Bortezomib, Pomalidomide, Dexamethasone for Systemic AL Amyloidosis

Proteasome inhibitor and immunmodulators have synergistic effect for plasma cell dyscrasia. Due to the fact that more than 70-80% of patients with amyloidosis have renal involvement, the application of lenalidomide is limited. Thus, the investigators designed this open-label, multicenter, phase 2 study for newly diagnosed or previously treated systemic AL amyloidosis with bortezomib, pomalidomide and dexamethasone regimen.

Inclusion criteria

• Diagnosis of AL amyloidosis, confirmed by histology and typed with immunohistochemistry, immunoelectron microscopy or mass spectrometry.
• Newly diagnosed or previous treated AL amyloidosis
• Patients must be ≥ 18 years of age.
• ECOG performance status 0, 1 or 2.
• Measurable disease defined by at least one of the following:

① serum free light chain (FLC) ≥2.0 mg/dL (20 mg/L) with an abnormal kappa:lambda ratio or the difference between involved and uninvolved free light chains (dFLC) ≥2mg/dL (20 mg/L).

②. presence of a monoclonal spike that is ≥5 g/l.

• Symptomatic organ involvement (heart, kidney, liver/GI tract, peripheral nervous system).
• Absolute neutrophil count (ANC) ≥1.0 X 10^9/L, Hemoglobin ≥70 g/L, Platelets ≥50 X 10^9/L
• eGFR ≥20 mL/min/ 1.73 m^2
• Written informed consent in accordance with local and institutional guidelines.
• Contraceptive use should be consistent with local regulations regarding the methods of contraception for those participating in clinical studies.

Exclusion criteria

• Fulfill with the criteria of active multiple myeloma or active lymphoplasmacytic lymphoma.
• Presence of other tumors which is/are in advanced malignant stage and has/have systemic metastasis;
• Severe or persistent infection that cannot be effectively controlled;
• Presence of severe autoimmune diseases or immunodeficiency disease;
• Patients with active hepatitis B or hepatitis C ([HBVDNA+] or [HCVRNA+]);
• Patients with HIV infection or syphilis infection;
• Any situations that the researchers believe will increase the risks for the subject or affect the results of the study.