Table 1.
Previous reports of crystalline podocytopathy and tubulopathy
Sex/age | Duration of onset to presentation | Clinical renal manifestation | Plasma cell dyscrasia | Glomerular pathology | Crystal distribution | IHC | Treatment | Prognosis |
---|---|---|---|---|---|---|---|---|
M/29 [12] | 12 months | Recurrent proteinuria after two kidney allografts, PCR 6 g/g, SCr 2.3 mg/dl | MGUS→IgG-κ MM |
Recurrent FSGS | Podocytes, proximal TEC | IF/IHC:Positive for κ in TEC, λ negative | Bortezomib, lenalidomide, dexamethasone | Lacking |
F/66 [11] | During evaluation for back pain | SCr 1.7 mg/dl, Fanconi syndrome, albumin 29 g/L, PCR 3.11μg/mgCr | IgG-κ MM | Non-specific | Podocytes, MC, GEC, TEC, tubular lumen,histiocytes | IF:Positive for κ; negative for λ | Bortezomib, melphalan, prednisolone | Overall improvement in her myeloma related laboratory results |
F/52 [10] | Routine health examination | Proteinuria 2.62 g/d, SCr 1.3 mg/dl | IgG-κ MM | FSGS | Podocytes, proximal TEC | IHC: κ positive in TEC, λ negative | Lacking | Lacking |
M/45 [9] | Routine annual physical examination | SCr 1.85 mg/dl, proteinuria 7.925 g/d, glycosuria | IgG-κ MM | Collapsing FSGS | Podocytes,MC, TEC | IF/IHC: negative for κ and λ in crystal areas | Therapy, details lacking | 2 m later, SCr 1.5 mg/dl, proteinuria 3.627 g/d |
M/53 [15] | 78 months of MGUS | SCr 1.3 mg/dl, proteinuria 1.18 g/d, albumin 38 g/L | IgG-κ MGUS | Foamy substance in podocytes | Podocytes and TEC | IF: κ TEC positive; λ negative | 4 cycles of DF and lenalidomide | SCr returned to 1.0 mg/dl |
F/54 [13] | 24 months of MM, 19 months of proteinuria | SCr1.0 → 3.9 mg/dl(2 yrs), proteinuria0.3 → 14.4 g/d (2 yrs., pamidronate), albumin 29 g/L | IgG-κ MM | Collapsing FSGS and LCN | Proximal TECs, podocytes,tubular casts | IF: Negative for κ and λ;IHC: Positive for κ, negative for λ | DF, CYC, thalidomide, bortezomib, HCT | SCr 1.8 mg/dl |
M/56 [8] | < 1 month | SCr1.2 → 9.2 mg/dl (3 m), proteinuria 5 g/L | IgG-κ MM | NA, ATN | Podocytes, TEC, interstitial macrophages, tubular lumen,BM, urine | IF: Negative for both κ and λ | Vincristine, doxorubicin, DF, HCT | SCr 6.3 mg/dl |
F/46 [7] | Unknown | Renal dysfunction | IgG-κ MM | NA | Podocytes, TECs, Interstitial histiocytes | IF: Positive for IgG-κ | Chemotherapy followed by HCT | SCr↓, crystalline- containing podocyte ↓ |
M/51 [6] | 6 months | Bence–Jones proteinuria 1.54 g/L, albumin 41.8 g/L | IgG-κ MM | Nonspecific | Podocytes, GEC, MC, TEC, Interstitial histiocytes,, MCs, hepatocytes and macrophages in liver | NA | Chemotherapy deferred due to lung carcinoma surgery | Died shortly after lung surgery due to multi- organ failure |
F/52 [5] | 60 months | SCr 1.8 → 2.0 mg/dl (5 yrs), Proteinuria 1.3 → 5 g/d(5 yrs), albumin 34 g/L | IgG-κ MM | 3/5 G sclerosed | Podocytes, PEC, TEC, interstitial histiocytes | IF: Negative for κ and λ;IHC: Positive for κ, negative for λ | NA | NA |
F/40 [14] | 14 months | Proteinuria 14.3 g/d, albumin 30 g/L, SCr 1.8 mg/dl | IgG-κ MGUS | FSGS | Podocytes, PEC, distal TECs, tubular lumina, BM | IHC: Positive for κ, negative for λ | NA | NA |
M/75 [4] | 60 months of MM | Proteinuria;chronic renal failure | IgG-κ MM | NA | Podocytes, PEC, TEC, interstitial histiocytes, cornea, myeloma cell, choroid plexus | IHC: Positive for κ and γ | NA | NA |
M/57 [3] | 6 months | SCr 3.2 mg/dl, Proteinuria 2 g/d | IgG-κ MM | FSGS | Podocytes, MC, GEC, PEC, proximal TEC, histiocytes and fibroblasts in the interstitium, synovium and BM | IF: Negative | Cytoxan, carmustine and prednisone, discontinued due to complications | 1.5 years later SCr 3.9 mg/dl, died due to cardiac arrest |
Abbreviation: FSGS focal segmental glomerulosclerosis, MM multiple myeloma, MGUS monoclonal gammopathy of undetermined significance, SCr serum creatinine, GEC glomerular endothelial cell, TEC tubular epithelial cell, MC mesangial cell, PEC parietal epithelial cell, BM bone marrow, NA not available, HCT autologous hematopoietic cell transplantation, ATN acute tubular necrosis, DF dexamethasone, IHC immunohistochemistry, PCR protein/creatinine ratio, LCN light chain cast nephropathy