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

Tometten, Inga; Felgentreff, Kerstin; Hoenig, Manfred; Hauck, Fabian; Albert, Michael H.; Niehues, Tim; Perez, Ruy; Ghosh, Sujal; Picard, Capucine; Stary, Jan; Formankova, Renata; Worth, Austen; Soler-Palacin, Pere; Garcia-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 and Rohr, Jan C. (2019): Increased proportions of gamma delta T lymphocytes in atypical SCID associate with disease manifestations. In: Clinical Immunology, Vol. 201: pp. 30-34

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 gamma delta T lymphocytes have been reported. However, it is unknown whether these gamma delta T cells modulate or reflect the patient's clinical phenotype. We investigated the frequency of elevated gamma delta T cell proportions and associations with clinical disease manifestations in a cohort of 76 atypical SCID patients. Increased proportions of gamma delta T lymphocytes were present in approximately 600/0 of these patients. Furthermore, we identified positive correlations between elevated proportions of gamma delta T cells and the occurrence of CMV infections and autoimmune cytopenias. We discuss that CMV infections might trigger an expansion of gamma delta T lymphocytes, which could drive the development of autoimmune cytopenias. We advocate that atypical SCID patients should be screened for elevated proportions of gamma delta T lymphocytes, CMV infection and autoimmune cytopenias.

• Item Type: Journal article
• Faculties: Medicine
• Subjects: 600 Technology > 610 Medicine and health
• ISSN: 1521-6616
• Language: English