Hemophagocytic Lymphohistiocytosis
Conditions
Brief summary
This study aimed to investigate the efficacy and safety of different doses of ruxolitinib as a salvage therapy for refractory/relapsed hemophagocytic lymphohistiocytosis(HLH).
Interventions
DRUGlow dose ruxolitinib
The dose for adult patients(Age\>=14 years) is generally 10 mg twice daily. For children(Age\<14 years ,weight \>=25kg),the dose was generally 5mg twice daily. For children(Age\<14 years, weight \<25kg), the dose was generally 2.5mg twice daily.
DRUGhigh dose ruxolitinib
The dose for adult patients(Age\>=14 years) is generally 20mg twice daily. For children(Age\<14 years ,weight \>=25kg),the dose was generally 10mg twice daily. For children(Age\<14 years, weight \<25kg), the dose was generally 5mg twice daily
Sponsors
Beijing Friendship Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
1 Years to 75 Years
Healthy volunteers
No
Inclusion criteria
1. meet hemophagocytic lymphohistiocytosis (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 ≤75 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 human immunodeficiency virus(HIV) antigen or antibody negative； 7. Hepatitis C virus (HCV) antibody is negative, or HCV antibody is positive, but HCV RNA is negative； 8. Both hepatitis B virus (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. Informed consent.
Exclusion criteria
1. Pregnancy or lactating Women； 2. Allergic to ruxolitinib； 3. Active bleeding of the internal organs； 4. uncontrollable infection； 5. Serious mental illness; 6. Non-melanoma skin cancer history; 7. Patients unable to comply during the trial and/or follow-up phase; 8. Participate in other clinical research at the same time.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Response rate | 1 years | complete response (CR) and partial response (PR) rates |
| Progression Free Survival | 1 years | from date of inclusion to date of progression, relapse, or death from any cause Overall Survival |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Overall Survival | 1 years | from the date of inclusion to date of death, irrespective of cause Adverse Events |
| Adverse events | 1 year | Adverse events including myelosuppression, infection, bleeding and so on. |
Countries
China
Contacts
Primary Contactjingshi wang
Outcome results
None listed