Journal of Clinical Immunology最新文献

{"title":"Cobalamin C Deficiency Presenting with CVID-like Immunologic Abnormalities: A Five-Patient Pediatric Case Series.","authors":"Ruxuan He, Jinrong Liu, Shunying Zhao, Haiming Yang","doi":"10.1007/s10875-026-02068-0","DOIUrl":"10.1007/s10875-026-02068-0","url":null,"abstract":"","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13503436/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148812222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Elevated IL-10 is Linked With the Expansion of T-bet^highCD21^low B Cells in Patients With Common Variable Immunodeficiency.","authors":"Victoria Cousin, Anna Graumann, Valerie Geiger, David Sökler, Patrick Bez, Sylvia Gutenberger, Cornelia Glaser, Geoffroy Andrieux, Melanie Boerries, Björn Christian Frye, Gernot Zissel, Celia Lourdes Calvillo, Javier Rodriguez-Ubreva, Esteban Ballestar, Fabian Hauck, Reinhard E Voll, Nina Chevalier, Baerbel Keller, Klaus Warnatz","doi":"10.1007/s10875-026-02058-2","DOIUrl":"10.1007/s10875-026-02058-2","url":null,"abstract":"<p><p>Common variable immunodeficiency (CVID) is frequently complicated by autoimmune and inflammatory manifestations associated with profound immune dysregulation (CVIDc), including expansion of T-bet^highCD21^low B cells (CD21^low B cells). Elevated serum IL-10 levels have repeatedly been reported in CVIDc, yet their relationship to CD21^low B-cell differentiation remains unclear. In this study, we determined a significant correlation between increased serum IL-10 levels and frequency of circulating CD21^low B cells, particularly marked in CVIDc patients. Transcriptomic and protein analyses identified multiple cellular sources of IL-10 in CVID, including monocytes, T cells, and a subset of CD21^low B cells in peripheral blood and inflamed tissues. CD21^low B cells expressed elevated levels of IL-10 receptor subunits and displayed intact IL-10-induced STAT3 signaling, indicating preserved responsiveness to IL-10. Functionally, IL-10 alone neither induced CD21^low B-cell differentiation nor inhibited IFN-γ-driven polarization. However, in vitro differentiation assays showed that IL-10 can substitute for IL-21 during CD40L/T cell-dependent differentiation of CD21^low-like B cells and promote their survival in vitro. These findings suggest that IL-10 may support the expansion and persistence of CD21^low B cells at inflammatory sites. Collectively, our data identify IL-10 as a component of the inflammatory environment associated with CD21^low B-cell expansion and suggest that IL-10 can functionally replace IL-21 during their differentiation and promote their survival in CVID-associated immune dysregulation.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13477453/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148764633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to: Rare Pathogenic Variants in Mitochondrial and Inflammation-Associated Genes May Lead to Inflammatory Cardiomyopathy in Chagas Disease.","authors":"Maryem Ouarhache, Sandrine Marquet, Amanda Farage Frade, Ariela Mota Ferreira, Barbara Ianni, Rafael Ribeiro Almeida, Joao Paulo Silva Nunes, Ludmila Rodrigues Pinto Ferreira, Vagner Oliveira-Carvalho Rigaud, Darlan Cândido, Charles Mady, Ricardo Costa Fernandes Zaniratto, Paula Buck, Magali Torres, Frederic Gallardo, Pauline Andrieux, Sergio Bydlowsky, Debora Levy, Laurent Abel, Clareci Silva Cardoso, Omar Ribeiro Santos-Junior, Lea Campos Oliveira, Claudia Di Lorenzo Oliveira, Maria Do Carmo Nunes, Aurelie Cobat, Jorge Kalil, Antonio Luiz Ribeiro, Ester Cerdeira Sabino, Edecio Cunha-Neto, Christophe Chevillard","doi":"10.1007/s10875-026-02061-7","DOIUrl":"10.1007/s10875-026-02061-7","url":null,"abstract":"","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13477482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148758267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune Dysregulation in Down Syndrome: Implications for Infectious Susceptibility and Vaccine Response.","authors":"Sofía Solari-Hernández, Enrique González-Madrid, Pablo A González, Susan M Bueno, Alexis M Kalergis","doi":"10.1007/s10875-026-02054-6","DOIUrl":"10.1007/s10875-026-02054-6","url":null,"abstract":"<p><p>Down syndrome (DS), caused by trisomy 21, is characterized by complex immune dysregulation that increases susceptibility to infections and alters vaccine responsiveness. Gene dosage effects involving interferon receptor loci on chromosome 21 contribute to chronic type I interferon hyperactivation, sustained JAK-STAT signaling, and persistent expression of interferon-stimulated genes. This baseline inflammatory state is accompanied by quantitative and functional defects in both innate and adaptive immunity, including impaired neutrophil chemotaxis, pro-inflammatory monocyte polarization, reduced naïve T- and B-cell compartments, restricted antigen receptor diversity, diminished class-switched memory B cells, and features of accelerated immunosenescence. Clinically, these immune alterations are associated with an increased risk of severe respiratory viral infections, such as respiratory syncytial virus, influenza, and SARS-CoV-2, as well as reduced magnitude and durability of vaccine-induced immunity. Cohort studies in both pediatric and adult populations have reported higher rates of hospitalization and mortality, along with lower peak antibody titers and more rapid waning of immunity following vaccination. This review integrates molecular, cellular, and clinical evidence to define the interferon-driven immune phenotype in DS and its implications for susceptibility to infection and vaccination response. A mechanistic understanding of this immune landscape provides a framework for developing tailored preventive strategies and optimizing vaccination approaches in this vulnerable population.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13534213/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148874270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgG4-Related Hepatic Disease Unmasking Hepatic Actinomycosis.","authors":"Anissa Desmoulin, Jasmine Muyard, Laure-Marie Dardaud, Anne-Gaëlle Leroy, Sabine Revuz","doi":"10.1007/s10875-026-02049-3","DOIUrl":"https://doi.org/10.1007/s10875-026-02049-3","url":null,"abstract":"<p><strong>Purpose: </strong>Hepatic actinomycosis is a rare granulomatous disease caused by an opportunistic Gram-positive bacillus of the genus Actinomyces. We report an unusual association between hepatic actinomycosis and IgG-4 related disease.</p><p><strong>Case description: </strong>A 70-year-old man was admitted to the University Hospital of Reunion Island for asthenia and abdominal pain. Laboratory tests showed hepatic cholestasis and inflammatory reaction without cytolysis. The computer tomography scanner (CT scan) revealed a large hepatic mass in segment IV. IgG4 related disease (IgG4-RD) was diagnosed following liver biopsy. Despite the use of corticosteroids, the lesions progressed. A new biopsy was performed and a PCR test returned positive for Actinomyces spp. We observed patient improvement after 4 months of treatment with amoxicillin and discontinuation of corticosteroids.</p><p><strong>Conclusions: </strong>Clinical presentation, imaging characteristics, and diagnostic challenges are strikingly similar between hepatic actinomycosis and IgG4-related disease. However, distinguishing between these two disorders is essential - as the treatment of one may worsen the course of the other. Knowing and understanding the interactions between these two disorders can only be achieved through an integrated and collaborative approach involving various disciplines.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":" ","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148470953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Detrimental NFKB2 Missense Variant is Associated with Hypogammaglobulinemia.","authors":"Manfred Fliegauf, Laura Gamez-Diaz, Pavla Mrovecova, Chiara Milena God, Valerie Flavia Geiger, Nadezhda Camacho-Ordonez, Sara Posadas-Cantera, Cliodhna Murray, Klaus Warnatz, Baerbel Keller, Bodo Grimbacher","doi":"10.1007/s10875-026-02051-9","DOIUrl":"10.1007/s10875-026-02051-9","url":null,"abstract":"<p><p>NFKB2 encodes the precursor p100 which undergoes processing to generate the mature NF-κB2 transcription factor subunit p52. Most of the known pathogenic NFKB2 variants render p100 un-processable and are typically linked to immunodeficiency disorders with antibody deficiency, susceptibility to infections and often autoimmunity. We describe a heterozygous germline NFKB2 missense variant (c.781C>T; R261W) associated with antibody deficiency and recurrent respiratory tract infections in a German family with incomplete penetrance. Patient-derived cells had reduced p52 levels, indicating protein insufficiency as the primary defect. Characterization of the R261W variant in vitro employing overexpression of EGFP-fused wildtype and mutant p100/p52 in HEK293T cells revealed restricted levels of mutant p100, suggesting unsustainable protein expression while maintenance of mutant p52 was almost precluded, confirming a detrimental protein defect. Enforced p100-processing driven by constitutively active NF-κB inducing kinase (NIK) caused subnuclear aggregation of mutant EGFP-p52 while DNA-binding activity was undetectable. Although ectopically over-expressed EGFP-p52-R261W also formed intra-nuclear aggregates - a previously established diagnostic indicator of protein-decaying NFKB1 mutations - its DNA-binding ability was not abolished. In summary, the single amino acid substitution R261W in the N-terminal Rel-homology domain of p100/p52 causes protein loss, particularly of the mature subunit p52. This genetic condition is associated with an inheritable form of hypogammaglobulinemia with mild clinical symptoms. We therefore recommend genetic screening for NFKB2 loss-of-expression variants in mildly affected patients with recurrent respiratory tract infections.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13356095/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148421414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Deciphering B cell Maturation Dynamics in Hyper-IgM Syndromes.","authors":"Hande Üçler Çınar, Murat Cansever, Şerife Erdem, Abdullah Arık, Ahmet Eken","doi":"10.1007/s10875-026-02046-6","DOIUrl":"https://doi.org/10.1007/s10875-026-02046-6","url":null,"abstract":"<p><strong>Purpose: </strong>Hyper-IgM syndromes (HIGM) are primary immunodeficiencies characterized by defective class-switch recombination (CSR) and impaired humoral immunity. While genetic causes such as CD40L and AICDA mutations are well established, a detailed comparison of B cell maturation dynamics across HIGM subtypes remains limited. To comprehensively characterize B cell immunophenotypes and functional responses in patients with HIGM and to delineate mutation-specific differences in B cell maturation and proliferation.</p><p><strong>Method: </strong>Four patients with genetically confirmed HIGM (one CD40L and three homozygous AICDA mutations, c.70C>T; p.R24W) and age- and sex-matched healthy controls were studied. Peripheral blood mononuclear cells were analyzed by multiparameter flow cytometry to define B cell subsets based on CD19, CD20, CD24, CD27, CD38, IgD, and IgM expression. B cell proliferation was assessed following CpG stimulation.</p><p><strong>Results: </strong>All patients exhibited a marked reduction of class-switched memory B cells (CD27⁺IgD⁻) and accumulation of naive B cells (CD27⁻IgD⁺), consistent with defective CSR. The CD40L-deficient patient demonstrated profound depletion of plasmablasts and precursor skewing, reflecting failure of germinal center formation. In contrast, AID patients showed preserved CD27 expression with variable expansion of transitional and plasmablast populations, suggesting intact T cell-dependent activation but intrinsic failure of CSR. Functional assays revealed heterogeneous proliferative responses in CD40L deficiency and AICDA-deficient patient, but impaired proliferation in another AICDA-deficient individual, highlighting inter-individual variability.</p><p><strong>Conclusion: </strong>Detailed immunophenotyping reveals distinct B cell maturation arrest points in CD40L- versus AICDA-associated HIGM. Flow cytometric analysis of B cell subsets provides valuable insights into disease mechanisms, supports differential diagnosis, and may inform clinical monitoring and therapeutic decision-making in HIGM.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":" ","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bronchiectasis in Inborn Errors of Immunity: Prevalence, Predictors, and Cardiopulmonary Complications in a Genetically Characterized Cohort.","authors":"Diana Marangu-Boore, Katherine Myint-Hpu, Esther Kang, Luigi D Notarangelo, Ottavia M Delmonte","doi":"10.1007/s10875-026-02044-8","DOIUrl":"https://doi.org/10.1007/s10875-026-02044-8","url":null,"abstract":"<p><strong>Purpose: </strong>Bronchiectasis poses a serious but incompletely defined burden in patients with inborn errors of immunity (IEI). We determined its prevalence, independent predictors, and cardiopulmonary complications in a genetically characterized IEI cohort to inform care in this vulnerable population.</p><p><strong>Methods: </strong>We conducted a cross-sectional analysis of patients enrolled in a National Institutes of Health prospective IEI protocol (2018-2024) who had undergone whole exome or genome sequencing and chest computed tomography (CT). Bronchiectasis was radiologically confirmed, excluding traction bronchiectasis. Predictors were identified by multivariable logistic regression. Cardiopulmonary outcomes included spirometry, lung volumes, diffusing capacity, six-minute walk distance, pulmonary hypertension by echocardiography, and mortality.</p><p><strong>Results: </strong>Of 229 enrolled patients, 131 were eligible, representing 31 distinct IEIs. Median age at first chest CT was 20 years (IQR 10-33). The most common CT finding was pulmonary nodules (55%). Bronchiectasis prevalence was 27% (95% CI 20-35%). Independent predictors were older age at first CT (aOR 1.04, 95% CI 1.01-1.08), combined immunodeficiency affecting cellular and humoral immunity (aOR 4.30, 95% CI 1.42-14.54), predominantly antibody deficiencies (aOR 5.83, 95% CI 1.66-22.26), and diseases of immune dysregulation (aOR 7.56, 95% CI 1.07-52.72). Patients with bronchiectasis had greater cardiopulmonary impairment across all measured domains and higher mortality (15% vs. 3%; P = 0.046).</p><p><strong>Conclusions: </strong>Bronchiectasis is common across IEI diagnostic classes and carries substantial cardiopulmonary morbidity and excess mortality. Older age at first chest CT and specific IUIS classes, particularly predominantly antibody deficiencies and diseases of immune dysregulation, independently predict bronchiectasis, identifying patients who would benefit most from early pulmonary surveillance.</p><p><strong>Trial protocol: </strong>NCT03394053.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":" ","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148404955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adult-Onset LRBA Deficiency Presenting with Rheumatoid Arthritis-Like Manifestations: A Case Report.","authors":"Keita Ninagawa, Yuki Kudo, Michihito Kono, Ryo Hisada, Tatsuya Atsumi","doi":"10.1007/s10875-026-02048-4","DOIUrl":"10.1007/s10875-026-02048-4","url":null,"abstract":"<p><p>Lipopolysaccharide-responsive beige-like anchor protein (LRBA) deficiency is a primary inborn error of immunity characterized by immune dysregulation and frequently associated with autoimmune connective tissue manifestations. We describe an adult woman diagnosed with rheumatoid arthritis who was subsequently found to have LRBA deficiency based on genetic testing and investigated inflammatory proteins potentially involved in the pathogenesis of LRBA deficiency. A 44-year-old woman with rheumatoid arthritis presented with persistent diarrhea and abdominal distention lasting over eight months. Abdominal imaging revealed intestinal pseudo-obstruction. Her medical history included megaloblastic anemia, type 1 diabetes, and chronic thyroiditis since childhood. Her brother had died of unexplained diarrhea during childhood. Given her multiple autoimmune manifestations and family history, a primary immune deficiency (PID) was suspected. Genetic analysis identified a heterozygous LRBA splice-site variant (c.1162-1G > A) and a possible heterozygous deletion involving exons 18-41, suggesting compound heterozygous LRBA variants. Thus, the diagnosis of LRBA deficiency-associated autoimmunity. Following the diagnosis, she was treated with abatacept, a CTLA4-immunoglobulin fusion protein, resulting in improvement of gastrointestinal symptoms. Proximity extension assay (Olink^® Target) revealed marked decreases in serum interleukin-17 A (from 3.36 to 2.22 normalized protein expression), tumor necrosis factor (3.26 to 0.69), and chemokine (C-C motif) ligand 20 (3.14 to 0.84) after treatment. LRBA deficiency is characterized by an imbalance of CD4⁺ T cells with increased Th1 and Th17 populations and reduced regulatory T cells. This case highlights that patients with PID, including LRBA deficiency, can present with autoimmune manifestations, and abatacept may represent an effective targeted treatment option for LRBA-deficiency.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"46 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13337776/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148390902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunopathological Profile of Patients with Thymic Epithelial Tumour and Good Syndrome in Advanced Stage.","authors":"Fabiana Napolitano, Erica Pietroluongo, Michele Francesco Di Tolla, Margaret Ottaviano, Vittoria D'Esposito, Pietro De Placido, Alberto Servetto, Giuseppe Portella, Roberto Bianco, Giovannella Palmieri, Pietro Formisano, Anna Maria Malfitano","doi":"10.1007/s10875-026-02050-w","DOIUrl":"https://doi.org/10.1007/s10875-026-02050-w","url":null,"abstract":"<p><strong>Purpose: </strong>Thymic epithelial tumors (TETs) are associated with Good Syndrome (GS), a secondary immunodeficiency characterized by hypogammaglobulinemia, B-cell lymphopenia, and recurrent infections. This study investigated the immunological profile of TET patients to identify immune alterations associated with GS, independently of autoimmune diseases (AD) and disease stage.</p><p><strong>Methods: </strong>Seventy patients with TETs were stratified according to GS status (GS+/GS-), AD status (AD+/AD-), and disease stage (advanced disease, IVA/B, or no evidence of disease, NED). Serum immunoglobulins, peripheral immune cell subsets, and circulating cytokines, chemokines, growth factors, and metabolic markers were evaluated. Results GS+ patients showed reduced IgM and IgG levels compared with GS- patients; however, after stratification, only IgM remained reduced in GS+AD- NED patients. Peripheral B-cell lymphopenia was consistently observed in GS+ patients across both disease stages, independently of AD status. Treg frequencies increased in GS- patients with advanced disease and in GS+AD+ NED patients. Monocytopenia and an inverted CD4+/CD8+ ratio were mainly observed in GS+AD+ patients with advanced disease. Cytokine profiling identified increased levels of IL-1β, IL-10, IL-17, TNFα, IFN-γ, IL-4, IL-5, IL-6, Eotaxin, G-CSF, and IP-10 in GS+ patients. Among these, IP-10 remained consistently elevated regardless of disease stage or AD status.</p><p><strong>Conclusion: </strong>Peripheral B-cell lymphopenia and elevated IP-10 levels represent the most consistent immunological features associated with GS, independent of disease stage and AD status, whereas serum immunoglobulin levels appear less reliable in advanced disease. These findings support the diagnostic value of immunophenotyping, particularly B-cell quantification and IP-10 measurement, for identifying GS in patients with TETs.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":" ","pages":""},"PeriodicalIF":4.1,"publicationDate":"2026-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148387599","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}