hemophagocytic lymphohistiocytosis
Conditions
Interventions
First-line treatment group:Dexamethasone + tofacitinib
Second-line Treatment Group:Tofacitinib + etoposide + dexamethasone
Third-line treatment group:High-dose decitabine
R/R/CAEBV group:gemcitabine + mitoxantrone + oxaliplatin + dexamethasone
Sponsors
Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
Eligibility
Sex/Gender
All
Age
14 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. Aged >= 14 years, primary or secondary HLH diagnosed according to HLH-2004 criteria, etiology including: genetic factors, rheumatic immune diseases, infections, malignant tumors, unknown causes; 2. Newly diagnosed or refractory/relapsed HLH; 3. Refractory/relapsed HLH is defined as non-remission of the disease after 14 days of standard treatment, or rapid progression of the disease after less than 14 days of treatment (expected to be life-threatening, unable to wait until 14 days and must adjust the treatment), or recurrence of HLH after remission and ineffective to the original treatment; Standard options include: HLH-1994 scheme, HLH-2004 scheme, CHOP-like scheme, DEP/L-DEP rescue scheme, ATG rescue scheme, glucocorticoid combined with cytokine antagonist (such as INF-gamma monoclonal antibody), glucocorticoid combined with CD52 monoclonal antibody, As well as improved regimens of such treatments in combination with rituximab, CD25 mAb, PD1 inhibitors, plasmapheresis, splenectomy or JAK inhibitors; 4. Expected survival time greater than 3 month; 5. Signed a written informed consent, comply with the study protocol requirements of the regular inspection, visit the relevant procedures.
Exclusion criteria
Exclusion criteria: 1. Severe allergy to tofacitinib or its excipients; 2. HLH associated with tuberculosis and HIV, as well as HLH caused by simple bacterial, fungal and protozoal infections, can be completely alleviated by controlling infection; 3. Previous use of tofacitinib; 4. The patients with malignant tumor complicated with late multiple metastases had a short expected survival time; 5. Complicated pregnancy; 6. Other major diseases that can not be controlled clinically; 7. Patients with severe renal insufficiency: GFR < 15 ml/min.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Over response rate at the end of induction therapy at the end of week 12 and maintenance therapy at week 40;1 year overall survival; | — |
Secondary
| Measure | Time frame |
|---|---|
| Complete Response Rate;Partial Response Rate;Non-response rate;Median tome-to-onset; | — |
Countries
China
Contacts
Public ContactLiang Tang
Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
Outcome results
None listed