Optimize First-line Treatment for AL Amyloidosis With t (11; 14)

Achievement of complete hematologic response (CHR) is vital for systemic AL amyloidosis. Currently, the CHR rate of daratumumab, bortezomib, and dexamethasone (DBD) is close to 60%. Considering that Bcl-2 inhibitor is effective for AL amyloidosis with t(11; 14) and the median hematologic onset time of DBD is 7 days. We design a a prospective study on AL amyloidosis with t(11; 14). All patients receive DBD at the beginning. Patient will receive DBD for at least 6 cycles if achieve rapid hematologic response at day 7, while other patients will receive daratumumab, venetoclax and dexamethasone.

Inclusion Criteria: 1. Diagnosis of systemic AL amyloidosis; 2. Daratumumab, bortezomib, dexamethasone used in 1st line treatment; 3. Life expectancy greater than 12 weeks; 4. HGB ≥70g/L; 5. Blood oxygen saturation \>90%; 6. Total bilirubin (TBil) ≤3×upper limit of normal (ULN); aspartate aminotransferase (AST) and alanine aminotransferase (ALT) ≤3.0×ULN; 7. Informed consent explained to, understood by and signed by the patient. Exclusion Criteria: 1. Fulfill with the criteria of active multiple myeloma or active lymphoplasmacytic lymphoma. 2. Presence of other tumors which is/are in advanced malignant stage and has/have systemic metastasis; 3. Severe or persistent infection that cannot be effectively controlled; 4. Presence of severe autoimmune diseases or immunodeficiency disease; 5. Patients with active hepatitis B or hepatitis C (\[HBVDNA+\] or \[HCVRNA+\]); 6. Patients with HIV infection or syphilis infection; 7. Any situations that the researchers believe will increase the risks for the subject or affect the results of the study.