Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations

Publication date: Available online 15 February 2019

Source: Clinical Immunology

Author(s): Inga Tometten, Kerstin Felgentreff, Manfred Hönig, Fabian Hauck, Michael H. Albert, Tim Niehues, Ruy Perez, Sujal Ghosh, Capucine Picard, Jan Stary, Renata Formankova, Austen Worth, Pere Soler-Palacín, Marina García-Prat, Luis M. Allende, Luis Ignacio Gonzalez-Granado, Polina Stepensky, Silvia Di Cesare, Alessia Scarselli, Caterina Cancrini

Abstract

Severe combined immunodeficiencies (SCID) comprise a group of genetic diseases characterized by abrogated development of T lymphocytes. In some case reports of atypical SCID patients elevated proportions of γδ T lymphocytes have been reported. However, it is unknown whether these γδ T cells modulate or reflect the patient's clinical phenotype. We investigated the frequency of elevated γδ T cell proportions and associations with clinical disease manifestations in a cohort of 76 atypical SCID patients. Increased proportions of γδ T lymphocytes were present in approximately 60% of these patients. Furthermore, we identified positive correlations between elevated proportions of γδ T cells and the occurrence of CMV infections and autoimmune cytopenias. We discuss that CMV infections might trigger an expansion of γδ T lymphocytes, which could drive the development of autoimmune cytopenias. We advocate that atypical SCID patients should be screened for elevated proportions of γδ T lymphocytes, CMV infection and autoimmune cytopenias.

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