Treatments
| Conditions . | Therapy . |
|---|---|
| POEMS syndrome | First line: most experience with ASCT and lenalidomide/dexamethasoneFirst line: if only 1-2 bone lesions and negative bone marrow, radiation |
| DADS-M-PN | First line: IVIGSecond line: rituximab |
| CANOMAD | First line: IVIG or plasmapheresisSecond line: rituximab |
| SLOMN | First line: IVIGSecond line: plasma cell–directed therapy |
| Light chain deposition disease | First line: most experience with ASCT and bortezomib/dexamethasone |
| PGMID | First line: rituximabSecond line: bortezomib/dexamethasone |
| Monoclonal fibrillary glomerulonephritis | First line: unknown |
| Immunotactoid GN | First line: rituximab or clone directed therapy |
| Inclusions/crystalline deposits | First line: may consider clone-directed therapy |
| Cryoglobulinemia | First line: treat underlying cause (eg, HCV, CTD, PCD); for severe cases, plasmapheresis, high-dose methylprednisolone, and/or cyclophosphamide may be consideredSecond line: rituximab |
| Scleromyxedema | First line: IVIGSecond line: add lenalidomide or bortezomib |
| Necrobiotica xanthogranuloma | First line: IVIGSecond line: may consider clone-directed therapy |
| Schnitzler syndrome | First line: anti–IL-1 monoclonal therapeuticsSecond line: Waldenstrom macroglobulinemia therapy |
| TEMPI syndrome | First line: plasma cell–directed therapy |
| Clarkson disease | Prophylactic IVIG |
| Crystal-storing histiocytosis29 | First line: observationSecond line: may consider clone-directed therapy |
| Monoclonal gammopathy keratopathy | No treatment required |
| Conditions . | Therapy . |
|---|---|
| POEMS syndrome | First line: most experience with ASCT and lenalidomide/dexamethasoneFirst line: if only 1-2 bone lesions and negative bone marrow, radiation |
| DADS-M-PN | First line: IVIGSecond line: rituximab |
| CANOMAD | First line: IVIG or plasmapheresisSecond line: rituximab |
| SLOMN | First line: IVIGSecond line: plasma cell–directed therapy |
| Light chain deposition disease | First line: most experience with ASCT and bortezomib/dexamethasone |
| PGMID | First line: rituximabSecond line: bortezomib/dexamethasone |
| Monoclonal fibrillary glomerulonephritis | First line: unknown |
| Immunotactoid GN | First line: rituximab or clone directed therapy |
| Inclusions/crystalline deposits | First line: may consider clone-directed therapy |
| Cryoglobulinemia | First line: treat underlying cause (eg, HCV, CTD, PCD); for severe cases, plasmapheresis, high-dose methylprednisolone, and/or cyclophosphamide may be consideredSecond line: rituximab |
| Scleromyxedema | First line: IVIGSecond line: add lenalidomide or bortezomib |
| Necrobiotica xanthogranuloma | First line: IVIGSecond line: may consider clone-directed therapy |
| Schnitzler syndrome | First line: anti–IL-1 monoclonal therapeuticsSecond line: Waldenstrom macroglobulinemia therapy |
| TEMPI syndrome | First line: plasma cell–directed therapy |
| Clarkson disease | Prophylactic IVIG |
| Crystal-storing histiocytosis29 | First line: observationSecond line: may consider clone-directed therapy |
| Monoclonal gammopathy keratopathy | No treatment required |
These treatments are based on case series and not on high levels of evidence.
ASCT, autologous stem cell transplant; CANOMAD, chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin M paraprotein, cold agglutinins, and disialosyl antibodies; CTD, connective tissue disease; DADS-M-PN, distal, acquired, demyelinating, symmetric neuropathy with M protein; GN, glomerulonephritis; HCV, hepatitis C virus; IL-1, interleukin-1; IVIG, intravenous immunoglobulin; PCD, plasma cell disorder; PGMID, proliferative glomerulonephritis with monoclonal immune deposition; POEMS, polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes; SLOMN, sporadic late-onset nemaline myopathy.