combined with sepsis was 0.841 (P＜0.001, 95%CI 0.750-0.932), and there was no statistical significance in AU-ROC compared with pSOFA(Z =0.406, P=0.6845> 0.05). In 36 non-survival and 58 survival, non-survival group had more significant reduction of Hb and PLT, more serious damage of organ functions, and higher incidence of SF＞4500 ng/ mL. The incidence of sepsis was higher in the non-survival group (52.8%, 19/36) than in the survival group (8.6%, 5/58) (P<0.05). Multivariate analysis showed that sepsis, PLT< 50×10⁹/L and aPTT>48.5s were risk factors for adverse prognosis of HLH, and AU-ROC was 0.891 (95%CI: 0.822-0.961). CONCLUSIONS:The combination of Hb < 60g/L, elevated PCT and SF > 4500 ng/ml is helpful to judge HLH with sepsis. Combined sepsis, platelet level < 50×10⁹/L and aPTT > 48.5s were independent risk factors for predicting poor prognosis of HLH.