For patients who met the inclusion criteria, treatment regimens were administered: Rituximab 375 mg/m², intravenously, once weekly for 4 weeks. Zanubrutinib 160 mg, orally, twice daily for 4 weeks. Combined drugs: prednisone 100 mg/m²/d, orally, d1-5; Ruxolitinib 15mg bid orally; With/without emapalumab as appropriate.
For patients who met the inclusion criteria, treatment regimens were administered: Rituximab 375 mg/m², intravenously, once weekly for 4 weeks (may be extended or adjusted according to clinical response). Zanubrutinib: 160 mg, orally, twice daily for 4 weeks as for rutuximab. Dose can be adjusted or extended according to tolerance and efficacy. When ≥3 grade hematological or intolerable non-hematological toxicity occur, zanubrutinib or rituximab will be suspended, and the dose will be reduced or resumed according to the toxicity grade after recovery. The dose of zanubrutinib should be adjusted according to the manufacturer's label with concomitant use of strong CYP3A inhibitor/inducer. Combined drugs: prednisone 100 mg/m²/d, orally, d1-5; Ruxolitinib 15mg bid orally; With/without emapalumab as appropriate.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
40
375 mg/m², intravenously, once weekly for 4 weeks
160 mg, orally, twice daily for 4 weeks as for rutuximab.
The First Affiliated Hospital of Soochow University
Suzhou, Jiangsu, China
RECRUITINGresponse rate of HLH after 4 weeks of treatment
RR will be evaluated according to the response evaluation criteria recommended by the International Histiocyte Society.
Time frame: after 4 weeks
OS at week 8
overall survival after 8 weeks of treatment
Time frame: after 8 weeks
AE
Adverse events will be evaluated according to NCI-CTCAE 5.0.
Time frame: till the end of 4 weeks
OS
OS at 1/2/3 months/ 6 months
Time frame: at 4/8/12/24 weeks
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