Journal of Clinical Immunology

, Volume 31, Issue 6, pp 927–935

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)

Authors

  • Abbas F. Jawad
    • Children’s Hospital of Philadelphia
  • Eline Luning Prak
    • Department of Pathology and Laboratory MedicineUniversity of Pennsylvania
  • Jean Boyer
    • Marlene and Stewart Greenebaum Cancer CenterUniversity of Pennsylvania School of Medicine
  • Donna M. McDonald-McGinn
    • Children’s Hospital of Philadelphia
  • Elaine Zackai
    • Children’s Hospital of Philadelphia
  • Kenyetta McDonald
    • Children’s Hospital of Philadelphia
    • Children’s Hospital of Philadelphia
    • Division of Allergy ImmunologyChildren’s Hospital of Philadelphia
Article

DOI: 10.1007/s10875-011-9569-8

Cite this article as:
Jawad, A.F., Prak, E.L., Boyer, J. et al. J Clin Immunol (2011) 31: 927. doi:10.1007/s10875-011-9569-8

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.

Keywords

DiGeorge syndromehomeostatic proliferationT cell senescenceB cell differentiationinfluenzavaccine

Supplementary material

10875_2011_9569_MOESM1_ESM.pdf (16 kb)
Supplemental Table IDemographic characteristics of subjects (PDF 15 kb)
10875_2011_9569_MOESM2_ESM.pdf (26 kb)
Supplemental Table IIB cell subsets (PDF 25.6 kb)
10875_2011_9569_MOESM3_ESM.pdf (27 kb)
Supplemental Data Fig. 1T 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)
10875_2011_9569_MOESM4_ESM.pdf (699 kb)
Supplemental Data Fig. 2B 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)
10875_2011_9569_MOESM5_ESM.pdf (67 kb)
Supplemental Data Fig. 3T 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)

Copyright information

© Springer Science+Business Media, LLC 2011