+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Severe impairment of regulatory T-cells and Th1-lymphocyte polarization in patients with Gaucher disease

Severe impairment of regulatory T-cells and Th1-lymphocyte polarization in patients with Gaucher disease

Jimd Reports 18: 107-115

We investigated peripheral blood T-lymphocyte subpopulations and intracellular expression of IFN-γ, IL-4, IL-10, and IL-13, by whole blood flow cytometry, in 22 type I Gaucher disease (GD) patients. Results were compared with those of 19 sex- and age-matched controls. Patients with GD exhibited decreased frequencies and absolute numbers of CD3+/CD4+ helper T lymphocytes (40.8 ± 9.8% vs. 49.4 ± 5.7%, p = 0.002, and 0.77 ± 0.33 vs. 1.04 ± 0.28 × 10(9)/μL, p = 0.011), as well as increased frequencies of CD3+CD8+ suppressor T lymphocytes (23.8 ± 8.0% vs. 18.4 ± 3.8%, p = 0.010), resulting in a significantly decreased CD4/CD8 cell ratio (p < 0.001). Moreover, they had significantly increased percentages of IFNγ-producing both CD4+ and CD8+ T cells (p = 0.0003 and p = 0.023, respectively), implying a TH-1 polarization pattern. Finally, patients with GD had decreased percentages and absolute numbers of CD4+CD25(dim) T lymphocytes (p = 0.033 and p = 0.007, respectively), of CD4+CD25(high) T lymphocytes (p = 0.039 and p = 0.016, respectively), and of CD4+CD25(high)FOXP3+ regulatory T cells (p = 0.036 and p = 0.019, respectively). Our results demonstrate that patients with GD have a significant numerical impairment of T-helper lymphocytes and a constitutive TH1 direction pattern of activation of both CD4+ and CD8+ cells, associated with a significant decrease of T-regs. Ineffective T-cell control may explain the chronic inflammatory reaction and the increased incidence of lymphoid malignancies, which have been repeatedly reported among patients with GD.

(PDF emailed within 0-6 h: $19.90)

Accession: 055739772

Download citation: RISBibTeXText

PMID: 25308560

DOI: 10.1007/8904_2014_357

Related references

Impairment of regulatory capacity of CD4+CD25+ regulatory T cells mediated by dendritic cell polarization and hyperthyroidism in Graves' disease. Journal of Immunology 186(8): 4734-4743, 2011

The Gaucher Registry Severe bone disease among patients with Gaucher disease in the absence of significant hematologic abnormalities. Blood. 96(11 Part 1): 8a, Ember 16, 2000

B- and T-Lymphocyte Attenuator Expression on Regulatory T-Cells in Patients with Severe Sepsis. Chinese Medical Journal 131(21): 2637-2639, 2018

Development of Gaucher's cells in bone marrow; cytological study of Gaucher's cells and pathogenesis of Gaucher's disease. Knjiga. Jugoslavenska Akademija Znanosti i Umjetnosti. Odjel Za Medicinske Nauke 284: 111-118, 2014

Exploring the patient journey to diagnosis of Gaucher disease from the perspective of 212 patients with Gaucher disease and 16 Gaucher expert physicians. Molecular Genetics and Metabolism 122(3): 122-129, 2017

Gaucher Cells In Healthy Relatives Of Patients With Gaucher's Disease. Lancet 2(7364): 866-866, 1964

Varied autopsy findings in five treated patients with Gaucher disease and parkinsonism include the absence of Gaucher cells. Molecular Genetics and Metabolism 118(1): 55-59, 2016

Atypical cytomorphology of Gaucher cells is frequently seen in bone marrow smears from untreated patients with Gaucher disease type 1. Folia Histochemica et Cytobiologica 53(1): 62-69, 2016

Clinical response to eliglustat in treatment-naïve patients with Gaucher disease type 1: Post-hoc comparison to imiglucerase-treated patients enrolled in the International Collaborative Gaucher Group Gaucher Registry. Molecular Genetics and Metabolism Reports 8: 17-19, 2016

Hyposmia and cognitive impairment in Gaucher disease patients and carriers. Movement Disorders 27(4): 526-532, 2012

Gammopathy and B lymphocyte clonality in patients with Gaucher type I disease. Blood Cells, Molecules & Diseases 50(3): 222-225, 2013

Coexistence of regulatory B cells and regulatory T cells in tumor-infiltrating lymphocyte aggregates is a prognostic factor in patients with breast cancer. Breast Cancer: -, 2018

Functional impairment of monocyte-derived dendritic cells in patients with severe chronic kidney disease. Nephrology, Dialysis, Transplantation 22(1): 128-138, 2006

Comparison between the biochemical properties of plasma chitotriosidase from normal individuals and from patients with Gaucher disease, GM1-gangliosidosis, Krabbe disease and heterozygotes for Gaucher disease. Clinical Biochemistry 40(5-6): 365-369, 2007

Enzyme replacement therapy reverses B lymphocyte and dendritic cell dysregulations in patients with Gaucher Disease. Blood Cells, Molecules & Diseases 68: 81-85, 2016