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Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency).

Abstract:

X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in hemophagocytic lymphohistiocytosis (HLH). XLP type 1 (XLP-1) is caused by mutations in the gene SH2D1A (also named SAP), whereas mutations in the gene XIAP underlie XLP type 2 (XLP-2). Here, a comparison of the clinical phenotypes associated with XLP-1 and XLP-2 was performed in cohorts of 33 and 30 patients, respe...

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Publication status:
Published

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Journal:
Blood More from this journal
Volume:
117
Issue:
5
Pages:
1522-1529
Publication date:
2011-02-01
DOI:
EISSN:
1528-0020
ISSN:
0006-4971
Language:
English
Keywords:
Pubs id:
pubs:120367
UUID:
uuid:fba28f77-7006-4833-a92b-6cab58974ff1
Local pid:
pubs:120367
Source identifiers:
120367
Deposit date:
2012-12-19

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