Introduction:

Chronic granulomatous disease (CGD) is classically known as phagocytic system disease. However, in recent years, the observation of the development of autoimmunity, colitis-like findings during follow-up has suggested that the acquired immune system may also be defective. The aim of this project is to investigate the percent of T and B lymphocytes, and function of T lymphocytes in patients with CGD and carriers.

Material and Methods:

Eleven patients followed for CGD (3 X-CGD, 8 OR-CGD) in our clinic and 6 carrier mothers of OR-CGD were included into the study. The percentages of lymphocytes and their subtypes, lymphocyte proliferation, regulator T cell (Treg), intracellular cytokine content of patients with CDG and carriers were determined in comparison to healthy controls.

Results:

There was no statistically significant difference in percentages of T cell, B cell and NK cell and subtypes of T and B cells in the mothers of patients compared to age matched healthy controls. The percentage of nau00efve B cells was significantly higher in the patients with CGD compared to those of healthy subjects (p<0.001). There were no significant differences in lymphocyte proliferation, Treg cell percentage, intracellularIL-17 levels, but IFN-u03b3 levels were significantly increased in both patients with CDG (p=0.030) and carriers (p=0.038) when compared with age-matched healthy controls.

Conclusion:

Increased naive B cells were found in CGD patients. High levels of IFN-u03b3 in patients and carriers need to be further evaluated by further studies. In addition, the normal percentages of Treg and IL-21 in the carriers as in healthy subjects may be offered as the protective mechanism for autoimmunity.

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