Table 6.

Consensus opinions for the treatment of selected MGRS conditions

MGRS entityCurrent consensus/recommendation
MIDDCKD 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)
PGNMIDCKD 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
ITGCLL-type regimens incorporating bendamustine/corticosteroids/cyclophosphamide with rituximab
For cases not associated with CLL, bortezomib-based regimens
Type 1 cryoglobulinemic GNTreatment 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 GNHCV+
 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 FSCKD 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