Elevated levels of activated CD4 T cells in common variable immunodeficiency: association with clinical findings

2006 ◽  
Vol 34 (4) ◽  
pp. 131-135 ◽  
Author(s):  
J. Carbone ◽  
E. Sarmiento ◽  
D. Micheloud ◽  
J. Rodríguez-Molina ◽  
E. Fernández-Cruz
2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Jan Stuchlý ◽  
Veronika Kanderová ◽  
Marcela Vlková ◽  
Ivana Heřmanová ◽  
Lucie Slámová ◽  
...  

Abstract Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B- and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. A hierarchical clustering of probability binning “bins” yielded a separate cluster of 22 CVID patients with an abnormal phenotype. We showed coordinated proportional changes in naïve CD4+ T-cells (decreased), intermediate CD27− CD28+ CD4+ T-cells (increased) and CD21low B-cells (increased) that were stable for over three years. Moreover, the lymphocytes’ immunophenotype in this patient cluster exhibited features of profound immunosenescence and chronic activation. Thrombocytopenia was only found in this cluster (36% of cases, manifested as Immune Thrombocytopenia (ITP) or Evans syndrome). Clinical complications more frequently found in these patients include lung fibrosis (in 59% of cases) and bronchiectasis (55%). The degree of severity of these symptoms corresponded to more deviation from normal levels with respect to CD21low B-cells, naïve CD4+ and CD27− CD28+ CD4+ T-cells. Next-generation sequencing did not reveal any common genetic background. We delineate a subgroup of CVID patients with activated and immunosenescent immunophenotype of lymphocytes and distinct set of clinical complications without common genetic background.


2008 ◽  
Vol 30 (4) ◽  
pp. 679-686 ◽  
Author(s):  
Giovanni Carlo Del Vecchio ◽  
Baldassarre Martire ◽  
Giuseppe Lassandro ◽  
Valerio Cecinati ◽  
Delia De Mattia ◽  
...  

2014 ◽  
Vol 211 (10) ◽  
pp. 2033-2045 ◽  
Author(s):  
Matthieu Perreau ◽  
Selena Vigano ◽  
Florence Bellanger ◽  
Céline Pellaton ◽  
Guillaume Buss ◽  
...  

In the present study, we have investigated the functional profile of CD4 T cells from patients with common variable immunodeficiency (CVID), including production of cytokines and proliferation in response to bacteria and virus-derived antigens. We show that the functional impairment of CD4 T cells, including the reduced capacity to proliferate and to produce IFN-γ and IL-2, was restricted to bacteria-specific and not virus-specific CD4 T cells. High levels of endotoxins were found in the plasma of patients with CVID, suggesting that CD4 T cell dysfunction might be caused by bacterial translocation. Of note, endotoxemia was associated with significantly higher expression of programmed death 1 (PD-1) on CD4 T cells. The blockade of the PD-1–PD-L1/2 axis in vitro restored CD4 T cell proliferation capacity, thus indicating that PD-1 signaling negatively regulates CD4 T cell functions. Finally, we showed that intravenous immunoglobulin G (IVIG) treatment significantly reduced endotoxemia and the percentage of PD-1+ CD4 T cells, and restored bacteria-specific CD4 T cell cytokine production and proliferation. In conclusion, the present study demonstrates that the CD4 T cell exhaustion and functional impairment observed in CVID patients is associated with bacterial translocation and that IVIG treatment resolves bacterial translocation and restores CD4 T cell functions.


2016 ◽  
Vol 166-167 ◽  
pp. 19-26 ◽  
Author(s):  
Laura Berrón-Ruiz ◽  
Gabriela López-Herrera ◽  
Alexander Vargas-Hernández ◽  
Leopoldo Santos-Argumedo ◽  
Constantino López-Macías ◽  
...  

2017 ◽  
Vol 180 ◽  
pp. 80-83 ◽  
Author(s):  
Karina Mescouto de Melo ◽  
Susanne Unger ◽  
Baerbel Keller ◽  
Sylvia Gutenberger ◽  
Ina Stumpf ◽  
...  

2009 ◽  
Vol 156 (3) ◽  
pp. 446-454 ◽  
Author(s):  
J. Horn ◽  
A. Manguiat ◽  
L. J. Berglund ◽  
V. Knerr ◽  
F. Tahami ◽  
...  

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