HLH is a hyper-inflammatory response activated by macrophages often seen in advanced ENKTL, with has high interleukin-18 (IL-18) concentrations associated with the inflammatory storm.[5,6] ENKTL-HLH has high mortality and lacks long-term follow-up, there is no standard treatment for it, so it is meaningful to explore the strategies of ENKTL-HLH. The gene discussed is IL18; the disease is hemophagocytic syndrome.