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PubMed · 7949815

Hyper-IgM immunodeficiency with disseminated cryptococcosis.

M Iseki·M Anzo·N Yamashita·N Matsuo
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Abstract

We describe two siblings with X-linked hyper-IgM immunodeficiency. One patient developed disseminated cryptococcosis. Co-culture of this patient's T cells with normal B cells suppressed IgG and IgA production. The CD40 ligand gene of one patient was examined and contained a nonsense mutation at nucleotide 475. CD40 ligand is a membrane protein which is expressed on activated T cells and induces B-cell proliferation. These results suggest that there is a defect in T- and B-cell interactions in this immunodeficiency syndrome. It is also possible that patients with this syndrome are predisposed to cryptococcal infections.

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Antigens, CDCD40 LigandCausalityChild, PreschoolCryptococcosisFatal OutcomeGenetic LinkageHumansHypergammaglobulinemiaImmunoglobulin AImmunoglobulin GImmunoglobulin MImmunologic Deficiency SyndromesInfantLigandsMaleMembrane GlycoproteinsMutationRecurrenceX ChromosomeCase ReportsJournal Article

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BibTeXRIS

M Iseki, M Anzo, N Yamashita, N Matsuo. 1994. Hyper-IgM immunodeficiency with disseminated cryptococcosis.. https://doi.org/10.1111/j.1651-2227.1994.tb13140.x

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