The Effects of Stimulation With Pma/Ionomycin on Cd4+ T Cell Proliferation and Surface Cd4 Molecule Modulation of Patients With Lrba Deficiency and Cvid With the Unsolved Genetic Defect Publisher Pubmed



Salami F^{1, 2} ; Shariati S^{1, 2} ; Rasouli SE³ ; Delavari S^{1, 2} ; Tavakol M³ ; Sadri H³ ; Asghari B⁴ ; Yazdani R^{1, 2} ; Rezaei N^{1, 2, 5} ; Abolhassani H⁶ ; Azizi G³ Show Affiliations
Source: Endocrine# Metabolic and Immune Disorders - Drug Targets Published:2022

Abstract

Background: Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiencies. LPS-responsive beige-like anchor protein (LRBA) deficiency is a combined immunodeficiency characterized by a CVID-like phenotype. Affected patients by LRBA and CVID present a wide range of clinical manifestations, including hypogammaglobulinemia, recurrent infections, autoimmunity, as well as T cell abnormality. Methods: The study population comprised of patients with CVID (n=10), LRBA deficiency (n=11), and healthy controls (n=12). CD4+ T cell frequency and CD4 MFI (mean fluorescence intensity) were evaluated using flow cytometry before and after stimulation with PMA/ION. Results: The frequencies of CD4+ T cells were significantly lower in patients with LRBA deficiency than in HCs before and after treatment. In the unstimulated state, the CD4+ T cells frequency in CVID patients was significantly lower than in HCs. There were no statistically significant differences between patients and healthy individuals in CD4+ T cell proliferation. Compared to HCs, LRBA and CVID patients showed a lower CD4 MFI in unstimulated conditions. Furthermore, CD4 MFI decreased in both patients and the control group following activation. Conclusion: Despite the reported decrease in CD4+ T cell frequency in patients with CVID and LRBA deficiency, our findings demonstrated that their CD4+ T cells have a normal proliferative response to stimuli similar to healthy individuals. © 2022 Bentham Science Publishers.