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hyper-IgM syndrome

 

Definition: an X-linked immunodeficiency disorder with very low serum concentrations of IgG and IgA with a normal or a markedly elevated concentration of polyclonal IgM; secondary to a mutation in the CD40 ligand gene, leading to defective T cell–dependent, B-cell isotope switching affected boys develop recurrent bacterial infections in the first or second years of life.

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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