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Blood, 1 February 2004, Vol. 103, No. 3, pp. 1020-1025. Prepublished online as a Blood First Edition Paper on October 2, 2003; DOI 10.1182/blood-2003-08-2824. This Article Full Text (PDF) All Versions of this Article: 2003-08-2824v1 103/3/1020    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Piliero, L. M Articles by Sullivan, K. E Search for Related Content PubMed PubMed Citation Articles by Piliero, L. M Articles by Sullivan, K. E Social Bookmarking                   What's this? Submitted August 15, 2003 Accepted September 28, 2003 T-cell homeostasis in humans with thymic hypoplasia due to chromosome 22q11.2 deletion syndrome Lisa M Piliero, Amy N Sanford, Donna M McDonald-McGinn, Elaine H Zackai, and Kathleen E Sullivan* Department of Allergy and Immunology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA * Corresponding author; email: sullivak{at}mail.med.upenn.edu. Patients with chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome) typically exhibit thymic hypoplasia, conotruncal cardiac defects, and hypoparathyroidism. The immunodeficiency that results from the thymic hypoplasia has been extensively described and consists primarily of T-cell lymphopenia. A curious feature of the T-cell lymphopenia is that the age-related rate of decline of T-cell numbers is slower in patients than controls. This leads to T-cell numbers in adulthood that are minimally decreased compared to controls. This suggests that homeostatic mechanisms might be acting to preserve the peripheral blood T-cell numbers in patients. We characterized changes in CD4/CD45RA and CD4/CD45RO T-cell populations in patients and controls of various ages and determined T-cell recombination excision circles and telomere length within the CD4/CD45RA population. Patients had evidence of accelerated conversion of naive to memory cells and had evidence of more extensive replicative history within the CD4/CD45RA compartment compared to controls. Oligoclonal TCR V families and missing V families were seen more often in patients than controls. These data are consistent with homeostatic proliferation of T cells in patients with limited T-cell production due to thymic hypoplasia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: K. Lore, R. Seggewiss, F. J. Guenaga, S. Pittaluga, R. E. Donahue, A. Krouse, M. E. Metzger, R. A. Koup, C. Reilly, D. C. Douek, et al. In Vitro Culture During Retroviral Transduction Improves Thymic Repopulation and Output After Total Body Irradiation and Autologous Peripheral Blood Progenitor Cell Transplantation in Rhesus Macaques Stem Cells, June 1, 2006; 24(6): 1539 - 1548. [Abstract] [Full Text] [PDF]

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