DEP Combine With PD-1 Antibody as an Treatment for EBV-HLH

DEP Combine With PD-1 Antibody as an Treatment for EBV Associated Hemophagocytic Lymphohistiocytosis (HLH)

This study aimed to investigate the efficacy and safety of DEP (liposomal doxorubicin, etoposide and methylprednisolone) together with PD-1 antibody as an treatment for EBV associated hemophagocytic lymphohistiocytosis.

PD-1 antibody added to the DEP regimen （with or without asparaginases） in EBV-HLH,

Inclusion Criteria:

1. Meet hemophagocytic lymphohistiocytosis (HLH)-04 diagnostic criteria; EBV-DNA in peripheral blood or EBER in tissue were positive, patients were diagnosed with EBV associated HLH (EBV-HLH).
2. . The expected survival time is more than 1 month.
3. Age >18 years old, gender is not limited.
4. Serum creatinine ≤ 1.5 times normal；
5. Serum human immunodeficiency virus(HIV) antigen or antibody negative； Hepatitis C virus (HCV) antibody is negative, or HCV antibody is positive, but HCV RNA is negative；HBV copies less than 1E+03 copies/ml.
6. No thyroid dysfunction. The left ventricular ejection fraction (LVEF) was normal.
7. No uncontrollable infection.
8. Contraception for both male or female.

8. Informed consent obtained.

Exclusion Criteria:

1. Allergic to doxorubicin, etoposide and sintilimab Injection
2. Serious immunoreaction: myocardial damage, hepatitis, pneumonia
3. Central nervous system symptoms
4. Serious mental illness;
5. Central nervous system symptoms
6. Serious mental illness;
7. Accumulated dose of doxorubicin above 300mg/m2 or epirubicin above 450mg/m2；
8. Pancreatitis history. Patients unable to comply during the trial and/or follow-up phase;
9. Participate in other clinical research at the same time.