Hemophagocytic lymphohistiocytosis
Conditions
Interventions
Treatment group:mDEP + PD-1 Blockade + Venetoclax
Sponsors
The First Affiliated Hospital of Soochow University
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Only patients who meet all the following inclusion criteria are eligible to be included in this study: 1) According to the HLH-04 diagnostic criteria, they meet the HLH diagnostic criteria and are diagnosed as EBV-related HLH in the etiological diagnosis. 2) EBV infection is indicated by a significant increase in EBV-DNA copies in peripheral blood or the detection of EBV-encoded small RNA (EBER) in tissues. 3) After initial treatment including HLH-94, the patients have not achieved at least partial remission. 4) The expected survival period is more than 1 month. 5) Before the start of the study, the serum creatinine is= 0.6 g/L through infusion. 6) Serum HIV antibody is negative; HCV antibody is negative, or HCV antibody is positive but HCV RNA is negative. The HBV surface antigen and HBV core antibody are both negative. If any of the above is positive, peripheral blood HBV DNA titer testing is required, and a value less than 1×103 copies/ml is acceptable for inclusion. 7) Left ventricular ejection fraction (LVEF) measured by echocardiography is >= 50%. 8) Patients have no severe and uncontrollable infections, such as lung infection, intestinal infection, or sepsis. 9) Pregnant women must undergo pregnancy tests to confirm they are not pregnant, and they must be willing to take effective contraceptive measures during the study period and for at least 12 months after the last dose; women in pregnancy and lactation periods cannot participate; all male participants must take contraceptive measures during the study period and for at least 3 months after the last dose. 10) Sign the informed consent form.
Exclusion criteria
Exclusion criteria: If any of the following conditions occur in a patient, that patient will be excluded from this study: 1) Patients diagnosed with NK/T lymphoma; 2) Patients allergic to doxorubicin liposomes, etoposide, PD-1 inhibitors, or vinclarak components, or having a severe allergic constitution; 3) Patients with severe myocardial damage, with myocardial enzymes CK and CK-MB elevated by more than 3 times the ULN (upper limit of normal); 4) Patients with heart disease classified as grade II or above according to the New York Heart Association (NYHA) score (including grade II); 5) Patients with thyroid function abnormalities that cannot be corrected by medication; 6) Patients with severe mental illness; 7) Patients with active massive bleeding in internal organs (including gastrointestinal bleeding, pulmonary alveolar bleeding, intracranial bleeding, etc.); 8) Patients who have received DEP regimen for HLH targeted treatment in the past, with ineffective treatment or recurrence; 9) Patients who have used a total cumulative dose of doxorubicin >= 300mg/m2 or epirubicin >= 450mg/m2, or who have had heart lesions caused by anthracycline drugs in the past; 10) Patients who are unable to comply with the study and/or follow-up, and who are also participating in other clinical studies.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Response rate (ORR, CR, PR);Safety; | — |
Secondary
| Measure | Time frame |
|---|---|
| EBV-DNA level (PBMC+plasma);2-month survival;Overall survival;Trend of related indicators; | — |
Countries
China
Contacts
Public ContactXuefeng He
The First Affiliated Hospital of Soochow University
Outcome results
None listed