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Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis.


ABSTRACT: Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, genetically heterogeneous autosomal recessive immune disorder that results when the critical regulatory pathways that mediate immune defense mechanisms and the natural termination of immune/inflammatory responses are disrupted or overwhelmed. To advance the understanding of FHL, we performed gene expression profiling of peripheral blood mononuclear cells from 11 children with untreated FHL. Total RNA was isolated and gene expression levels were determined using microarray analysis. Comparisons between patients with FHL and normal pediatric controls (n = 30) identified 915 down-regulated and 550 up-regulated genes with more than or equal to 2.5-fold difference in expression (P ≤ .05). The expression of genes associated with natura

SUBMITTER: Sumegi J 

PROVIDER: S-EPMC3087540 | biostudies-literature | 2011 Apr

REPOSITORIES: biostudies-literature

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