Linkage analysis has shown that the alteration of the ELANE gene (also referred to as ELA2, HLE, or NE) is localized in the chromosome 19p13.3, in the exon 4 or 5 of the gene, or at the junction of exon 4 and intron 4, determining aberrant function with alteration in the development of the myeloid precursor, as well as cell loss and neutropenia [1,3,4,8]. The gene discussed is ELANE; the disease is Decreased total neutrophil count.