Hemophagocytic lymphohistiocytosis
Conditions
Interventions
Treatment group:Ruxolitinib±Emapalumab
Sponsors
The First Affiliated Hospital of Soochow University
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Meet the HLH-2004 or HLH diagnostic criteria (satisfy >= 5 diagnostic criteria, or have a clear cause and meet typical clinical manifestations); 2. Diagnosed with intermediate-low risk HLH, including one of the following types: ? HLH caused by non-EBV-related infection; ? HLH related to rheumatic immune diseases (such as adult Still's disease, systemic lupus erythematosus, etc.); ? HLH related to drugs; ? HLH secondary to B-cell lymphoma; ? HLH with unknown cause but in active clinical stage and not considering chemotherapy for the time being; 3. Expected to be able to receive treatment for >= 14 days; 4. Pregnant women must undergo pregnancy tests to confirm they are not pregnant, and are willing to take effective contraceptive measures during the trial period and within >= 12 months after the last administration; Pregnant and lactating women cannot participate; All male participants must take contraceptive measures during the trial period and within = 3 months after the last administration; 5. Can provide informed consent, understand and comply with all requirements of the study.
Exclusion criteria
Exclusion criteria: 1. Confirmed diagnosis of EBV infection-related HLH (EBV-HLH); 2. Confirmed diagnosis of T/NK cell lymphoma-related HLH; 3. Confirmed diagnosis of B cell lymphoma-related HLH, for those who have failed previous treatment with ruxolitinib ± eculizumab; 4. Requires immediate chemotherapy with etoposide and other drugs for high-risk/rapid-progressing type HLH; 5. Those who had severe allergic reactions to ruxolitinib or eculizumab in the past; 6. Those with active tuberculosis or other severe infections that are not under control; 7. Those with severe organ dysfunction (such as patients with grade II or above heart disease as determined by the New York Heart Association (NYHA) score, patients with severe renal dysfunction (glomerular filtration rate 20), central nervous system involvement, etc.); 8. Pregnant or lactating women; 9. Those participating in other interventional clinical studies that may affect the efficacy assessment; 10. Other situations where the researcher assesses that the risks outweigh the benefits and are not suitable for inclusion in the study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Response rate (ORR, CR, PR); | — |
Secondary
| Measure | Time frame |
|---|---|
| safety;Survival at 60 days;Overall survival at 6 months;Trend of related indicators; | — |
Countries
China
Contacts
Public ContactXuefeng He
The First Affiliated Hospital of Soochow University
Outcome results
None listed