%0 Journal Article
%T Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment
%A Al-Muhsen Saleh
%J Annals of Saudi Medicine
%D 2010
%I King Faisal Specialist Hospital and Research Centre
%X 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.
%U http://www.saudiannals.net/article.asp?issn=0256-4947;year=2010;volume=30;issue=3;spage=239;epage=242;aulast=Al-Muhsen