Fig. 2

Flow cytometric analysis

a: Dihydrorhodamine assay by flowcytometry showing normal oxidative burst by neutrophils There is a normal shift from baseline after stimulation in the patient compared to healthy control and the patient’s mother’s leucocytes. The Stimulation index of neutrophils after stimulation Vs. unstimulated cells were more than 100 in healthy control, mother, and patient.

b: Flow cytometric analysis of leukocyte adhesion molecules (CD11b, CD11c, CD18). Expression of CD11b, CD11c and Cd18 was absent (<1%) on granulocytes in case 1, and in case 2, there is absence (<1%) of CD11c and CD18 the patient?s granulocytes, while expression of CD11b was 74%