Correct!
1. Bortezomib / cyclophosphamide / dexamethasone
3. The same chemotherapeutic agents as initial multiple myeloma
4. 1 and 3
The initial treatment of primary amyloidosis is the same as the initial treatment of multiple myeloma which is the combination of bortezomib, cyclophosphamide and dexamethasone (3). The goal is to reduce the neoplastic or abnormal plasma cell clone. Bortezomib is a proteasome inhibitor and cyclophosphamide is an alkylating agent. Survival in amyloidosis correlates with response to treatment. Serum N-terminal pro-BNP is a useful prognostic biomarker in cardiac amyloidosis.
Future therapies include an amyloid-directed monoclonal antibody that is designed to remove amyloid fibrils from the affected organs with ongoing phase 2b and phase 3 clinical trials. There is also an anti-SAP antibody (SAP is a protein that stabilizes amyloid fibrils) studied in a pilot trial with some patients having dramatic reversal of liver amyloid deposition leading to removal of the amyloid rather than just preventing further deposition.
References