hemophagocytic lymphohistiocytosis
Conditions
Interventions
Single arm group:All enrolled patients will receive ruxolitinib and dexamethasone (RuD) as frontline therapy. Patients who have a favorable response will remain on RuD regimen for a total of 8-weeks.
Sponsors
The First Affiliated Hospital, School of Medicine, Zhejiang University
Eligibility
Sex/Gender
All
Age
18 Years to 90 Years
Inclusion criteria
Inclusion criteria: ? Patients aged = 18 years of both genders; ? Patient has a newly diagnosed HLH according to HLH-2004 diagnostic criteria; ? Patient has active HLH without prior HLH therapy; ? Patient who has received corticosteroid treatment but still have intermittent fever (body temperature >= 38?) and are assessed to have active HLH at enrollment are eligible to participate; ? Patient, parent, or legal authorized representative (LAR) must provide informed consent.
Exclusion criteria
Exclusion criteria: Patients who had any one of the following were ineligible: ? Patient has severe CNS involvement presenting with obvious brain MRI abnormalities or neurological dysfunctions including convulsions, disturbance of consciousness, paralysis; ? Patients with active mycobacteria, histoplasma capsulatumparasitic, leishmania, fungal infection or other uncontrolled infection, or HIV infection ? Patient has severe organ dysfunction, including serious renal dysfunction (creatinine clearance 20, cardiorespiratory failure requiring any extracorporeal life support; ? Patient with organ active bleeding (including gastrointestinal hemorrhage, alveolar hemorrhage, intracranial hemorrhage, etc.); ? Patient with a history of malignant tumor before the diagnosis of HLH, which is still in active stage; ? Pregnant and lactating women, and patients of childbearing age who are unwilling to take contraceptive measures; ? Patient is taking another investigational agent; ? Patients are unable to comply during thestudy.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Overall Response (ORR) rate; | — |
Secondary
| Measure | Time frame |
|---|---|
| the 1-year overall survival (OS) rate of the treatment protocol;Complete Response (CR) rate;Duration of responses;the 6-month event-free survival rate;Adverse events (AEs) of the treatment protocol;the ORR and CR rate at the end of frontline therapy with the RuD regimen;the ORR and CR rate with intensive therapy;Duration of responses of RuD frontline therapy and intensive therapy, respectively;the 6-month event-free survival rate of RuD frontline therapy and intensive therapy, respectively; | — |
Countries
China
Contacts
Public ContactXiujin Ye
The First Affiliated Hospital, School of Medicine, Zhejiang University
Outcome results
None listed