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Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment
Severe combined immunodeficiency (SCID) is a primary immunodeficiency disorder with heterogenous genetic etiologies. We describe a typical case in a 9-year-old boy that was masked by a clinically functional maternal T cell engraftment leading to late presentation with Pneumocystis jiroveci pneumonia...
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Formato: | Texto |
Lenguaje: | English |
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Medknow Publications
2010
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2886877/ https://www.ncbi.nlm.nih.gov/pubmed/20427943 http://dx.doi.org/10.4103/0256-4947.62834 |
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author | Al-Muhsen, Saleh Z. |
author_facet | Al-Muhsen, Saleh Z. |
author_sort | Al-Muhsen, Saleh Z. |
collection | PubMed |
description | Severe combined immunodeficiency (SCID) is a primary immunodeficiency disorder with heterogenous genetic etiologies. We describe a typical case in a 9-year-old boy that was masked by a clinically functional maternal T cell engraftment leading to late presentation with Pneumocystis jiroveci pneumonia and cytomegalovirus infection, probably following exhaustion of maternally engrafted cells. Based on immunological findings, he had a T- B+SCID phenotype. This report suggests that in rare cases, engrafted maternal T cell might persist for long time leading to partial constitution of immune function and delayed clinical presentation of SCID. |
format | Text |
id | pubmed-2886877 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2010 |
publisher | Medknow Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-28868772010-07-02 Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment Al-Muhsen, Saleh Z. Ann Saudi Med Case Report Severe combined immunodeficiency (SCID) is a primary immunodeficiency disorder with heterogenous genetic etiologies. We describe a typical case in a 9-year-old boy that was masked by a clinically functional maternal T cell engraftment leading to late presentation with Pneumocystis jiroveci pneumonia and cytomegalovirus infection, probably following exhaustion of maternally engrafted cells. Based on immunological findings, he had a T- B+SCID phenotype. This report suggests that in rare cases, engrafted maternal T cell might persist for long time leading to partial constitution of immune function and delayed clinical presentation of SCID. Medknow Publications 2010 /pmc/articles/PMC2886877/ /pubmed/20427943 http://dx.doi.org/10.4103/0256-4947.62834 Text en © Annals of Saudi Medicine http://creativecommons.org/licenses/by/2.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Al-Muhsen, Saleh Z. Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title | Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title_full | Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title_fullStr | Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title_full_unstemmed | Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title_short | Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment |
title_sort | delayed presentation of severe combined immunodeficiency due to prolonged maternal t cell engraftment |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2886877/ https://www.ncbi.nlm.nih.gov/pubmed/20427943 http://dx.doi.org/10.4103/0256-4947.62834 |
work_keys_str_mv | AT almuhsensalehz delayedpresentationofseverecombinedimmunodeficiencyduetoprolongedmaternaltcellengraftment |