torralba-Cabeza MÁ; Department of Internal Medicine, Lozano Blesa University Clinical Hospital, Zaragoza, Spain.; Aragon Health Research Institute, Zaragoza, Spain.; Working Group on Rare Diseases, Spanish Society of Internal Medicine, Zaragoza, Spain., Aparicio Tormo JR; Endoscopy Unit, Dr. Balmis General University Hospital, Alicante, Spain., Alsina L; Pediatric Allergy and Clinical Immunology Department, Clinical Immunology and Primary Immunodeficiencies Unit, Sant Joan de Déu Hospital, Barcelona, Spain.; Department of Surgery and Surgical Specialties, Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain.; Study Group for Immune Dysfunction Diseases in Children (GEMDIP), Sant Joan de Déu Research Institute (IRSJD), Barcelona, Spain., Moral Moral P; Primary Immunodeficiency Unit, Department of Internal Medicine, La Fe University and Polytechnic Hospital, Valencia, Spain.
Πηγή: Frontiers in immunology [Front Immunol] 2026 Aug 17; Vol. 17, pp. 1897051. Date of Electronic Publication: 2026 Aug 17 (Print Publication: 2026).
Τύπος έκδοσης: Journal Article; Consensus Statement
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101560960 Publication Model: eCollection Cited Medium: Internet ISSN: 1664-3224 (Electronic) Linking ISSN: 16643224 NLM ISO Abbreviation: Front Immunol Subsets: MEDLINE
Imprint Name(s): Original Publication: [Lausanne : Frontiers Research Foundation]
Ιατρικοί όροι (MeSH): Common Variable Immunodeficiency*/complications , Common Variable Immunodeficiency*/immunology , Common Variable Immunodeficiency*/epidemiology , Common Variable Immunodeficiency*/diagnosis , Early Detection of Cancer*/methods , Neoplasms*/diagnosis , Neoplasms*/etiology , Neoplasms*/epidemiology, Spain/epidemiology ; Humans ; Delphi Technique ; Adult ; Risk Factors
Περίληψη: Introduction: Common variable immunodeficiency (CVID) is the most frequent symptomatic inborn error of immunity (IEI) in adults and is characterized by recurrent infections, immune dysregulation, and an increased risk of malignancy. Cancer represents a major cause of morbidity and mortality in this population, particularly hematological malignancies and gastric carcinoma. Data from the Spanish cohort report malignancy rates of up to 15%, with substantial variability in surveillance practices across centers. Despite this well-established cancer predisposition, standardized, evidence-based screening strategies are lacking, and no specific recommendations for malignancy surveillance in CVID currently exist. Therefore, this two-round Delphi consensus aimed to develop expert-based recommendations for cancer screening and surveillance in patients with CVID.
Methods: The process included 39 multidisciplinary experts from 22 Spanish hospitals, including adult and pediatric centers, who evaluated a structured questionnaire comprising 87 statements across four domains: epidemiology/risk factors, solid tumors, hematological malignancies, and immunological biomarkers. The questionnaire was developed from a targeted literature review, and statements were rated using a five-point Likert scale, with consensus defined as ≥70% agreement/disagreement.
Results: Overall, 70 statements, corresponding to 80.5%, reached agreement. Consensus supported systematic genetic testing at diagnosis and annual immunophenotyping to identify malignancy risk markers. In adults, baseline upper gastrointestinal endoscopy with histology-guided follow-up was recommended, together with annual Helicobacter pylori testing and monitoring of micronutrients and pernicious anemia-related antibodies. Hematological surveillance should include systematic evaluation of lymphadenopathy, with imaging ± biopsy when indicated, and bone marrow aspirate/biopsy in persistent (>6 months) cytopenias, while unexplained weight loss should prompt computed tomography imaging. Pediatric management should remain individualized and age-adapted, avoiding direct extrapolation from adult screening strategies.
Discussion: This Delphi consensus proposes a structured approach to malignancy screening and surveillance in CVID from the time of diagnosis, encompassing gastric, hematological, and immunological assessment. These cancer-focused recommendations are intended to complement existing CVID management guidance, supporting earlier detection of malignancy and reducing variability in clinical practice.
(Copyright © 2026 Cabanero-Navalon, de Andrés-Martín, Gil, Bracke, Carrillo-Linares, Cos, Gasior, Gil-Niño, González-Granado, López Osle, Robles-Marhuenda, Sánchez Martínez, Sánchez-Ramón, Soler-Palacin, Solanich, Torralba-Cabeza, Aparicio Tormo, Alsina and Moral Moral.)
Competing Interests: The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The authors LG-G, PS-P declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.
References: Cancers (Basel). 2023 Jan 03;15(1):. (PMID: 36612313)
J Allergy Clin Immunol. 2024 Jan;153(1):12-27. (PMID: 37890706)
Front Immunol. 2019 Mar 26;10:586. (PMID: 30984175)
J Hum Immun. 2025 Apr 15;1(1):e20250002. (PMID: 41608113)
Front Immunol. 2024 Oct 16;15:1465159. (PMID: 39478863)
Eur J Pediatr. 2020 May;179(5):689-697. (PMID: 32162064)
Front Genet. 2024 Oct 02;15:1409754. (PMID: 39415980)
Expert Rev Clin Immunol. 2019 Oct;15(10):1105-1113. (PMID: 31452405)
J Allergy Clin Immunol Pract. 2017 Jul - Aug;5(4):938-945. (PMID: 28351785)
Front Immunol. 2023 Oct 06;14:1231242. (PMID: 37868983)
Orphanet J Rare Dis. 2018 Nov 12;13(1):201. (PMID: 30419968)
North Clin Istanb. 2024 Jun 24;11(3):201-207. (PMID: 39005741)
J Clin Immunol. 2024 Oct 28;45(1):34. (PMID: 39466473)
Blood. 2008 Jan 1;111(1):77-85. (PMID: 17898316)
J Allergy Clin Immunol Pract. 2020 Nov - Dec;8(10):3342-3347. (PMID: 33161963)
Front Immunol. 2022 Jul 18;13:928062. (PMID: 35924244)
Front Immunol. 2022 May 31;13:834137. (PMID: 35711410)
J Allergy Clin Immunol. 2026 Mar;157(3):739-753. (PMID: 41242640)
Clin Exp Immunol. 2011 Jul;165(1):1-7. (PMID: 21470209)
Front Immunol. 2022 Oct 28;13:1033666. (PMID: 36389743)
Blood. 2012 Feb 16;119(7):1650-7. (PMID: 22180439)
Pediatr Blood Cancer. 2025 Sep;72(9):e31860. (PMID: 40551365)
Endoscopy. 2025 May;57(5):504-554. (PMID: 40112834)
Int J Mol Sci. 2018 Feb 02;19(2):. (PMID: 29393912)
Endoscopy. 2019 Apr;51(4):365-388. (PMID: 30841008)
J Allergy Clin Immunol Pract. 2019 Jul - Aug;7(6):1763-1770. (PMID: 30776527)
J Clin Immunol. 2019 Oct;39(7):678-687. (PMID: 31377970)
J Clin Immunol. 2018 Oct;38(7):768-777. (PMID: 30219982)
Medicine (Baltimore). 2023 Feb 17;102(7):e32829. (PMID: 36800594)
Ann Allergy Asthma Immunol. 2021 Jul;127(1):19-27. (PMID: 33716149)
Am J Clin Pathol. 2012 Aug;138(2):185-9. (PMID: 22904128)
Allergol Immunopathol (Madr). 2017 Nov - Dec;45(6):602-615. (PMID: 28411962)
J Clin Immunol. 2017 Aug;37(6):592-602. (PMID: 28755066)
Gastroenterol Hepatol. 2024 Oct;47(8):793-803. (PMID: 37598725)
J Investig Allergol Clin Immunol. 2024 Jul;34(4):233-245. (PMID: 39077769)
Front Immunol. 2019 Nov 22;10:2678. (PMID: 31824486)
Eur J Immunol. 2025 Mar;55(3):e202451433. (PMID: 40079712)
Clin Rev Allergy Immunol. 2020 Aug;59(1):109-121. (PMID: 31720921)
J Allergy Clin Immunol Pract. 2016 Jan-Feb;4(1):38-59. (PMID: 26563668)
Eur J Haematol. 2021 Nov;107(5):503-516. (PMID: 34255892)
Dig Dis Sci. 2025 Sep;70(9):2956-2965. (PMID: 40455348)
Contributed Indexing: Keywords: Delphi technique; common variable immunodeficiency; mass screening; neoplasms; population surveillance
Entry Date(s): Date Created: 20260901 Date Completed: 20260901 Latest Revision: 20261005
Update Code: 20261005
PubMed Central ID: PMC13526613
DOI: 10.3389/fimmu.2026.1897051
PMID: 42676356
Βάση Δεδομένων: MEDLINE
Περιγραφή
ISSN:1664-3224
DOI:10.3389/fimmu.2026.1897051