Flow Cytometric Assay of Respiratory Burst Capacity in Peripheral Blood Monocytes of Gaucher Type 1 Patients

Abstract

Abstract Background: There is an apparently increased tendency toward infections in patients with Gaucher disease, possibly due to defective neutrophil function rather than a decreased neutrophil count. Since macrophages are the main cell type affected in Gaucher disease, our aim was to determine the contribution of these cells to the susceptibility of Gaucher patients to infection by studying the respiratory burst capacity of peripheral blood monocytes. Methods: The study was performed in eleven Gaucher type 1 patients and eleven sex and age matched control subjects by measuring peripheral blood monocytes’ respiratory burst capacity using flow cytometry. The respiratory burst capacity was measured as dihydrorhodamine-123 median fluorescence in patients and respective controls. Results: There was no statistical difference in the median fluorescence among the patients and respective controls (p>0.05) after phorbol 12-myristate 13-acetate stimulation. Also, statistical difference was not reached among patients treated with enzyme replacement therapy at the time and those untreated. Conclusions: Flow cytometry might represent a more accurate and more reliable measure of respiratory burst compared to the methods of other researchers. Respiratory burst disturbance in monocytes does not seem to contribute to increased susceptibility to infection in Gaucher patients.