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. 2018 Nov 12;19:322. doi: 10.1186/s12882-018-1108-x

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