characterizing t cells in scid patients presenting with reactive or residual t lymphocytes

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2012
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Abstract
Introduction. Patients with severe combined immunodeficiency (SCID) may present with residual circulating T cells. While all cells are functionally deficient, resulting in high susceptibility to infections, only some of these cells are causing autoimmune symptoms. Methods. Here we compared T-cell functions including the number of circulating CD3+ T cells, in vitro responses to mitogens, T-cell receptor (TCR) repertoire, TCR excision circles (TREC) levels, and regulatory T cells (Tregs) enumeration in several immunodeficinecy subtypes, clinically presenting with nonreactive residual cells (MHC-II deficiency) or reactive cells. The latter includes patients with autoreactive clonal expanded T cell and patients with alloreactive transplacentally maternal T cells. Results. MHC-II deficient patients had slightly reduced T-cell function, normal TRECs, TCR repertoires, and normal Tregs enumeration. In contrast, patients with reactive T cells exhibited poor T-cell differentiation and activity. While the autoreactive cells displayed significantly reduced Tregs numbers, the alloreactive transplacentally acquired maternal lymphocytes had high functional Tregs. Conclusion. SCID patients presenting with circulating T cells show different patterns of T-cell activity and regulatory T cells enumeration that dictates the immunodeficient and autoimmune manifestations. We suggest that a high-tolerance capacity of the alloreactive transplacentally acquired maternal lymphocytes represents a toleration advantage, yet still associated with severe immunodeficiency.
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lev2012clinicalcharacterizing Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Atar Lev, Amos J. Simon, Luba Trakhtenbrot, Itamar Goldstein, Meital Nagar, Polina Stepensky, Gideon Rechavi, Ninette Amariglio, Raz Somech
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Journal Applied Surface Science
Year 2012
DOI
10.1155/2012/261470
URL
http://dx.doi.org/10.1155/2012/261470
Keywords
immunologic diseases allergy

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