Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Evaluation of Specific Antibody Responses in Patients With Selective Iga Deficiency and Ataxia Telangiectasia Publisher Pubmed



Khanmohammadi S1, 2 ; Shad TM1, 2 ; Delavari S1, 2 ; Shirmast P1 ; Bagheri Y3 ; Azizi G4 ; Aghamohammadi A1 ; Abolhassani H1, 5 ; Yazdani R1, 6 ; Rezaei N1, 6
Authors
Show Affiliations
Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, Tehran, Iran
  2. 2. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  3. 3. Clinical Research Development Unit (CRDU), 5 Azar Hospital, Golestan University of Medical Sciences, Gorgan, Iran
  4. 4. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  5. 5. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
  6. 6. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran

Source: Endocrine# Metabolic and Immune Disorders - Drug Targets Published:2022


Abstract

Background: Specific Antibody Deficiency (SAD) is a primary immunodefi-ciency disease (PID) characterized by the occurrence of recurrent infections and inadequate antibody response to polysaccharide new antigens. Objective: This study aims to determine the titer of specific antibodies against unconju-gated 23-valent pneumococcal polysaccharide vaccine (PPSV-23), the presence of SAD, and its association with clinical and laboratory findings in Ataxia-telangiectasia (A-T) and selective immunoglobulin A deficiency (SIgAD) patients. Methods: 32 A-T patients and 43 SIgAD patients were included in this cross-sectional study. Samples of the patients were obtained before and three weeks after vaccination with PPSV-23. Specific immunoglobulin G (IgG) directed towards pneumococcal capsular antigen and specific antibodies against whole pneumococcal antigens was measured. Results: Comparison of the response to vaccination revealed that 81.3% of A-T patients and 18.6% of the SIgAD patients had an inadequate response to PPSV-23 (p<0.001). The prevalence of recurrent infection (p=0.034) and pneumonia (p=0.003) in SIgAD patients was significantly higher in non-responders than responders. Likewise, the number of marginal zone B cells (p=0.037), transitional B cells (p=0.019), plasmablasts (p=0.019), CD8+ naive T cells (p=0.036), and percentage of CD8+ T cells (p=0.047), switched memory B cells (SMB) (p=0.026) and immunoglobulin M (IgM) memory B cells (p=0.022) in SIgAD patients were significantly lower in non-responder group than responder group. In con-trast, the percentage of CD4 T+ cells in A-T patients was lower in the non-responder group than responders (p=0.035). Conclusion: SAD is more frequent in A-T patients than SIgAD patients. The role of SMB and T cells should not be underestimated in SAD. © 2022 Bentham Science Publishers.
Related Docs
View other Related Docs
1. Cutaneous Granulomatosis and Class Switching Defect As a Presenting Sign in Ataxia-Telangiectasia: First Case From the National Iranian Registry and Review of the Literature, Immunological Investigations (2020)
2. Variable Abnormalities in T and B Cell Subsets in Ataxia Telangiectasia, Journal of Clinical Immunology (2021)
3. The Spectrum of Atm Gene Mutations in Iranian Patients With Ataxia-Telangiectasia, Pediatric Allergy and Immunology (2021)
4. Ataxia-Telangiectasia: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Expert Review of Clinical Immunology (2020)
5. Clinical Phenotype Classification for Selective Immunoglobulin a Deficiency, Expert Review of Clinical Immunology (2015)
6. The Heterogeneous Pathogenesis of Selective Immunoglobulin a Deficiency, International Archives of Allergy and Immunology (2019)
7. B Cells and T Cells Abnormalities in Patients With Selective Iga Deficiency, Allergy# Asthma and Clinical Immunology (2023)
8. Selective Iga Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Scandinavian Journal of Immunology (2017)
Experts (# of related papers)
View all Related Experts
Nima Rezaei (39)
Gholamreza Hassanpour (8)
Other Related Docs
9. Effect of Class Switch Recombination Defect on the Phenotype of Ataxia-Telangiectasia Patients, Immunological Investigations (2021)
10. Atypical Ataxia Presentation in Variant Ataxia Telangiectasia: Iranian Case-Series and Review of the Literature, Frontiers in Immunology (2022)
11. Role of Apoptosis in Common Variable Immunodeficiency and Selective Immunoglobulin a Deficiency, Molecular Immunology (2016)
12. Genetic Risk Variants for Class Switching Recombination Defects in Ataxia-Telangiectasia Patients, Journal of Clinical Immunology (2022)
13. Atm Expression and Activation in Ataxia Telangiectasia Patients With and Without Class Switch Recombination Defects, Journal of Clinical Immunology (2025)
14. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders, Journal of Investigational Allergology and Clinical Immunology (2017)
15. Pulmonary Manifestations of Predominantly Antibody Deficiencies, Pulmonary Manifestations of Primary Immunodeficiency Diseases (2018)
16. Monogenic Mutations Associated With Iga Deficiency, Expert Review of Clinical Immunology (2016)
17. Autoimmunity in Patients With Selective Iga Deficiency, Journal of Investigational Allergology and Clinical Immunology (2015)
18. Primary Immunodeficiency Diseases in Iran: Past, Present and Future, Archives of Iranian Medicine (2021)
19. Primary Immunodeficiency and Thrombocytopenia, International Reviews of Immunology (2022)
20. The Clinical and Immunological Features of Patients With Primary Antibody Deficiencies, Endocrine# Metabolic and Immune Disorders - Drug Targets (2018)
21. Immunodeficiencies, Clinical Immunology (2022)
22. Inborn Errors of Immunity and Cancers, Cancer Immunology: A Translational Medicine Context# Second Edition (2020)
23. X-Linked Tlr7 Deficiency Underlies Critical Covid-19 Pneumonia in a Male Patient With Ataxia-Telangiectasia, Journal of Clinical Immunology (2022)
24. Autoimmunity in Primary Antibody Deficiencies, International Archives of Allergy and Immunology (2017)
25. Primary Immunodeficiencies and Cancers, Cancer Immunology: A Translational Medicine Context (2015)
26. Evaluation of Mir-210 Expression in Common Variable Immunodeficiency: Patients With Unsolved Genetic Defect, Allergologia et Immunopathologia (2021)
27. Application of Flow Cytometry in Predominantly Antibody Deficiencies, Endocrine# Metabolic and Immune Disorders - Drug Targets (2021)
28. Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment, Journal of Investigational Allergology and Clinical Immunology (2015)
29. The Clinical Significance of Complete Class Switching Defect in Ataxia Telangiectasia Patients, Expert Review of Clinical Immunology (2017)
30. Comprehensive Assessment of Skin Disorders in Patients With Common Variable Immunodeficiency (Cvid), Journal of Clinical Immunology (2022)
31. Potential Role of Regulatory B Cells in Immunological Diseases, Immunology Letters (2019)
32. Evaluation of Expression of Lrba and Ctla-4 Proteins in Common Variable Immunodeficiency Patients, Immunological Investigations (2022)
33. A Comparison of Clinical and Immunologic Phenotypes in Familial and Sporadic Forms of Common Variable Immunodeficiency, Scandinavian Journal of Immunology (2017)
34. Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis, Journal of Clinical Immunology (2025)
35. T Cell Repertoire Abnormality in Immunodeficiency Patients With Dna Repair and Methylation Defects, Journal of Clinical Immunology (2022)
36. Comparison of Various Classifications for Patients With Common Variable Immunodeficiency (Cvid) Using Measurement of B-Cell Subsets, Allergologia et Immunopathologia (2017)
37. Common Presentations and Diagnostic Approaches, Stiehm's Immune Deficiencies: Inborn Errors of Immunity (2020)
38. Autoimmune Manifestations Among 461 Patients With Monogenic Inborn Errors of Immunity, Pediatric Allergy and Immunology (2021)
39. Evaluation of Effective Factors on Il-10 Signaling in B Cells in Patients With Selective Iga Deficiency, European Cytokine Network (2021)
40. Ataxia-Telangiectasia: A Review of Clinical Features and Molecular Pathology, Pediatric Allergy and Immunology (2019)
41. Evaluation of Radiation Sensitivity in Patients With Hyper Igm Syndrome, Immunological Investigations (2020)
42. Autoimmunity in a Cohort of 471 Patients With Primary Antibody Deficiencies, Expert Review of Clinical Immunology (2017)
43. Clinical Complications and Their Management in a Child With Ataxia-Telangiectasia (A-T): A Case Report Study, Clinical Case Reports (2021)
44. Class Switch Recombination Process in Ataxia Telangiectasia Patients With Elevated Serum Levels of Igm, Journal of Immunoassay and Immunochemistry (2015)
45. Known and Potential Molecules Associated With Altered B Cell Development Leading to Predominantly Antibody Deficiencies, Pediatric Allergy and Immunology (2021)
46. Impact of Sars-Cov-2 Pandemic on Patients With Primary Immunodeficiency, Journal of Clinical Immunology (2021)
47. Evaluation of Respiratory Complications in Patients With X-Linked and Autosomal Recessive Agammaglobulinemia, Pediatric Allergy and Immunology (2020)
48. The First Cohort of Iranian Patients With Hyper Immunoglobulin E Syndrome: A Long-Term Follow-Up and Genetic Analysis, Pediatric Allergy and Immunology (2019)
49. Immunophenotypic and Functional Analysis of Lymphocyte Subsets in Common Variable Immunodeficiency Patients Without Monogenic Defects, Scandinavian Journal of Immunology (2022)
50. Ataxia Telangiectasia Syndrome: Moonlighting Atm, Expert Review of Clinical Immunology (2017)