Version July 2025
Ig: immunoglobulin; IgM: immunoglobulin M; MIDD: monoclonal immunoglobulin deposition diseases; MM: multiple myeloma; RCd: rituximab, cyclophosphamide, dexamethasone; SGLT2: sodium-glucose cotransporter 2; VCd: bortezomib, cyclophosphamide, dexamethasone; WM: Waldenström macroglobulinemia.
* Treatment should be selected and administered in consultation with a hematologist or oncologist who is experienced in using antimyeloma and antilymphoma agents. The choice of therapy depends on access and availability, especially with anti-CD38 monoclonal antibodies, which are not approved or covered for this indication. We prefer monoclonal antibody therapy as initial therapy for most patients and reserve combination therapy for patients who cannot receive monoclonal antibody therapy or are refractory. Glucocorticoids alone are only used for patients who have issues with access or tolerance to our preferred regimens. The optimal duration of clone-directed therapy is uncertain. In general, we aim for a limited duration of approximately 6 months and then follow the patient by observation. Patients who have not responded after 6 months of initial therapy may require a modification of their treatment regimen.
¶ In patients who have monoclonal Ig deposition in the kidney, no detectable monoclonal protein in the serum or urine, and normal kidney function and proteinuria <1 g/day, the decision to aggressively treat with chemotherapy is more difficult, since there is no clear evidence that a pathologic clone is responsible for the kidney disease. In such patients, a more conservative approach to treatment is preferred.
Δ Rather than empiric therapy, some clinicians might choose to treat these patients conservatively with antiproteinuric therapy and blood pressure control for 3 months with plans to escalate to clone-directed therapy if there is no improvement or the patient has worsening disease.
