Four different inborn errors of immunity causing HLH with hypopigmentation/albinism have been described: Chediak-Higashi syndrome, genetically characterized by mutations in the lysosomal trafficking regulator (LYST) gene (50, 51); Griscelli syndrome type 2 due to mutations in Ras-Related Protein Rab-27A (RAB27A) (52); and Hermansky-Pudlak syndrome type 2 and type 10 caused by mutations in the adaptor related protein complex 3 beta 1 (AP3B1) (53) and adaptor related protein complex 3 delta 1 (AP3D1) (54), respectively. This evidence concerns the gene LYST and Chediak-Higashi syndrome.