Background and Aims: Patients with Hyper-IgE syndrome suffer from fungal and bacterial infections, especially Candida albicans and Staphylococcus aureus. Due to the important role of T helper17 (Th17) lymphocytes in defense against fungal infections, the percentage of Th17 lymphocytes was studied in the patients with autosomal recessive hyper-IgE syndrome (AR-HIES).

Materials and Methods: In this case-control study, six patients with AR-HIES (with DOCK-8 mutation) and seven healthy age and sex-matched controls were included. Peripheral blood mononuclear cells were isolated from their venous blood and the percentage of Th17 lymphocytes were determined by flow cytometry.

Results: There was no statistical difference between the percentage of Th17 lymphocytes (p=0.15) in the case and control groups. Also in comparison to the control subjects, the numbers of eosinophils were dramatically increased (p=0.000). Also, there was a significant negative correlation between serum IgE levels and Th17 lymphocytes’ percentage (r=-0.927, p=0.006) and a significant positive correlation between eosinophils number and Th17 lymphocytes’ percentage (r=0.557, p=0.01). Serum IgE levels showed a significant positive correlation with the numbers of eosinophils in the patients’ peripheral blood with AR-HIES (r=0.961, p=0.003).

Conclusions: The numbers of Th17 in the patients with AR-HIES may not show statistical differences between the cases and controls. The numbers of eosinophils significantly increased in the patients AR-HIES compare to the controls.