Investigating serum free light chains in patients with common variable immunodeficiency disorder in compare with other immunodeficiency diseases.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος: Investigating serum free light chains in patients with common variable immunodeficiency disorder in compare with other immunodeficiency diseases.
Συγγραφείς: Bemanian MH; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Khoshmirsafa M; Immunology Research Center, Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran.; Department of immunology, school of medicine, Iran University of Medical Sciences, Tehran, Iran., Ahmadi M; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran. mina.ahmadi65@yahoo.com., Nabavi M; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Arshi S; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Chavoshzadeh Z; Department Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran., Shokri S; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Fallahpour M; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Jafariaghdam N; Immunology Research Center, Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran., Afrashteh F; Immunology Research Center, Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran., Asefi S; Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran., Mesdaghi M; Department Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran.; Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran., Sharafian S; Department Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Πηγή: Scientific reports [Sci Rep] 2026 Mar 13; Vol. 16 (1). Date of Electronic Publication: 2026 Mar 13.
Τύπος έκδοσης: Journal Article; Comparative Study
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
Imprint Name(s): Original Publication: London : Nature Publishing Group, copyright 2011-
Ιατρικοί όροι (MeSH): Common Variable Immunodeficiency*/blood , Common Variable Immunodeficiency*/diagnosis , Common Variable Immunodeficiency*/immunology , Immunoglobulin kappa-Chains*/blood , Immunoglobulin lambda-Chains*/blood , Immunoglobulin Light Chains*/blood , Immunologic Deficiency Syndromes*/blood, Agammaglobulinemia/blood ; Agammaglobulinemia/diagnosis ; Biomarkers/blood ; Humans ; Female ; Male ; Adult ; Cross-Sectional Studies ; Middle Aged ; Young Adult ; Adolescent ; Child
Περίληψη: Serum free light chains (sFLCs) have recently been introduced as a diagnostic biomarker in common variable immunodeficiency (CVID) patients. Most patients with CVID have undetectable or lower levels of sFLCs compared to people with other types of immune system deficiencies with hypogammaglobulinemia, except for patients with agammaglobulinemia (AGG). Decreased production of kappa (κ) and lambda (λ) light chains over immunoglobulins (Ig) suggests a malfunction in early B cell development or plasma cell dysfunction may cause CVID. In this cross-sectional study, immunoturbidimetry was used to measure sFLCs in 70 immunodeficiency patients, including 39 patients with CVID, 31 patients with other immunodeficiencies with hypogammaglobulinemia such as combined immunodeficiency (CID), AGG, and other primary immunodeficiencies (PIDs), and 20 healthy controls. The levels of both κ and λ chains were significantly higher in patients with CID than patients with CVID and AGG patients (p < 0.05). The median (Q1–Q3) κ level in patients with other primary immunodeficiency disorders (PIDs) was significantly higher than that observed in patients with AGG and CVID (p < 0.01). Similarly, the median (Q1–Q3) λ level was higher in patients with other PIDs compared with those with AGG and CVID (p < 0.05).ROC analysis of κ and λ disclosed an area under the curve (AUC) for κ was 0.98 (95% CI: 0.96–0.99, p < 0.0001) and for λ was 0.95 (95% CI: 0.89–0.99, p < 0.0001) in patients with CVID. The sFLCs test can help distinguish between CVID and other immunodeficiency patients with hypogammaglobulinemia and the diagnosis of hypogammaglobulinemia.
Competing Interests: Declarations. Competing interests: The authors declare no competing interests.New loaders + training/eval loop (CrossEntropyLoss) Ethics approval: The present study was conducted according to the principles expressed in the Helsinki Declaration and the ethics committee of the Iran University of Medical Sciences (Ethical code: IR.IUMS.FMD.REC.1401.396. Consent to participate: Informed consent for participation was obtained from all patients and their legal guardians included in the study.
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Grant Information: 1401-3-103-24229 This work was supported by Iran University of Medical Sciences
Contributed Indexing: Keywords: Agammaglobulinemia; CVID; Common variable immunodeficiency; Kappa light chain; Lambda light chain; Serum free light chain
Substance Nomenclature: 0 (Biomarkers)
0 (Immunoglobulin kappa-Chains)
0 (Immunoglobulin lambda-Chains)
0 (Immunoglobulin Light Chains)
Entry Date(s): Date Created: 20260314 Date Completed: 20260627 Latest Revision: 20260627
Update Code: 20260627
PubMed Central ID: PMC13111665
DOI: 10.1038/s41598-026-41057-6
PMID: 41826376
Βάση Δεδομένων: MEDLINE
Περιγραφή
ISSN:2045-2322
DOI:10.1038/s41598-026-41057-6