Cytokine Guided Risk Stratification and Treatment in Pediatric Hemophagocytic Lymphohistiocytosis
Hemophagocytic Lymphohistiocytoses, Cytokine Storm
About this trial
This is an interventional treatment trial for Hemophagocytic Lymphohistiocytoses focused on measuring Hemophagocytic Lymphohistiocytoses, Cytokine Storm, Ruxolitinib, Interferon-gamma, Interleukin-10, Child, Stratification therapy
Eligibility Criteria
Inclusion Criteria:
- Age from one day to 18 years old;
- Newly diagnosed HLH, fulfilling the HLH criteria;
- To observed the early diagnosis role of cytokines, patients who is suspected to be HLH and fulfill 3 out of 8 criteria can be pre-enrolled.
Exclusion Criteria:
- treated with steroids or etoposide within 72 hours before diagnosis.
Sites / Locations
- The Children's Hospital of Zhejiang University School of MedicineRecruiting
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Experimental
Experimental
Experimental
Experimental
Non-severe DXM group
Non-severe Ruxo group
Severe HLH-94 group
Severe HLH-94 plus ruxolitinib group
Dexamethasone (DXM): week1-2: 10 mg/m2.d, week 3-4: 5 mg/m2.d, week5-6: 2.5 mg/m2.d, week7: 1.25 mg/m2.d, week8: tapering.
Ruxolitinib(Ruxo): body weight (BW)<10kg: 2.5mg Bid; 10-20kg: 5mg Bid; >20kg: 10mg Bid; Orally for 4 weeks.
DXM: week1-2: 10 mg/m2.d, week 3-4: 5 mg/m2.d, week5-6: 2.5 mg/m2.d, week7: 1.25 mg/m2.d, week8: tapering. Etoposide (VP16): 100-150mg/m2 twice in the first two weeks, and once every week to week 8.
DXM: week1-2: 10 mg/m2.d, week 3-4: 5 mg/m2.d, week5-6: 2.5 mg/m2.d, week7: 1.25 mg/m2.d, week8: tapering. Etoposide (VP16): 100-150mg/m2 twice in the first two weeks, and once every week to week 8. Ruxolitinib(Ruxo): body weight (BW)<10kg: 2.5mg Bid; 10-20kg: 5mg Bid; >20kg: 10mg Bid; Orally for 4 weeks.