Taking the CC genotype at rs479333 of the NLRC4 gene as a reference, after adjusting the age, gender, BMI, history of hypertension, diabetes, coronary heart disease, and history of EBV infection, HLH patients with CG genotype and GG genotype had a higher risk of death (RR = 3.475, 95% CI: 1.488-5.775, p = 0.003; RR = 2.986, 95% CI: 1.014-5.570, p = 0.047). Here, NLRC4 is linked to hemophagocytic syndrome.