Journal of Clinical Immunology

, Volume 31, Issue 6, pp 927-935

First online:

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)

  • Abbas F. JawadAffiliated withChildren’s Hospital of Philadelphia
  • , Eline Luning PrakAffiliated withDepartment of Pathology and Laboratory Medicine, University of Pennsylvania
  • , Jean BoyerAffiliated withMarlene and Stewart Greenebaum Cancer Center, University of Pennsylvania School of Medicine
  • , Donna M. McDonald-McGinnAffiliated withChildren’s Hospital of Philadelphia
  • , Elaine ZackaiAffiliated withChildren’s Hospital of Philadelphia
  • , Kenyetta McDonaldAffiliated withChildren’s Hospital of Philadelphia
  • , Kathleen E. SullivanAffiliated withChildren’s Hospital of PhiladelphiaDivision of Allergy Immunology, Children’s Hospital of Philadelphia Email author 

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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.


DiGeorge syndrome homeostatic proliferation T cell senescence B cell differentiation influenza vaccine