In vitro studies of monocyte function in two siblings with Papillon-Lefèvre syndrome

Blood monocytes were isolated from two siblings with Papillon-Lefèvre syndrome (PLs) and compared to corresponding cells from their healthy cousin. The number of monocytes isolated were within normal limits in all three test participants. Aggregating tendency was increased when PLs monocytes were cultured in the presence of autologous sera. The monocyte ability of specific immune phagocytosis was decreased in PLs patients. The monocyte morphology, non-specific phagocytosis, lysosomal enzyme activities, and response to E. coli endotoxin were similar in patients and control.