{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T22:13:27Z","timestamp":1791152007778,"version":"4.1.0"},"reference-count":44,"publisher":"American Society of Hematology","issue":"3","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2004,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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 with 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 with controls. Oligoclonal T-cell receptor (TCR) V\u03b2 families and missing V\u03b2 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. (Blood. 2004;103:1020-1025)<\/jats:p>","DOI":"10.1182\/blood-2003-08-2824","type":"journal-article","created":{"date-parts":[[2003,10,7]],"date-time":"2003-10-07T00:16:34Z","timestamp":1065485794000},"page":"1020-1025","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":126,"title":["T-cell homeostasis in humans with thymic hypoplasia due to chromosome 22q11.2 deletion syndrome"],"prefix":"10.1182","volume":"103","author":[{"given":"Lisa M.","family":"Piliero","sequence":"first","affiliation":[{"name":"From The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy N.","family":"Sanford","sequence":"additional","affiliation":[{"name":"From The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna M.","family":"McDonald-McGinn","sequence":"additional","affiliation":[{"name":"From The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine H.","family":"Zackai","sequence":"additional","affiliation":[{"name":"From The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen E.","family":"Sullivan","sequence":"additional","affiliation":[{"name":"From The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2020021222160771100_REF1","unstructured":"Driscoll DA, Budarf ML, Emanuel BS. 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