RESEARCH ARTICLE


Selective IgM Deficiency with T Cell Defects and Mycobacterium Avium Complex (MAC) Infection



Sudhir Gupta*, Sudhanshu Agrawal, Sastry Gollapudi
Medical Sciences I, C-240, University of California at Irvine, Irvine, CA 92-697, USA.


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© 2011 Gupta et al;

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the Medical Sciences I, C-240, University of California at Irvine, Irvine, CA 92-697, USA; Tel: (949) 824-5818; Fax: (949) 824-4362; E-mail: sgupta@uci.edu


Abstract

Primary selective IgM deficiency in adults is associated with normal T cell function, and patients clinically manifest with recurrent pyogenic bacterial infections. In this study, we present three patients with selective IgM deficiency with significant defects in T cells and NK cell cytotoxicity, and Mycobacterial avium intracellulare (MAC) infection. T cell defect is characterized by markedly reduced numbers of T cells and T cell subsets, decreased T cell proliferative responses to mitogens and antigens, including PPD, and significantly decreased production of IFN-γ; however, expression of IFN-γ receptors is normal. We propose that selective IgM deficiency with significant T cell functional defects might represent a distinct clinical entity, which is associated with increased susceptibility to MAC infection.

Keywords: IFN-γ, Deficiency,, T Cell Deficiency, NK cytotoxicity.