Clinical immunology (Orlando, Fla.) · 2019
Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations.
СтатьяResearch Support, N.I.H., IntramuralПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Tometten Inga, Felgentreff Kerstin, Hönig Manfred, Hauck Fabian, Albert Michael H, Niehues Tim, Perez Ruy, Ghosh Sujal, Picard Capucine, Stary Jan, Formankova Renata, Worth Austen, Soler-Palacín Pere, García-Prat Marina, Allende Luis M, Gonzalez-Granado Luis Ignacio, Stepensky Polina, Di Cesare Silvia, Scarselli Alessia, Cancrini Caterina, Speckmann Carsten, Gilmour Kimberly, Notarangelo Luigi, Ehl Stephan, Rohr Jan C
Аннотация
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.