Original Research
AB051. Immunopathological profile of patients with thymic epithelial tumour and Good syndrome in advanced stage
Anna Maria Malfitano1, Erica Pietroluongo2, Michele Franceso Di Tolla1, Fabiana Napolitano1, Vittoria D’Esposito3, Rocco Morra2, Alberto Servetto2, Roberto Bianco2, Pietro Formisano1, Giovannella Palmieri4
1Department of Translational Medicine, University of Federico II, Naples, Italy;
2Department of Clinical Medicine and Surgery, University of Federico II, Naples, Italy;
3Institute of Endotypes in Oncology, Metabolism and Immunology “G. Salvatore”-National Research Council (IEOMI-CNR), Naples, Italy;
4Rare Tumors Coordinating Center of Campania Region (CRCTR), Naples, Italy
Correspondence to: Giovannella Palmieri, MD. Rare Tumors Coordinating Center of Campania Region (CRCTR), Via Pansini, 5, Naples, Naples, 80131, Italy. Email: giovpalm@unina.it.
Background: Thymic epithelial tumours (TETs) are frequently accompanied by Good syndrome (GS), a rare immunodeficiency characterized by hypogammaglobulinemia and B lymphopenia and other autoimmune diseases (ADs). Altered immunological mechanisms make necessary the monitoring of the immunophenotype in TET patients. The objective of this study is to evaluate differences in immunoglobulins, immunophenotype, and cytokine profiles in TET patients with or without GS (GS+/GS−) and in the presence/absence of other AD (AD+/AD−), and to assess whether these alterations differ according to disease stage (IVA/IVB) and in patients with no evidence of disease (NED).
Methods: Seventy TET patients were enrolled from 2019 to 2023 at Rare Tumors Coordinating Center of Campania Region. Immunoglobulin A (IgA), immunoglobulin M (IgM) and immunoglobulin G (IgG) were measured and the immunophenotype was analysed by 8-color immunophenotyping kit and Treg detection kit (CD4/CD25/CD127). Cytokines in serum samples were quantified by Enzyme-Linked Immunosorbent Assay (ELISA) multiplex assay.
Results: We observed reduction of IgM (0.8±0.1) and IgG (9.0±0.6) in GS+ patients; however, considering the stages and other AD presence, the decrease was maintained only for IgM in GS+AD− NED patients (0.2±0.1). B lymphopenia was detected in GS− patients in IVA/B (1.5±0.2) vs. NED, and in GS+ patients in both IVA/B (0.3±0.1) and NED (0.4±0.1) stages and independent of other AD presence. No difference was observed in other immune cells with the exclusion of Treg that increased in IVA/B (6.7±0.8) stage vs. NED in GS− patients, particularly in the presence of AD. Increase of Treg was detected in GS+AD+ NED (6.4±1.1) patients vs. both GS−AD+ and GS+AD− NED patients. A trend like that observed for Treg was reported for a panel of proinflammatory cytokines, namely interleukin-1 beta (IL-1β), interleukin-8 (IL-8), tumor necrosis factor alpha (TNFα), Regulated on Activation, Normal T Cell Expressed and Secreted (RANTES), and others.
Conclusions: Our results suggest that B lymphopenia is more specific than hypogammaglobulinemia to detect GS at different stages of the disease and independent of the presence of other AD. Unlike B cells, Treg increase in disease progression in GS− patients and particularly in AD+ patients. The increase of Treg in NED patients is associated with both GS+ and AD+. In addition, changes in Treg might parallel proinflammatory cytokine modulation. Overall, we suggest that immune modulation represents a key factor in TET diagnostic profile.
Keywords: Thymic epithelial tumours (TETs); Good syndrome (GS); autoimmune diseases (ADs); immunophenotype; cytokines
Acknowledgments
E.P. is supported by a Scholarship from ‘Associazione TUTOR’, year 2024–2025.
Funding: None.
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://med.amegroups.com/article/view/10.21037/med-25-ab051/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Institutional Review Board of the University of Naples Federico II (Protocol IRB approval n. 76.21), and individual consent for this retrospective analysis was waived.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
doi: 10.21037/med-25-ab051
Cite this abstract as: Malfitano AM, Pietroluongo E, Di Tolla MF, Napolitano F, D’Esposito V, Morra R, Servetto A, Bianco R, Formisano P, Palmieri G. AB051. Immunopathological profile of patients with thymic epithelial tumour and Good syndrome in advanced stage. Mediastinum 2025;9:AB051.
