Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes
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 fu...
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2012-01-01
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| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1155/2012/261470 |
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| author | Atar Lev Amos J. Simon Luba Trakhtenbrot Itamar Goldstein Meital Nagar Polina Stepensky Gideon Rechavi Ninette Amariglio Raz Somech |
| author_facet | Atar Lev Amos J. Simon Luba Trakhtenbrot Itamar Goldstein Meital Nagar Polina Stepensky Gideon Rechavi Ninette Amariglio Raz Somech |
| author_sort | Atar Lev |
| collection | DOAJ |
| description | 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. |
| format | Article |
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| institution | OA Journals |
| issn | 1740-2522 1740-2530 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
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| series | Clinical and Developmental Immunology |
| spelling | doaj-art-40d4d0d0e6ff41589c8f71b5b69f31d52025-08-20T02:07:51ZengWileyClinical and Developmental Immunology1740-25221740-25302012-01-01201210.1155/2012/261470261470Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T LymphocytesAtar Lev0Amos J. Simon1Luba Trakhtenbrot2Itamar Goldstein3Meital Nagar4Polina Stepensky5Gideon Rechavi6Ninette Amariglio7Raz Somech8Cancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelDepartment of Pediatric Hematology-Oncology, Hadassah Medical Center, Hadassah Hebrew University, 91120 Jerusalem, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelCancer Research Center, Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, IsraelIntroduction. 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.http://dx.doi.org/10.1155/2012/261470 |
| spellingShingle | Atar Lev Amos J. Simon Luba Trakhtenbrot Itamar Goldstein Meital Nagar Polina Stepensky Gideon Rechavi Ninette Amariglio Raz Somech Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes Clinical and Developmental Immunology |
| title | Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes |
| title_full | Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes |
| title_fullStr | Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes |
| title_full_unstemmed | Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes |
| title_short | Characterizing T Cells in SCID Patients Presenting with Reactive or Residual T Lymphocytes |
| title_sort | characterizing t cells in scid patients presenting with reactive or residual t lymphocytes |
| url | http://dx.doi.org/10.1155/2012/261470 |
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