A novel thymoma-associated immunodeficiency with increased naive T cells and reduced CD247 expression

The Journal of Immunology : Official Journal of the American Association of Immunologists
Petros ChristopoulosPaul Fisch

Abstract

The mechanisms underlying thymoma-associated immunodeficiency are largely unknown, and the significance of increased blood γδ Τ cells often remains elusive. In this study we address these questions based on an index patient with thymoma, chronic visceral leishmaniasis, myasthenia gravis, and a marked increase of rare γδ T cell subsets in the peripheral blood. This patient showed cutaneous anergy, even though he had normal numbers of peripheral blood total lymphocytes as well as CD4(+) and CD8(+) T cells. Despite his chronic infection, analyses of immunophenotypes and spectratyping of his lymphocytes revealed an unusual accumulation of naive γδ and αβ T cells, suggesting a generalized T cell activation defect. Functional studies in vitro demonstrated substantially diminished IL-2 and IFN-γ production following TCR stimulation of his "untouched" naive CD4(+) T cells. Biochemical analysis revealed that his γδ and αβ T cells carried an altered TCR complex with reduced amounts of the ζ-chain (CD247). No mutations were found in the CD247 gene that encodes the homodimeric ζ protein. The diminished presence of CD247 and increased numbers of γδ T cells were also observed in thymocyte populations obtained from three other thymoma patient...Continue Reading

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Citations

Sep 18, 2020·Cells·Lena GaissmaierPetros Christopoulos
Oct 4, 2015·The Journal of Immunology : Official Journal of the American Association of Immunologists·James S Welsh, Steven P Howard
Oct 4, 2015·The Journal of Immunology : Official Journal of the American Association of Immunologists·Paul FischWolfgang W A Schamel
Jan 9, 2021·Immunology Letters·Kissy Guevara-HoyerSilvia Sánchez-Ramón
Mar 7, 2021·Virchows Archiv : an International Journal of Pathology·Alexander MarxYosuke Yamada
Jun 3, 2021·International Journal of Molecular Sciences·Mariam ElshiatyPetros Christopoulos
Jul 2, 2021·Frontiers in Neurology·Victoria LeopardiOliver A Garden

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