Selective IgM Deficiency with T Cell Defects and Mycobacterium Avium Complex (MAC) Infection
Sudhir Gupta*, Sudhanshu Agrawal, Sastry Gollapudi
Identifiers and Pagination:Year: 2012
First Page: 8
Last Page: 12
Publisher Id: TOIJ-5-8
Article History:Received Date: 11/11/2011
Revision Received Date: 15/1/2012
Acceptance Date: 16/1/2012
Electronic publication date: 22/3/2012
Collection year: 2012
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.
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.