This study is trying to evaluate the efficacy and safety of dose-modified Emapalumab and Ruxolitinib (E-Ru) regimens for the treatment of active hemophagocytic lymphohistiocytosis.
Fulfillment of at least five of the eight HLH-2004 criteria for HLH;
Age 1-70 years old;
No prior chemotherapy for HLH;
Confirmed not pregnant and willing to use effective contraceptive measures during the study period, with the last dose of medication administered at least 12 months prior;
According to the New York Heart Association (NYHA) score, patients with heart disease of grade II or above (including grade II);
HIV-infected patients;
Patients with severe renal dysfunction (glomerular filtration rate <15 mL/min);
Patients with severe liver cirrhosis (MELD score>20);