title: Syndrome of selective IgM deficiency with severe T cell deficiency associated with disseminated cutaneous mycobacterium avium intracellulaire infection.
abstract: Cutaneous non-disseminated, non-tuberculous mycobacterial infections have been reported in both immunocompetent and immunocompromised subjects. Systemic Mycobacterium avium intracellulaire (MAI) have been reported in non-HIV patients with Idiopathic CD4 lymphocytopenia. We report a comprehensive immunological analysis in syndrome of selective IgM deficiency and T lymphocytopenia (both CD4+ and CD8+) with disseminated cutaneous MAI infection. Naïve (TN) and Central memory (TCM) subsets of both CD4+ and CD8+ T cells were decreased, whereas terminally differentiated effector memory (TEMRA) subset of CD4+ and CD8+ T cells were markedly increased. IFN-γ producing T cells were markedly decreased. Although CD14(high)CD16- proinflammatory monocytes were modestly increased, IFN-γR+ monocytes were markedly decreased. The expression of TLR3, TLR5, TLR7, and TLR9 on monocytes was decreased. Germinal center B cells (CD19+IgD-CD38+CD27(lo)) and B1 cells (CD20+CD27+CD43+CD70-) were markedly decreased. A role of immune alterations, including B cells and antibodies in disseminated cutaneous MAI infection is discussed.
authors.affiliation: Division of Basic and Clinical Immunology, University of California Irvine.
authors.forename: Asal
authors.initials: A
authors.lastname: Gharib
authors.affiliation: Division of Basic and Clinical Immunology, University of California Irvine.
authors.forename: Ankmalika Gupta
authors.initials: AG
authors.lastname: Louis
authors.affiliation: Division of Basic and Clinical Immunology, University of California Irvine.
authors.forename: Sudhanshu
authors.initials: S
authors.lastname: Agrawal
authors.affiliation: Division of Basic and Clinical Immunology, University of California Irvine.
authors.forename: Sudhir
authors.initials: S
authors.lastname: Gupta
keywords: B1 cells; IFN-γ; TLR; germinal center B cells; memory T cells