A higher chronic expansion of effector cytotoxic CD8(+)DR(+) T-lymphocytes has been reported in common variable immunodeficiency (CVID) patients with complications such as splenomegaly, autoimmune disease and/or granulomatous disease. In order to document the features associated with this T cell activation involving the CD8(+) T-compartment, we examined the diversity of the alpha/beta TCR repertoire of the patient's CD8(+) T-lymphocytes using the qualitative analysis of the CDR3 lengths (Immunoscope). Ten CIVD patients were enrolled in this study, four without complications (Group 1), six with complications (Group 2). All patients exhibited non-gaussian altered CDR3 length distributions, albeit to different extent within the different Vβ families. CVID patients with activated CD8(+) T-cells show a reduction of their TCR repertoire diversity which is more severe in patients with complications. Viral reactivations such as CMV are suspected to be part of the mechanisms underlying immunosenescence.
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http://dx.doi.org/10.1016/j.rinim.2013.05.004 | DOI Listing |
Infect Dis Rep
December 2024
Department of Medicine, University of Wisconsin-Madison, Madison, WI 53706, USA.
Background/objectives: Common Variable Immunodeficiency Disease (CVID) and other immunodeficiencies can present in subtle and variable ways. Whether or not a genetic lesion can be identified, there are not well understood biomarkers that quantitatively describe how severe a deficiency is. Here we discuss two possible ranking systems, CD4/CD8 T cell ratios and Immune Health Grades, and how such data maybe applicable to some immunodeficiencies.
View Article and Find Full Text PDFThromb Haemost
December 2024
McMaster University, Hamilton, Canada.
The McMaster Immune Thrombocytopenia (ITP) Summit was an educational seminar from leading experts in immune thrombocytopenia and related disorders geared towards hematologists, internists, immunologists, and clinical and translational scientists. The focus of the Summit was to review the mechanisms, diagnosis and treatment of primary versus secondary ITP. Specific objectives were to describe the unique features of secondary ITP, and to review its mechanisms in the context of autoimmune disease and infection.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.
While Trisomy X syndrome is typically characterized by developmental and cognitive variations, it is not commonly associated with immunodeficiencies. We report the unique case of a 6-year-old girl with Trisomy X presenting with selective IgA deficiency, challenging the conventional understanding of this chromosomal condition. The patient exhibited recurrent respiratory infections and gastrointestinal symptoms, evaluated in the context of her genetic background of Trisomy X and significantly low levels of IgA (0.
View Article and Find Full Text PDFActa Neurol Belg
December 2024
Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland.
Immunol Res
December 2024
Department of Immunology, North Bristol NHS Trust, Bristol, UK.
CVID (common variable immunodeficiency) is associated with a variety of gastrointestinal disorders including those mimicking Crohn's disease and ulcerative colitis. At present there is no clear trial data for the treatment of CVID enteropathy. There are no specific recommendations for treatment; however, it is commonly treated in a similar manner to inflammatory bowel disease, with corticosteroids, 5-aminosalicylates (5-ASA), azathioprine and anti-TNF therapy all being used.
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