Title:Evaluation of Specific Antibody Responses in Patients with Selective IgA
Deficiency and Ataxia Telangiectasia
Volume: 22
Issue: 6
Author(s): Shaghayegh Khanmohammadi, Tannaz Moeini Shad, Samaneh Delavari, Paniz Shirmast, Yasser Bagheri, Gholamreza Azizi, Asghar Aghamohammadi, Hassan Abolhassani, Reza Yazdani and Nima Rezaei*
Affiliation:
- Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, Tehran, Iran
- Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
Keywords:
Primary immunodeficiency, inborn errors of immunity, Ataxia telangiectasia, selective IgA deficiency, specific antibody deficiency, 23-valent pneumococcal polysaccharide vaccine, PPSV-23.
Abstract:
Background: Specific Antibody Deficiency (SAD) is a primary immunodeficiency
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 unconjugated
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+
naïve 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 contrast,
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.
