Interestingly, besides the constant neutropenia and lymphopenia, all 8 patients present monocytopenia and half of the patients present profound monocytopenia below 0.1 G/L, contrasting with the monocytosis commonly observed in other congenital neutropenias, such as the elastase neutrophil expressed (ELANE) syndrome. This evidence concerns the gene ELANE and Decreased total monocyte count.