Hemophagocytic Lymphohistiocytosis
Conditions
Keywords
Pegaspargase, hemophagocytic lymphohistiocytosis, Epstein Barr virus, initial treatment
Brief summary
This study aimed to investigate the efficacy and safety of Pegaspargase together with liposomal doxorubicin, etoposide and high dose methylprednisolone (L-DEP) as an initial treatment for Epstein Barr virus-induced hemophagocytic lymphohistiocytosis.
Interventions
DRUGPegaspargase
2000U/m2 day5
25 mg/m2 day 1
DRUGetoposide
100 mg/m2 was administered once on the first day of every week
DRUGmethylprednisolone
15 mg/kg days 1 to 3, 0.75 mg/kg days 4 to 7
DRUGEtoposide
150 mg/m2 twice weekly for 2 weeks and then weekly
DRUGdexamethasone
initially 10 mg/m2 for 2 weeks followed by 5 mg/m2 for 2 weeks, 2.5 mg/m2 for 2 weeks, 1.25 mg/m2 for one week, and one week of tapering
Sponsors
Beijing Friendship Hospital
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
14 Years to 75 Years
Healthy volunteers
No
Inclusion criteria
1. Patients were older than 14 years of age 2. Diagnosed as EBV-Hemophagocytic Lymphohistiocytosis (HLH) 3. Patients did not receive any treatment for HLH before 4. 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. history of acute and chronic pancreatitis 8. Participate in other clinical research at the same time
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Overall response(complete response+ partial response) rate of Participants | Change from before and 2,4,6 and 8 weeks after initiating L-DEP or HLH-94 therapy | A complete response was defined as normalization of all of the quantifiable symptoms and laboratory markers of HLH, including levels of sCD25(pg/ml), ferritin(μg/L), and triglyceride(mmol/L); hemoglobin(g/L); neutrophil counts(×109/L); platelet counts(×109/L); and alanine aminotransferase (ALT(U/L)). A partial response was defined as at least a 25% improvement in 2 or more quantifiable symptoms and laboratory markers as follows: sCD25(pg/ml) response was\>1.5-fold decreased; ferritin (μg/L)and triglyceride(mmol/L) decreased at least 25%; for patients with an initial neutrophil count of\<0.5 ×109/L, a response was defined as an increase by at least 100% to\>0.5× 109/L; for patients with a neutrophil count of 0.5 to 2.0 × 109/L, an increase by at least 100% to \>2.0 × 109/L was considered a response; and for patients with ALT \>400 U/L, response was defined as an ALT (U/L)decrease of at least 50%. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Compare survival between two arms | from the time patients received L-DEP or HLH-94 therapy up to 12 months or September 2019 | — |
| Number of participants with treatment-related adverse events as assessed by CTCAE v4.0 | through study completion, an average of 1 years | Adverse events including pancreatitis, liver function damage, myelosuppression, infection, bleeding and so on. |
| Change of Epstein-Barr virus(EBV)-DNA before and after therapy | Change from before and 2, 4, 6 and 8 weeks after initiating L-DEP or HLH-94 therapy | — |
Countries
China
Contacts
Primary Contactjingshi wang, M.M.
Outcome results
None listed