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Previous Volume 314:409-413 February 13, 1986 Number 7 Next

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Abstract

Immunodeficiency with hyperimmunoglobulinemia M is a syndrome characterized by normal to elevated serum levels of IgM and low levels or absence of IgG and IgA. The defect in this syndrome is thought to reside within the B lymphocyte, which may be unable to undergo a "switch" in immunoglobulin class from IgM to IgG or IgA. To address this question more directly, we cultured B cells from nine patients with this syndrome with pokeweed mitogen and either "switch" T cells or normal control T cells. In cultures with normal T cells, only IgM was secreted, whereas in cultures with switch T cells, IgG as well as IgM, or IgM, IgG, and IgA were secreted. Furthermore, analysis of the immunoglobulin heavy-chain genes in these B cells by means of genetic probes of constant and switch regions revealed normal gene patterns. These data suggest that B cells from patients with hyperimmunoglobulinemia M may not be abnormal, as previously proposed, and that, at least in some patients with this syndrome, a defect in switch T cells may be pathogenic.

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• Rosen, F. S., Cooper, M. D., Wedgwood, R. J.P. (1995). The Primary Immunodeficiencies. NEJM 333: 431-440 [Full Text]  
• Geha, R. S., Rosen, F. S. (1994). The Genetic Basis of Immunoglobulin-Class Switching. NEJM 330: 1008-1009 [Full Text]  
• Allen, R., Armitage, R., Conley, M., Rosenblatt, H, Jenkins, N., Copeland, N., Bedell, M., Edelhoff, S, Disteche, C., Simoneaux, D., et, al. (1993). CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome. Science 259: 990-993 [Abstract]