Table 3.
Clinical cases of monoclonal gammopathies of renal significance
| Variable | Case 1 | Case 2 | Case 3 |
| Presentation | 50-yr-old woman presented with hypertension, headaches, hematuria, and proteinuria | 76-yr-old man with edema, hematuria, and proteinuria | 69-yr-old woman presented with subacute worsening of known hypertension and headaches |
| eGFR at diagnosis | 43 ml/min per 1.73 m2 | 38 ml/min per 1.73 m2 | 32 ml/min per 1.73 m2 |
| Proteinuria at diagnosis | 4.2 g/d | 5.6 g/d | 3.6 g/d |
| Kidney biopsy diagnosis | PGNMID (IgGκ) | PGNMID (IgGλ) | Diffuse endocapillary and roliferative membranous GN with monoclonal IgGκ deposits |
| Hematologic evaluation | |||
| Paraprotein workup | SPEP: IgGκ 0.1 gm/dl | SPEP: IgGλ 0.7 gm/dl | SPEP, serum IFE, 24-hr UPEP and IFE negative, serum-free light chain assay normal |
| Clone workup | 80% involvement by κ-restricted B cells (CD5+, CD 19+, CD20+, CD23+, and CD38-) | Bone marrow biopsy morphology normal | Bone marrow biopsy morphology and immunohistochemistry normal |
| Peripheral blood flow cytometry: monoclonal B cell population present (CD5+, CD10+, CD19+, CD20+, CD23+, and CD38-) | Bone marrow flow cytometry: clonal plasma cell population present (λ-restricted) restriction (CD138+, CD38+, CD19-, CD56+, CD117+, and cytoplasmic–λ positive) | No clone detected | |
| Treatment | Prednisone and rituximaba | Cyclophosphamide, bortezomib, and dexamethasone for nine cyclesb | Rituximab, cyclophosphamide, and prednisonec |
| Last eGFR | 68 ml/min per 1.73 m2 | 53 ml/min per 1.73 m2 | 48 ml/min per 1.73 m2 |
| Last proteinuria | <0.1 g/d | 0.14 g/d | 0.1 g/d |
| Follow-up | 36 mo | 18 mo | 11 mo |
| Hematologic outcome | Not assessed | SPEP: IgGλ 0.2 gm/dl | Not evaluable |
PGNMID, proliferative GN with monoclonal Ig deposits; SPEP, serum protein electrophoresis; IFE, immunofixation; UPEP, urine protein electrophoresis.
a
Rituximab 375 mg/m2 for four doses.
b
Intravenous cyclophosphamide 300 mg/m2 on days 1, 8, and 15; intravenous bortezomib 1.3 mg/m2 on days 1, 8, and 15; oral dexamethasone 20 mg on days 1, 8, and 15 on 28-day cycle.
c
Rituximab 375 mg/m2 on day 1, intravenous cyclophosphamide 750 mg/m2 on day 1; oral prednisone 100 mg daily on days 1–5; repeat on 21-day cycle.