Recurrent Severe Infections Caused by a Novel Leukocyte Adhesion Deficiency

THE recruitment of neutrophils to sites of inflammation is initiated by the local production of bacteria-derived attractants, inflammatory cytokines, and other host-derived factors. These factors induce the rolling of neutrophils on the blood-vessel wall,^{1 2 3 4} followed by firm adhesion and extravasation into the surrounding infected or inflamed tissue.^{2 3 4} In recent years, there have been rapid advances in identifying the specific adhesion molecules that mediate the process of neutrophil recruitment.^{1 , 5 , 6} The initial rolling of neutrophils is mediated by members of the selectin family.^{2 , 4 , 7 , 8} These include E-selectin and P-selectin, which are expressed on the surface of activated endothelial cells, and L-selectin, which is…