Incidence of detectable monoclonal protein, most commonly found MGs, and the reported hematological disorders associated with MGRS
| Type of monoclonal immunoglobulin deposition . | Associated renal pathologies . | Incidence of detectable MG . | Most common type of paraprotein/MG . | Associated hematological disorders . |
|---|---|---|---|---|
| Intact immunoglobulin molecule/immunoglobulin molecule with truncated heavy chain* | ALH amyloidosis4,10,28,29 * | 97%-100% | IgGλ | MM, CLL |
| LHCDD10,28,29,46,47 * | SPEP/IFE: 80%-100% | IgGκ | MM (50%) | |
| UPEP/IFE: 80%-100% | ||||
| sFLC assay: 100% | ||||
| Cryoglobulinemic GN28,29,48,49 | Type 1: 76%-82.5% | Type I IgG/IgM with κ | Type 1: MM, WM, CLL, B-NHL, MGRS, HCL | |
| Type 2: 40%-49% | Type II IgMκ | Type 2: B-CLPD, LPL, MALToma, WM | ||
| PGNMID10,29,31,50 | 20%-30% | IgG3 | Rare (4.4%): MM, CLL, NHL | |
| ITG9,28,29,51 | 63%-71% | IgG1 | CLL (19%), LPL (13%), MM (13%) | |
| FG28,52-54 | 15%-17% | IgG | MM, CLL | |
| Light chains only | AL amyloidosis4,10,28,29 | 97%-100% | VλVI | MM, LPL, CLL |
| LCDD10,28,29,46,47 | SPEP/IFE: 25%-76% | VκIV | MM (39%-59%), MGRS (39%), LPL | |
| UPEP/IFE: 42%-90% | ||||
| sFLC assay: 100% | ||||
| LCPT9,28,29,41,55 | 93.4%-97% | VκI | MGRS (27%-46%), MM (14%-33%), SMM (15%-51%), NHL (4%), CLL (2%), WM (8%) | |
| CSH9,28,29,56 | 81.8%-90% | κ | MM, LPL, MGRS (rare) | |
| Heavy chains only | AH amyloidosis4,10,28,29 * | 97%-100% | IgG | MM |
| HCDD10,28,29,46,47 * | SPEP/IFE: 67%-100% | IgG1 | MM (29%) | |
| UPEP/IFE: 50%-100% | ||||
| sFLC assay: 100% | ||||
| No monoclonal protein xdemonstrable in kidneys | C3G (C3GN and DDD)28,29,45,57-60 † | 33%-83% | IgGκ | MGRS (60%-77.8%), MM (4%-13.9%), SMM (5.6%-30%), CLL/lymphoma (5.6%-6%), type 1 cryoglobulinemia (2.8%) |
| TMA29,57 † | 13.7% (>50 y, 21%; >60 y, 24%) | IgGκ | MGRS (75%), MM (5%), SMM (5%), POEMS (10%), T-cell lymphocytic leukemia (5%) | |
| Atypical | MN secondary to monoclonal immunoglobulin19,37 | 0%-21.4% | IgG1κ | CLL/SLL (17.8%-28.5%), MM (7.1%), SMZL (3%) |
| Type of monoclonal immunoglobulin deposition . | Associated renal pathologies . | Incidence of detectable MG . | Most common type of paraprotein/MG . | Associated hematological disorders . |
|---|---|---|---|---|
| Intact immunoglobulin molecule/immunoglobulin molecule with truncated heavy chain* | ALH amyloidosis4,10,28,29 * | 97%-100% | IgGλ | MM, CLL |
| LHCDD10,28,29,46,47 * | SPEP/IFE: 80%-100% | IgGκ | MM (50%) | |
| UPEP/IFE: 80%-100% | ||||
| sFLC assay: 100% | ||||
| Cryoglobulinemic GN28,29,48,49 | Type 1: 76%-82.5% | Type I IgG/IgM with κ | Type 1: MM, WM, CLL, B-NHL, MGRS, HCL | |
| Type 2: 40%-49% | Type II IgMκ | Type 2: B-CLPD, LPL, MALToma, WM | ||
| PGNMID10,29,31,50 | 20%-30% | IgG3 | Rare (4.4%): MM, CLL, NHL | |
| ITG9,28,29,51 | 63%-71% | IgG1 | CLL (19%), LPL (13%), MM (13%) | |
| FG28,52-54 | 15%-17% | IgG | MM, CLL | |
| Light chains only | AL amyloidosis4,10,28,29 | 97%-100% | VλVI | MM, LPL, CLL |
| LCDD10,28,29,46,47 | SPEP/IFE: 25%-76% | VκIV | MM (39%-59%), MGRS (39%), LPL | |
| UPEP/IFE: 42%-90% | ||||
| sFLC assay: 100% | ||||
| LCPT9,28,29,41,55 | 93.4%-97% | VκI | MGRS (27%-46%), MM (14%-33%), SMM (15%-51%), NHL (4%), CLL (2%), WM (8%) | |
| CSH9,28,29,56 | 81.8%-90% | κ | MM, LPL, MGRS (rare) | |
| Heavy chains only | AH amyloidosis4,10,28,29 * | 97%-100% | IgG | MM |
| HCDD10,28,29,46,47 * | SPEP/IFE: 67%-100% | IgG1 | MM (29%) | |
| UPEP/IFE: 50%-100% | ||||
| sFLC assay: 100% | ||||
| No monoclonal protein xdemonstrable in kidneys | C3G (C3GN and DDD)28,29,45,57-60 † | 33%-83% | IgGκ | MGRS (60%-77.8%), MM (4%-13.9%), SMM (5.6%-30%), CLL/lymphoma (5.6%-6%), type 1 cryoglobulinemia (2.8%) |
| TMA29,57 † | 13.7% (>50 y, 21%; >60 y, 24%) | IgGκ | MGRS (75%), MM (5%), SMM (5%), POEMS (10%), T-cell lymphocytic leukemia (5%) | |
| Atypical | MN secondary to monoclonal immunoglobulin19,37 | 0%-21.4% | IgG1κ | CLL/SLL (17.8%-28.5%), MM (7.1%), SMZL (3%) |
B-CLPD, B-cell chronic lymphoproliferative disorder; B-NHL, B-cell NHL; DDD, dense deposition disease; HCL, hairy cell leukemia; IFE, immunofixation electrophoresis; LHCDD, light and heavy chain deposition disease; LPL, lymphoplasmacytic lymphoma; MALToma, mucosa-associated lymphoid tissue lymphoma; SLL, small lymphocytic lymphoma; SMZL, splenic marginal zone lymphoma; SPEP, serum protein electrophoresis; UPEP, urine protein electrophoresis.
Shows entities with truncated heavy chains.
Provisional MGRS entities.