The LYST gene is mutated in autosomal recessive mode in inborn errors of immunity syndromes and, in particular, in familial hemophagocytic lymphohistiocytosis (FHL) syndromes with hypopigmentation: Chediak–Higashi syndrome and hemophagocytic lymphohistiocytosis (HLH) [26]. Here, LYST is linked to Familial hemophagocytic lymphohistiocytosis.