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Clin Immunol. 2019 Feb 15;201:30-34. doi: 10.1016/j.clim.2018.11.006. [Epub ahead of print]

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

Author information

1
Center for Chronic Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
2
Department of Pediatrics and Adolescent Medicine, Ulm University, Ulm, Germany.
3
Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, LMU, Munich, Germany.
4
HELIOS Children's Hospital Krefeld, Pediatric Immunology and Rheumatology, Krefeld, Germany.
5
Department of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Center of Child and Adolescent Health, Heinrich-Heine-University, Dusseldorf, Germany.
6
Paris Descartes-Sorbonne Paris Cité University, Imagine Institute Paris, Paris, France; Paediatric Haematology-Immunology and Rheumatology Unit, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France; Center for Primary Immunodeficiencies, Necker-Enfants Malades Hospital, APHP, Paris, France; Laboratory of Lymphocyte Activation and Susceptibility to EBV Infection, INSERM UMR 1163, Imagine Institute, University Paris Descartes Sorbonne Paris Cité, Paris, France.
7
Department of Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic.
8
Great Ormond Street Hospital NHS Trust, London, United Kingdom.
9
Pediatric Infectious Diseases and Immunodeficiencies Unit (UPIIP), Hospital Universitari Vall d'Hebron (HUVH), Vall d'Hebron Research Institute (VHIR), Universitat Autònoma de Barcelona (UAB), Barcelona, Spain; Jeffrey Model Foundation Excellence Center, Barcelona, Spain.
10
Immunology Department, Hospital Universitario 12 de Octubre, Research Institute (i+12). Madrid, Spain.
11
Immunodeficiencies Unit, Department of Pediatrics, University Hospital 12 de Octubre, Research Institute Hospital 12 Octubre (i+12), Madrid, Spain; Complutense University of Madrid, Madrid, Spain.
12
Department of Bone Marrow Transplantation, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
13
University Department of Pediatrics, Unit of Immune and Infectious Diseases, Children's Hospital Bambino Gesù, Rome, Italy.
14
University Department of Pediatrics, Unit of Immune and Infectious Diseases, Children's Hospital Bambino Gesù, Rome, Italy; Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
15
Center for Chronic Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Center for Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
16
Laboratory of Clinical Immunology and Microbiology, LCIM, National Institute of Allergy and Infectious Diseases, NIAID, National Institutes of Health, NIH, Bethesda, MD, USA.
17
Center for Chronic Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Center for Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address: jan.rohr@uniklinik-freiburg.de.

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.

KEYWORDS:

Atypical SCID; Autoimmune cytopenia; CMV infection; Lymphopenia; Viral infection; γδ T lymphocytes

PMID:
30776520
DOI:
10.1016/j.clim.2018.11.006

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