Marginal zone lymphoma accompanied by Sjögren's syndrome
Conditions
Interventions
Treatment group:Orelabrutinib combined with rituximab
Sponsors
The FIrst Affiliated Hospital, College of Medicine, Zhejiang University
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. Age >= 18 years old, gender not restricted; 2. Patients diagnosed with primary Sjögren's syndrome who meet the new 2016 ACR/EULAR classification criteria for primary Sjögren's syndrome; 3. Patients complaining of dry mouth and dry eyes; 4. Patients with positive autoantibodies (anti-SSA/Ro-60); 5. Patients who have not received hormone, immunosuppressant, or biological agent treatment within 4 weeks prior to screening; 6. Pregnant women of childbearing age with negative pregnancy test results; agree to use effective contraceptive measures during the trial; 7. Patients who have not participated in any drug trials within 12 weeks prior to enrollment; 8. Histopathologically confirmed CD20-positive marginal zone lymphoma including MALT, SMZL, NMZL, with at least one lesion outside the spleen exceeding 1.5 cm in any axis; 9. MZL that has progressed, relapsed, or is not suitable for local treatment after previous local treatment (local treatment includes surgery, radiotherapy, anti-Helicobacter pylori treatment, anti-hepatitis C treatment); 10. ECOG performance status score of 0-2; 11. Having treatment indications (having B symptoms, blood cell decline, bleeding, large mass, rapid tumor progression, etc.); 12. Main organ functions meeting the following standards: a) Blood routine: Absolute neutrophil count >= 1.5 × 10^9/L, platelet count >= 75 × 10^9/L, hemoglobin >= 75 g/L; if accompanied by bone marrow invasion, absolute neutrophil count >= 1.0 × 10^9/L, platelet count >= 50 × 10^9/L, hemoglobin >= 50 g/L; b) Blood biochemistry: Total bilirubin = 3 months; 14. Voluntarily signed written informed consent form before trial screening.
Exclusion criteria
Exclusion criteria: 1. Currently or previously have had other malignant tumors, unless radical treatment has been performed and there is evidence of no recurrence or metastasis within the past 5 years; 2. Lymphoma has involved the central nervous system or has transformed to a higher grade; 3. Non-hematological toxicities from previous anti-tumor treatments have not recovered to 470ms, males >450ms; d) Have symptomatic or drug-requiring coronary artery heart disease subjects; e) Have difficult-to-control hypertension (after improving lifestyle, using reasonable and tolerable doses of 3 or more antihypertensive drugs (including diuretics) for more than 1 month and blood pressure still not reached the standard, or using 4 or more antihypertensive drugs to effectively control blood pressure); 5. Had active bleeding within 2 months prior to screening, or were taking anticoagulant drugs, or the investigator considered there was a clear bleeding tendency; 6. Urine protein =2+, and 24-hour urine protein quantification >=2g/24 hours; 7. Had a history of deep vein thrombosis or pulmonary embolism within the past 6 months; 8. Have a history of organ transplantation or allogeneic bone marrow transplantation; 9. Had major surgery within 6 weeks prior to screening or minor surgery within 2 weeks prior to screening. Major surgery is defined as surgery using general anesthesia, but endoscopic examinations for diagnostic purposes are not considered major surgery. Insertion of vascular access devices will be exempted from this exclusion criterion; 10. Active infection or uncontrolled HBV (HBsAg positive and/or HBcAb positive and HBV DNA titer positive), HCV Ab positive, HIV/AIDS or other severe infectious diseases; 11. Currently have subjects with pulmonary fibrosis, interstitial pneumonia, pneumoconiosis, radiation pneumonia, drug-related pneumonia, etc., which significantly affect lung function; 12. Have previously received treatment with BTK, BCR pathway inhibitors (such as PI3K, Syk) and BCL-2 inhibitors; 13. Suitable and prepared for stem cell transplantation; 14. Any mental or cognitive disorders that may limit their understanding, execution of the informed consent form, and compliance with the study; 15. Subjects who are drug addicts or alcoholics; 16. Pregnant, lactating women and fertile subjects who do not wish to take contraceptive measures; 17. The investigator considers other situations that are not suitable for participating in this trial.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| objective response rate, ORR; | — |
Secondary
| Measure | Time frame |
|---|---|
| 24-Month Complete Response Rate, CR24;2-year overall survival rate;Time to Complete Response within 24 Months, TTCR24;Best Overall Response ;2-Year Progression-Free Survival Rate;The differences between ESSDAI at the 24th week and the baseline;The differences between ESSPRI at the 24th week and the baseline;Duration of Response, DOR;Safety;Complete Response Rate, CRR;Time to Response, TTR; | — |
Countries
China
Contacts
Public ContactJie Jin
The FIrst Affiliated Hospital, College of Medicine, Zhejiang University
Outcome results
None listed