Role of Apoptosis in the Pathogenesis of Common Variable Immunodeficiency (CVID)

Title:Role of Apoptosis in the Pathogenesis of Common Variable Immunodeficiency (CVID)

Volume: 17 Issue: 4

Author(s): Mazdak Ganjalikhani-Hakemi, Reza Yazdani*, Mohammad Esmaeili, Hassan Abolhassani, Wiliam Rae, Gholamreza Azizi, Majid Z. dizaji, Mohammadreza Shaghaghi, Abbas Rezaei, Faezeh Abbasi-Rad, Sanaz Afshar-Qasemloo, Saeed Mohammadi, Nima Rezaei and Asghar Aghamohammadi*

Affiliation:

• Children’s Medical Center Hospital, 62 Qarib St., Keshavarz Blvd., Tehran14194,,Iran

• Children’s Medical Center Hospital, 62 Qarib St., Keshavarz Blvd., Tehran14194,,Iran

Keywords: Common variable immunodeficiency, early apoptosis, late apoptosis, B cell subsets, anti-apoptotic, BCL-2.

Abstract: Background: Common variable immunodeficiency (CVID) is a heterogeneous immune deficiency characterized by hypogammaglobulinemia. Since B cell maturation and differentiation is defective in this disorder, we evaluated apoptosis in B cells of patients with CVID compared with healthy donors (HD).

Methods: Determination of peripheral blood B-cell subsets in CVID and HDs, was performed using flow cytometry. We compared total apoptosis, early apoptosis and late apoptosis/necrosis in unstimulated and stimulated B-cells of patients with CVID and HDs. We also assessed the expression of the anti-apoptotic molecule BCL2 mRNA levels in B-cells by real-time PCR in CVID patients compared with HDs.

Results: Total B-cell apoptosis was increased in both unstimulated and stimulated B-cells from CVID patients compared with HDs (p=0.02 and p=0.004). Early apoptosis in stimulated B-cells (p=0.04) and late apoptosis/necrosis of B-cells in both unstimulated and stimulated B-cells (p=0.04 and p=0.03, respectively) were significantly higher in CVID patients compared with HDs. There was a significant inverse correlation between the percentages of post germinal center B-cells in the peripheral blood of CVID patients compared with percentage of apoptotic B-cells. However, anti-apoptotic BCL2 expression was not significantly reduced in B-cells from CVID patients compared with HDs (p=0.16).

Conclusion: Increased apoptosis of B-cells may be a factor in abnormality of differentiated B-cell subsets and the impaired endogenous immunoglobulin production in CVID patients. Further studies of the expression of pro/anti-apoptotic mediators in B-cells of CVID patients may shed light on the mechanism behind this increased B-cell apoptosis, and present potential therapeutic interventions in the future.

Cite this article as:

Ganjalikhani-Hakemi Mazdak, Yazdani Reza*, Esmaeili Mohammad, Abolhassani Hassan, Rae Wiliam, Azizi Gholamreza, dizaji Z. Majid, Shaghaghi Mohammadreza, Rezaei Abbas , Abbasi-Rad Faezeh , Afshar-Qasemloo Sanaz, Mohammadi Saeed, Rezaei Nima and Aghamohammadi Asghar*, Role of Apoptosis in the Pathogenesis of Common Variable Immunodeficiency (CVID), Endocrine, Metabolic & Immune Disorders - Drug Targets 2017; 17 (4) . https://dx.doi.org/10.2174/1871530317666170919120245

DOI https://dx.doi.org/10.2174/1871530317666170919120245	Print ISSN 1871-5303
Publisher Name Bentham Science Publisher	Online ISSN 2212-3873