Author/Authors :
Mohsenzadegan، Monireh نويسنده , , Fattahi، Fahimeh نويسنده Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. Fattahi, Fahimeh , Fattahi، Fatemeh نويسنده , , Mirshafiey، Abbas نويسنده , , FAZLOLLAHI، MOHAMMAD REZA نويسنده , , Naderi Beni، Fariba نويسنده Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran. Naderi Beni, Fariba , MOVAHEDI، MASOUD نويسنده , , Pourpak، Zahra نويسنده ,
Abstract :
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder characterized by a greatly increased susceptibility to severe fungal and bacterial infections caused by defects in NADPH oxidase of phagocytic cells. We aimed to investigate immunophenotype alterations of naïve and memory B cells and B1a cells in peripheral whole blood from Iranian patients with CGD.
Flow cytometric analysis was performed on peripheral blood samples from 31 CGD patients and 23 healthy controls (HC) to study naïve (IgD+/CD27-), memory (CD27+) B and B1a (CD5+) cells. Soluble CD27 (sCD27) and immunoglobulins were also measured by ELISA and the nephelometric method, respectively.
We found significantly higher levels of naïve B cells and B1a cells but lower levels of memory B cells in CGD patients compared to HC. There was no significant difference in soluble CD27 (sCD27) alteration between CGD patients and HC.
Our findings suggested a role for NADPH oxidase in process of B cell differentiation and impairing conversion of naïve B cells to memory B cells and altered B1a cells in CGD patients. Increased susceptibility of CGD patients to opportunistic infections and autoimmune disorders could be partly explained by the altered phenotype of B lymphocytes in these patients.