Severe infections are seen in ELANE and HAX1 mutations, thus alloHSCT is indicated in these patients, particularly if they require high-dose G-CSF to maintain their neutrophil count, or progress to MDS/AML (it has been found that patients who require more than 8 mcg/kg/day of G-CSF to maintain a neutrophil count above 0.5 × 109/L have an increased risk of sepsis and MDS/AML) (43, 46, 55). The gene discussed is ELANE; the disease is Sepsis.