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Fibrillary inclusions in light chain proximal tubulopathy associated with myeloma.

Corbett RW, Cook HT, Duncan N, Moss J - Clin Kidney J (2012)

View Article: PubMed Central - PubMed

Affiliation: Imperial Renal and Transplant Centre, Imperial College Healthcare NHS Trust, London, UK.

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The urinary protein–creatinine ratio was elevated at 819 mg/mmol, with no evidence of glycosuria, hypoalbuminaemia, acidaemia or impaired renal function (serum creatinine 78 μmol/L)... Serum electrophoresis identified a monoclonal IgG kappa band quantified at 20 g/L with associated immunoparesis... Immunoflorescence for light chains was negative on frozen sections but protease-digested paraffin sections showed kappa but not lambda light chain deposition in tubular epithelial cells... These findings are consistent with a proximal tubulopathy with fibrillary inclusions related to a kappa light chain plasma cell dyscrasia... Subsequent bone marrow was hypercellular with 61% plasma cells, which were kappa light chain restricted; she was started on a CTD (cyclophosphamide, thalidomide and dexamethasone) chemotherapeutic regime and has preserved her renal function but has continuing proteinuria... Light chain proximal tubulopathy is an increasingly recognized though still uncommon renal complication of myeloma almost exclusively related to light chain kappa... Some individuals may manifest an associated Fanconi syndrome that was not seen in this woman... Diagnosis can be difficult with frequently non-specific light microscopy findings; on ultrastructural examination, crystalloid inclusions are frequently seen but occasionally may be fibrillary in nature... Crystalloid deposition is thought to occur due to a resistance of the light chain-variable region to lysosymal proteolysis in the proximal tubule... The unusual fibrillary appearances seen here may relate to the physicochemical properties determined by the amino acid sequence in the variable region.

No MeSH data available.


Higher power view of cytoplasmic vacuole showing numerous closely packed filaments 6 nm in diameter.
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fig3: Higher power view of cytoplasmic vacuole showing numerous closely packed filaments 6 nm in diameter.

Mentions: On renal biopsy (Figure 1), there was marked vacuolation of tubular epithelial cells and electron microscopy demonstrated filaments 6 nm in diameter in these cells (Figures 2 and 3). Congo red staining for amyloid was negative. Immunoflorescence for light chains was negative on frozen sections but protease-digested paraffin sections showed kappa but not lambda light chain deposition in tubular epithelial cells. These findings are consistent with a proximal tubulopathy with fibrillary inclusions related to a kappa light chain plasma cell dyscrasia.


Fibrillary inclusions in light chain proximal tubulopathy associated with myeloma.

Corbett RW, Cook HT, Duncan N, Moss J - Clin Kidney J (2012)

Higher power view of cytoplasmic vacuole showing numerous closely packed filaments 6 nm in diameter.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400460&req=5

fig3: Higher power view of cytoplasmic vacuole showing numerous closely packed filaments 6 nm in diameter.
Mentions: On renal biopsy (Figure 1), there was marked vacuolation of tubular epithelial cells and electron microscopy demonstrated filaments 6 nm in diameter in these cells (Figures 2 and 3). Congo red staining for amyloid was negative. Immunoflorescence for light chains was negative on frozen sections but protease-digested paraffin sections showed kappa but not lambda light chain deposition in tubular epithelial cells. These findings are consistent with a proximal tubulopathy with fibrillary inclusions related to a kappa light chain plasma cell dyscrasia.

View Article: PubMed Central - PubMed

Affiliation: Imperial Renal and Transplant Centre, Imperial College Healthcare NHS Trust, London, UK.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

The urinary protein–creatinine ratio was elevated at 819 mg/mmol, with no evidence of glycosuria, hypoalbuminaemia, acidaemia or impaired renal function (serum creatinine 78 μmol/L)... Serum electrophoresis identified a monoclonal IgG kappa band quantified at 20 g/L with associated immunoparesis... Immunoflorescence for light chains was negative on frozen sections but protease-digested paraffin sections showed kappa but not lambda light chain deposition in tubular epithelial cells... These findings are consistent with a proximal tubulopathy with fibrillary inclusions related to a kappa light chain plasma cell dyscrasia... Subsequent bone marrow was hypercellular with 61% plasma cells, which were kappa light chain restricted; she was started on a CTD (cyclophosphamide, thalidomide and dexamethasone) chemotherapeutic regime and has preserved her renal function but has continuing proteinuria... Light chain proximal tubulopathy is an increasingly recognized though still uncommon renal complication of myeloma almost exclusively related to light chain kappa... Some individuals may manifest an associated Fanconi syndrome that was not seen in this woman... Diagnosis can be difficult with frequently non-specific light microscopy findings; on ultrastructural examination, crystalloid inclusions are frequently seen but occasionally may be fibrillary in nature... Crystalloid deposition is thought to occur due to a resistance of the light chain-variable region to lysosymal proteolysis in the proximal tubule... The unusual fibrillary appearances seen here may relate to the physicochemical properties determined by the amino acid sequence in the variable region.

No MeSH data available.