Systemic Light Chain Amyloidosis
Conditions
Interventions
Systemic light chain amyloidosis group:Lishatrokla (C1D1 200mg, C1D2 400mg, C1D3 - C1D28 600mg
start at C2 with 600mg/day, 28 days per cycle, until intolerable or the investigator deems it necessary to stop the treatment)
Daratumumab (administered subcutaneously or intravenously
subcutaneous 1800mg each time, intravenous 16mg/kg
for weeks 1-8, once a week (total 8 administrations)
for weeks 9-24, every 2 weeks (total 8 administrations)
starting from week 25, every 4 weeks until disease progression)
Dexamethasone (administered intravenously or orally
20mg/day, administered on days 1, 2, 8, 9, 15, 16, 22, and 23)
Recurrent or refractory systemic light chain amyloidosis group:Rishatoclax (C1D1 200mg, C1D2 400mg, C1D3-C1D28 600mg, C2 600mg/ day, 28d/Cycle, until intolerable or investigator's determination)
Dexam
Sponsors
The First Affiliated Hospital of Kunming Medical University
Eligibility
Sex/Gender
All
Age
18 Years to 80 Years
Inclusion criteria
Inclusion criteria: Systemic light amyloidosis group Age > = 18 years old and = 10g/L (2) 24-hour urine protein electrophoresis shows monoclonal protein >200mg (3) dFLC>50mg/L 6. Physical fitness condition score = 2 points; 7. The functions of important organs are basically normal, meeting the following requirements: Blood routine: Absolute neutrophil count > = 1.0×10^9/L, platelet count > = 75×10^9/L, hemoglobin > = 80g/L (without blood transfusion); Liver and kidney function: Serum total bilirubin = 30mL/ (min·1.73m^2); 8. Voluntarily sign the informed consent form and be able to cooperate in completing the examinations and follow-ups at each stage of the trial. Recurrent or refractory systemic light amyloidosis group 1. At least one previous systemic treatment for AL has been received, and there is clear evidence of recurrence or refractory (refractory is defined as disease progression during or within 60 days after the end of systemic treatment for AL, or no > = PR effect has been achieved after >=2 cycles of systemic treatment); 2. Patients who have previously received CD38 monoclonal antibody treatment are allowed to be enrolled, except for those clearly refractory to CD38 monoclonal antibody (the refractory criteria refer to the previous item or are determined by the researcher). 3. The remaining inclusion criteria are the same as those for the systemic light amyloidosis group.
Exclusion criteria
Exclusion criteria: 1. Patients to be enrolled in the systemic light chain amyloidosis group should have received targeted treatment for light chain amyloidosis in the past; patients to be enrolled in the recurrent or refractory systemic light chain amyloidosis group should have received treatment targeting Bcl-2 in the past; 2. Have other malignant tumors; 3. Have other types of amyloidosis; 4. Have severe active infections (such as sepsis, active tuberculosis, uncontrolled pneumonia, etc.); 5. Have severe neurological diseases (such as status epilepticus, severe cognitive impairment, uncontrolled peripheral neuropathy resulting in inability to take care of oneself); 6. Have active HBV/HCV infection or HIV infection; 7. Are allergic to "Lishatoukla" and any excipients of it; 8. Pregnant or lactating women (female patients need to take effective contraceptive measures during the trial and within 30 days after discontinuation of the treatment, male patients themselves and their sexual partners need to take effective contraceptive measures during the trial and within 90 days after discontinuation of the treatment); 9. Have severe mental disorders, drug abuse history, or situations that make it impossible to cooperate with the trial follow-up; 10. Other situations that the investigator deems not suitable for participating in this study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| A partial response rate of >= very good was achieved within 2 treatment courses.; | — |
Secondary
| Measure | Time frame |
|---|---|
| Organ response rate and >= complete response rate at any time;Progression-free survival period;Overall survival period;Safety; | — |
Countries
China
Contacts
Public ContactMingxia Shi
The First Affiliated Hospital of Kunming Medical University
Outcome results
None listed