The optimal treatment in MGRS is a clone directed therapy; where treatment is directed specifically to the cell line responsible for the pathologic<unk>  immunoglobulin or M-protein. The goal of therapy is to preserve kidney function, reduce the risk of MGRS recurrence after kidney transplant and maintain a sustained hematologic response. The difference of involved-uninvolved free light chains less than 4 or greater than a 90% reduction of involved free light chains are the minimum hematologic responses needed for the preservation of kidney function. Plasma cell dyscrasias are best treated with the proteasome inhibitor bortezomib, often given with the steroid dexamethasone. Bortezomib can also be given before and after an autologous stem cell transplant (ASCT) with high dose of the chemotherapy medication mephalan given before ASCT to eradicate the<unk><unk>  prior to transplant and prevent recurrence. Alternative treatment options include immunomodulatory drugs such as thalidomide or<unk> idomide. The<unk>  antibody against the plasma cell surface protein CD38 daratumumab may also be used with very high efficacy against AL amyloidosis. B-cell dyscrasias associated with MGRS have less evidence for specific medications that are effective in achieving a hematologic response. With CD20 expressing B-cells and lymphoplasmacytic clones; rituximab is the preferred treatment and it can be combined with dexamethasone and cyclophosphamide. The benefit of ASCT in B-cell related MGRS is less established.
Monoclonal gammopathy of renal significance
Monoclonal gammopathy of undetermined significance
Familial renal amyloidosis
AL amyloidosis
Secondary systemic amyloidosis
Myeloma cast nephropathy
Haemodialysis-associated amyloidosis
AA amyloidosis