Considering the possible role of the polymorphisms of cell adhesion molecules, chemokines, and transglutaminase in DS pathogenesis, the aim of this study was to investigate the association of rs1024611 in CCL2, rs5498 in ICAM-1, and rs7270785 and rs4811528 in TGM2 with the development of differentiation syndrome in patients treated with ATRA/ATO. Here, TGM2 is linked to Dravet syndrome.