Source:http://linkedlifedata.com/resource/pubmed/id/18458578
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
5
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pubmed:dateCreated |
2008-5-9
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pubmed:abstractText |
Maternal engraftment of T cells in severe combined immunodeficiency can lead to graft-versus-host disease of the skin and liver. We report the case of an infant with X-linked severe combined immunodeficiency, confirmed by DNA sequencing of the common gamma chain gene locus, in which this disorder's characteristic peripheral lymphocyte phenotype [T(-)B(+)NK(-)] was obscured by the postnatal onset of hemophagocytic syndrome that included severe B-cell lymphopenia, neutropenia, and anemia. Hemophagocytosis was most likely owing to maternal graft-versus-host disease, as perforin-expressing CD8 T cells, presumably of maternal origin, were prominent in the bone marrow and there was no concurrent severe infection.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
1077-4114
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
30
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
396-400
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pubmed:dateRevised |
2011-10-6
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pubmed:meshHeading |
pubmed-meshheading:18458578-Bone Marrow,
pubmed-meshheading:18458578-Humans,
pubmed-meshheading:18458578-Immunoglobulin gamma-Chains,
pubmed-meshheading:18458578-Infant,
pubmed-meshheading:18458578-Lymphohistiocytosis, Hemophagocytic,
pubmed-meshheading:18458578-Lymphoma, B-Cell,
pubmed-meshheading:18458578-Male,
pubmed-meshheading:18458578-Severe Combined Immunodeficiency,
pubmed-meshheading:18458578-T-Lymphocytes,
pubmed-meshheading:18458578-Treatment Outcome,
pubmed-meshheading:18458578-X-Linked Combined Immunodeficiency Diseases
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pubmed:year |
2008
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pubmed:articleTitle |
Maternal T-cell engraftment associated with severe hemophagocytosis of the bone marrow in untreated X-linked severe combined immunodeficiency.
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pubmed:affiliation |
Division of Pediatric Stem Cell Transplantation, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA. dvorakc@peds.ucsf.edu
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pubmed:publicationType |
Journal Article,
Case Reports,
Research Support, Non-U.S. Gov't
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