Table 3.

Clinical cases of monoclonal gammopathies of renal significance

VariableCase 1Case 2Case 3
Presentation50-yr-old woman presented with hypertension, headaches, hematuria, and proteinuria76-yr-old man with edema, hematuria, and proteinuria69-yr-old woman presented with subacute worsening of known hypertension and headaches
eGFR at diagnosis43 ml/min per 1.73 m238 ml/min per 1.73 m232 ml/min per 1.73 m2
Proteinuria at diagnosis4.2 g/d5.6 g/d3.6 g/d
Kidney biopsy diagnosisPGNMID (IgGκ)PGNMID (IgGλ)Diffuse endocapillary and roliferative membranous GN with monoclonal IgGκ deposits
Hematologic evaluation
 Paraprotein workupSPEP: IgGκ 0.1 gm/dlSPEP: IgGλ 0.7 gm/dlSPEP, serum IFE, 24-hr UPEP and IFE negative, serum-free light chain assay normal
 Clone workup80% involvement by κ-restricted B cells (CD5+, CD 19+, CD20+, CD23+, and CD38-)Bone marrow biopsy morphology normalBone 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
TreatmentPrednisone and rituximabaCyclophosphamide, bortezomib, and dexamethasone for nine cyclesbRituximab, cyclophosphamide, and prednisonec
Last eGFR68 ml/min per 1.73 m253 ml/min per 1.73 m248 ml/min per 1.73 m2
Last proteinuria<0.1 g/d0.14 g/d0.1 g/d
Follow-up36 mo18 mo11 mo
Hematologic outcomeNot assessedSPEP: IgGλ 0.2 gm/dlNot 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.