A protocol named as "HLH-DR" for patients with refractory and secondary hemophagocytic lymphohistiocytosismay.
A modified protocol about ruxolitinib combined with dexamethasone which includes oral ruxolitinib (2.5 mg twice daily for patients if the age\<14 years and the weight \<25kg，5 mg twice daily for patients if the age\<14 years and the weight≥25kg, 10mg twice daily for patients if the age ≥14 years and\<18 years）. Dexamethasone (10mg/m2.d) delayes for the first 2 weeks （form week 1 to 2），(5mg/m2.d) from week 3 to 4，(2.5mg/m2.d) from week 5 to 6），1.25mg/m2.d for week 7，and dropped off on week 8. Every 2 weeks, patients can be evaluated based the diagnosed index
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
SINGLE
Enrollment
50
Ruxolitinib （2.5 mg twice daily for patients if the age\<14 years and the weight \<25kg，5 mg twice daily for patients if the age\<14 years and the weight≥25kg, 10mg twice daily for patients if the age ≥14 years and\<18 years）
Dexamethasone (10mg/m2.d) delayes for the first 2 weeks（form week 1 to 2),(5mg/m2.d) from week 3 to 4，(2.5mg/m2.d) from week 5 to 6），1.25mg/m2.d for week 7，and dropped off on week 8.
Yan Yue
Beijing, Chaoyang District, China
RECRUITINGYan Yue
Beijing, China
RECRUITINGSeverity of disease
Serum ferritin\>2000ng/ml, disease actvie; serum ferritin\<2000ng/ml, disease control;
Time frame: 2 weeks
Axillary temperature
36\<Axillary temperature\<37.2, disease control; 37.2\<Axillary temperature\<38.2, disease partly control; Axillary temperature\>38.2, disease active
Time frame: 2 weeks
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