This study aimed to investigate the efficacy and safety of ruxolitinib together with liposomal doxorubicin, etoposide and methylprednisolone (DEP-Ru) as a salvage therapy for refractory/relapsed hemophagocytic lymphohistiocytosis.

Inclusion Criteria:

1. meet HLH-04 diagnostic criteria;
2. treated with HLH-94 no less than 2 weeks before enrollment and did not achieve at least PR; or relapsed patients after remission；
3. Life expectancy exceeds 1 month;
4. Age≥1 year old and ≤70 years old, gender is not limited；
5. Before the start of the study, total bilirubin ≤ 10 times the upper limit of normal; serum creatinine ≤ 1.5 times normal；
6. Serum HIV antigen or antibody negative；
7. HCV antibody is negative, or HCV antibody is positive, but HCV RNA is negative；
8. Both HBV surface antigen and HBV core antibody were negative. If any of the above is positive, peripheral blood hepatitis B virus DNA titer should be detected, and less than 1×103 copies/ml can enter the group；
9. Echocardiographic examination of LVEF ≥ 50%;
10. Informed consent.

Exclusion Criteria:

1. Heart function above grade II (NYHA)；
2. Accumulated dose of doxorubicin above 300mg/m2 or epirubicin above 450mg/m2；
3. Pregnancy or lactating Women；
4. Allergic to Pegaspargase, doxorubicin or etoposide；
5. Active bleeding of the internal organs；
6. uncontrollable infection；
7. Serious mental illness;
8. Non-melanoma skin cancer history;
9. Patients unable to comply during the trial and/or follow-up phase;
10. Participate in other clinical research at the same time.