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

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 CancriniCarsten Speckmann, Kimberly Gilmour, Luigi Notarangelo, Stephan Ehl, Jan C Rohr

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)30-34
Number of pages5
JournalClinical Immunology
Volume201
DOIs
Publication statusPublished - Apr 2019

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T-Lymphocytes
Infection
Severe Combined Immunodeficiency
Inborn Genetic Diseases
Atypical Severe combined immunodeficiency
Phenotype

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Tometten, I., Felgentreff, K., Hönig, M., Hauck, F., Albert, M. H., Niehues, T., ... Rohr, J. C. (2019). Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations. Clinical Immunology, 201, 30-34. https://doi.org/10.1016/j.clim.2018.11.006

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

In: Clinical Immunology, Vol. 201, 04.2019, p. 30-34.

Research output: Contribution to journalArticle

Tometten, I, Felgentreff, K, Hönig, M, Hauck, F, Albert, MH, Niehues, T, Perez, R, Ghosh, S, Picard, C, Stary, J, Formankova, R, Worth, A, Soler-Palacín, P, García-Prat, M, Allende, LM, Gonzalez-Granado, LI, Stepensky, P, Di Cesare, S, Scarselli, A, Cancrini, C, Speckmann, C, Gilmour, K, Notarangelo, L, Ehl, S & Rohr, JC 2019, 'Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations', Clinical Immunology, vol. 201, pp. 30-34. https://doi.org/10.1016/j.clim.2018.11.006
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. / Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations. In: Clinical Immunology. 2019 ; Vol. 201. pp. 30-34.
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AU - Tometten, Inga

AU - Felgentreff, Kerstin

AU - Hönig, Manfred

AU - Hauck, Fabian

AU - Albert, Michael H

AU - Niehues, Tim

AU - Perez, Ruy

AU - Ghosh, Sujal

AU - Picard, Capucine

AU - Stary, Jan

AU - Formankova, Renata

AU - Worth, Austen

AU - Soler-Palacín, Pere

AU - García-Prat, Marina

AU - Allende, Luis M

AU - Gonzalez-Granado, Luis Ignacio

AU - Stepensky, Polina

AU - Di Cesare, Silvia

AU - Scarselli, Alessia

AU - Cancrini, Caterina

AU - Speckmann, Carsten

AU - Gilmour, Kimberly

AU - Notarangelo, Luigi

AU - Ehl, Stephan

AU - Rohr, Jan C

N1 - Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.

PY - 2019/4

Y1 - 2019/4

N2 - 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.

AB - 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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