Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations.
Clin Immunol
; 201: 30-34, 2019 04.
Article
en En
| MEDLINE
| ID: mdl-30776520
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.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Inmunodeficiencia Combinada Grave
/
Infecciones por Citomegalovirus
/
Linfocitos Intraepiteliales
/
Enfermedades Hematológicas
Tipo de estudio:
Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
Clin Immunol
Asunto de la revista:
ALERGIA E IMUNOLOGIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Alemania