hemophagocytic lymphohistiocytosis
Conditions
Interventions
Treatment group:anti-CD25 rhMAb:1mg/Kg/dose, IV every other week, 6 doses required.
Sponsors
Union Hospital, Tongji Medical College, Huazhong University of Science and Technology
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: (1) Primary or secondary HLH diagnosed according to the HLH-2004 criteria at age =18 years, etiology includes: Genetic factors, rheumatic immune diseases, infections, malignant tumors, and unknown causes. (2) Refractory/recurrent HLH meets the HLH-2004 criteria. (3)Refractory/recurrent HLH is defined as disease that has not resolved after 14 days of standard regimens, or has rapidly progressed in less than 14 days (life-threatening disease that cannot be expected to wait until 14 days and for which regimens must be adjusted), or has returned to HLH after remission and has not responded to prior therapy; The standard regimens include: HLH-1994 regimen, HLH-2004 regimen, CHOP-like regimen, DEP/L-DEP rescue regimen, ATG rescue regimen, glucocorticosteroids in combination with cytokine antagonists (e.g., INF-gamma monoclonal antibody), and the above treatments in combination with rituximab, PD1 inhibitors, plasmapheresis, splenectomy or JAK inhibitors (4) Expected survival > 3 months (5) Signed written informed consent
Exclusion criteria
Exclusion criteria: (1) Severe allergy to anti-CD25 rhMAb or its excipients. (2) Tuberculosis, HIV-associated HLH, and HLH caused by simple bacterial, fungal, or protozoal infections that resolve completely with infection control. (3) Stabilized HLH after initial HLH treatment or other regimens (4) Previously treated with anti-CD25 rhMAb (5) Patients with multiple metastatic malignant tumors in advanced stages who have a very short survival expectancy (6) Combined pregnancy (7) Patients with other major clinically uncontrollable diseases (8) Patients with acute hepatitis or liver function abnormalities greater than two times the normal value.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Overall response rate at the end of treatment at the end of week 12;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