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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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Detalles Bibliográficos
Autor principal: Al-Muhsen, Saleh Z.
Formato: Texto
Lenguaje:English
Publicado: Medknow Publications 2010
Materias:
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.
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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
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