Skip to main content

Table 1 Previous reports of crystalline podocytopathy and tubulopathy

From: Monoclonal light chain crystalline podocytopathy and tubulopathy associated with monoclonal gammopathy of renal significance: a case report and literature review

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

  1. 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