Consensus opinions for the treatment of selected MGRS conditions
| MGRS entity . | Current consensus/recommendation . |
|---|---|
| MIDD | CKD stage I-III: bortezomib-based therapy, followed by ASCT (in the absence of extrarenal manifestations and good performance status) |
| CKD stage IV-V: eligible for renal transplant, bortezomib-based therapy followed by ASCT; not eligible for renal transplant, bortezomib-based therapy only (to protect extrarenal organs, heart) | |
| PGNMID | CKD stage I-II with proteinuria < 1 g/d and nonprogressive disease: symptomatic treatment |
| CKD stage I-II with proteinuria > 1 g/d or progressive disease and CKD stage III-IV: chemotherapy* with or without ASCT | |
| CKD stage V: eligible for renal transplant, chemotherapy followed by ASCT; not eligible for renal transplant, symptomatic management; no identifiable MG: no consensus, may benefit from chemotherapy prior to renal transplant | |
| ITG | CLL-type regimens incorporating bendamustine/corticosteroids/cyclophosphamide with rituximab For cases not associated with CLL, bortezomib-based regimens |
| Type 1 cryoglobulinemic GN | Treatment indication: symptomatic/progressive systemic disease (renal), depends on underlying clone |
| Plasma cell: antimyeloma drugs (ASCT may be considered) | |
| LPL clone: treat along lines of WM (rituximab backbone) | |
| B-cell clone: rituximab-based therapy | |
| Type 2 cryoglobulinemic GN | HCV+ |
| Minimally symptomatic: antiviral therapy | |
| Symptomatic vasculitis: rituximab/high-dose dexamethasone (+ antiviral therapy) | |
| Rapidly progressive renal disease: TPE + definitive therapy as for symptomatic vasculitis (above) | |
| HCV− | |
| Minimally symptomatic: observation | |
| Symptomatic vasculitis: rituximab | |
| WM/B-cell NHL: rituximab-based regimens (according to the underlying condition) | |
| LCPT with FS | CKD stage I-III: chemotherapy based on bortezomib/cyclophosphamide/thalidomide/bendamustine, ASCT may be considered for nonresponding patients |
| CKD stage IV-V: eligible for renal transplant, bortezomib-based therapy followed by ASCT; not eligible for renal transplant, symptomatic management |
| MGRS entity . | Current consensus/recommendation . |
|---|---|
| MIDD | CKD stage I-III: bortezomib-based therapy, followed by ASCT (in the absence of extrarenal manifestations and good performance status) |
| CKD stage IV-V: eligible for renal transplant, bortezomib-based therapy followed by ASCT; not eligible for renal transplant, bortezomib-based therapy only (to protect extrarenal organs, heart) | |
| PGNMID | CKD stage I-II with proteinuria < 1 g/d and nonprogressive disease: symptomatic treatment |
| CKD stage I-II with proteinuria > 1 g/d or progressive disease and CKD stage III-IV: chemotherapy* with or without ASCT | |
| CKD stage V: eligible for renal transplant, chemotherapy followed by ASCT; not eligible for renal transplant, symptomatic management; no identifiable MG: no consensus, may benefit from chemotherapy prior to renal transplant | |
| ITG | CLL-type regimens incorporating bendamustine/corticosteroids/cyclophosphamide with rituximab For cases not associated with CLL, bortezomib-based regimens |
| Type 1 cryoglobulinemic GN | Treatment indication: symptomatic/progressive systemic disease (renal), depends on underlying clone |
| Plasma cell: antimyeloma drugs (ASCT may be considered) | |
| LPL clone: treat along lines of WM (rituximab backbone) | |
| B-cell clone: rituximab-based therapy | |
| Type 2 cryoglobulinemic GN | HCV+ |
| Minimally symptomatic: antiviral therapy | |
| Symptomatic vasculitis: rituximab/high-dose dexamethasone (+ antiviral therapy) | |
| Rapidly progressive renal disease: TPE + definitive therapy as for symptomatic vasculitis (above) | |
| HCV− | |
| Minimally symptomatic: observation | |
| Symptomatic vasculitis: rituximab | |
| WM/B-cell NHL: rituximab-based regimens (according to the underlying condition) | |
| LCPT with FS | CKD stage I-III: chemotherapy based on bortezomib/cyclophosphamide/thalidomide/bendamustine, ASCT may be considered for nonresponding patients |
| CKD stage IV-V: eligible for renal transplant, bortezomib-based therapy followed by ASCT; not eligible for renal transplant, symptomatic management |
HCV, hepatitis C virus; TPE, therapeutic plasma exchange.
Adapted from Fermand et al.90
For PGNMID cases, choice of chemotherapy should be clone directed. For plasma cell clone, bortezomib-based regimens, like cyclophosphamide/bortezomib/dexamethasone; for B-cell clone, rituximab-based regimens may be used. Because clone is detected in only ∼30% of cases of PGNMID, an empirical therapy directed at the hypothesized clone may be used.92