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A Prospective Study of Influenza Vaccination and a Comparison of Immunologic Parameters in Children and Adults with Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome/Velocardiofacial Syndrome)

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Abstract

Prior to the advent of cardiac bypass, most children with congenital cardiac anomalies and chromosome 22q11.2 deletion syndrome died. With improved technology, there is now a wave of young adults with chromosome 22q11.2 deletion syndrome requiring clinical care. Fifteen young children and 20 adults with chromosome 22q11.2 deletion had flow cytometry, functional T cell analyses, and functional B cell analyses to characterize their immune system. Subjects were vaccinated with the annual inactivated influenza vaccine, and responses were evaluated by hemagglutination inhibition titer assessment. The pattern of T cell subset abnormalities was markedly different between pediatric and adult patients. In spite of the cellular deficits observed in adults, titers produced after influenza vaccine administration were largely intact. We conclude that disruption to T cell production appears to have secondary consequences for T cell differentiation and B cell function although the clinical impact remains to be determined.

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Acknowledgments

This study was supported by NIH grant NO1-AI-50024. The authors acknowledge the recruiting assistance of Tiketta McIntyre and Ximena Rivera Cuellar.

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Authors and Affiliations

  1. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Abbas F. Jawad, Donna M. McDonald-McGinn, Elaine Zackai, Kenyetta McDonald & Kathleen E. Sullivan

  2. Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Eline Luning Prak

  3. Marlene and Stewart Greenebaum Cancer Center, University of Pennsylvania School of Medicine, Philadelphia, PA, USA

    Jean Boyer

  4. Division of Allergy Immunology, Children’s Hospital of Philadelphia, 3615 Civic Center Blvd., Philadelphia, PA, 19104, USA

    Kathleen E. Sullivan

Authors
  1. Abbas F. Jawad
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  2. Eline Luning Prak
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  3. Jean Boyer
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  4. Donna M. McDonald-McGinn
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  5. Elaine Zackai
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  6. Kenyetta McDonald
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  7. Kathleen E. Sullivan
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Corresponding author

Correspondence to Kathleen E. Sullivan.

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Supplemental Table I

Demographic characteristics of subjects (PDF 15 kb)

Supplemental Table II

B cell subsets (PDF 25.6 kb)

Supplemental Data Fig. 1

T cell responses in subjects. Peripheral blood mononuclear cells were stimulated with PMA and ionomycin to define the overall competence of memory T cells for γ-interferon production. The patients and controls do not differ significantly. Means and standard deviations are shown (PDF 26 kb)

Supplemental Data Fig. 2

B cell subsets were defined by flow cytometry. The absolute counts were obtained by multiplying the subpopulation by the CD19+ B cell count. These stacked bar graphs demonstrate the variability from person to person and define the absolute B cell counts for each population (PDF 698 kb)

Supplemental Data Fig. 3

T cell responses to influenza. Samples obtained 1–2 months after vaccination were studied for responses to influenza antigens. T cell proliferation was measured by CFSE after stimulation with intact virus (upper panel). The fraction of cells responding (% Divided) and the Proliferation Index (PI) were defined using FlowJo. The proliferation index is defined as the average number of divisions that the responding cells underwent. Means and standard deviations are shown. There were no differences between the groups. The lower panel indicates the responses to influenza antigens in an ELISPOT analysis. Means and standard deviations are shown. There are no differences between groups (PDF 67 kb)

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Jawad, A.F., Prak, E.L., Boyer, J. et al. A Prospective Study of Influenza Vaccination and a Comparison of Immunologic Parameters in Children and Adults with Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome/Velocardiofacial Syndrome). J Clin Immunol 31, 927–935 (2011). https://doi.org/10.1007/s10875-011-9569-8

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  • DOI: https://doi.org/10.1007/s10875-011-9569-8

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