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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
|
pubmed:dateCreated |
1997-1-7
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pubmed:abstractText |
We report the occurrence of X-linked lymphoproliferative disease (XLP) in two brothers in a Malaysian family. In this disorder, a primary Epstein-Barr virus (EBV) infection is followed by an abnormal proliferation of transformed B-cells that cannot be controlled by suppressor T-cells, leading to the development of deranged immune function. This results in fatal infectious mononucleosis, acquired hypogammaglobulinaemia, virus-infected haemophagocytic syndrome and non-Hodgkin's lymphoma. The diagnosis should be considered when there is a family history of any male having a fulminant course of infectious mononucleosis, an otherwise benign disease. Early diagnosis is important as bone marrow transplantation is the only curative option in this disorder.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
|
pubmed:status |
MEDLINE
|
pubmed:month |
Jun
|
pubmed:issn |
0037-5675
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pubmed:author | |
pubmed:issnType |
Print
|
pubmed:volume |
37
|
pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
325-7
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pubmed:dateRevised |
2005-11-16
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pubmed:meshHeading |
pubmed-meshheading:8942243-Child, Preschool,
pubmed-meshheading:8942243-Diagnosis, Differential,
pubmed-meshheading:8942243-Fatal Outcome,
pubmed-meshheading:8942243-Humans,
pubmed-meshheading:8942243-Infectious Mononucleosis,
pubmed-meshheading:8942243-Lymphoproliferative Disorders,
pubmed-meshheading:8942243-Male
|
pubmed:year |
1996
|
pubmed:articleTitle |
Two brothers in a Malaysian family with X-linked lymphoproliferative disease--a case report.
|
pubmed:affiliation |
Department of Paediatrics, University Hospital, Kuala Lumpur, Malaysia.
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pubmed:publicationType |
Journal Article,
Review,
Case Reports
|