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Crystalloid glomerulopathy in monoclonal gammopathy of renal significance (MGRS).

Vankalakunti M, Bonu R, Shetty S, Siddini V, Babu K, Ballal SH - Clin Kidney J (2014)

Bottom Line: The immunoperoxidase staining showed kappa light chain restriction.Subsequently, the bone marrow showed 6% plasma cells which confirmed the diagnosis of monoclonal gammopathy of renal significance.He responded well to bortezomib treatment with resolution of the nephrotic syndrome and normalization of renal function after 7 months.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology , Manipal Hospital , Bangalore , India.

ABSTRACT
We report a case of monoclonal gammopathy of renal significance in a 63-year-old man who presented with nephrotic-range proteinuria and renal insufficiency. The kidney biopsy showed a membranoproliferative glomerulonephritis pattern with extensive crystalloid deposits in the glomerular capillary endothelial cells and very few in the tubular epithelial cells. The immunoperoxidase staining showed kappa light chain restriction. Subsequently, the bone marrow showed 6% plasma cells which confirmed the diagnosis of monoclonal gammopathy of renal significance. He responded well to bortezomib treatment with resolution of the nephrotic syndrome and normalization of renal function after 7 months.

No MeSH data available.


Related in: MedlinePlus

Crystals showing kappa light chain restriction (A) and negative lambda light chain (B) (×40, immunoperoxidase). Immunofluorescence revealed linear capillary wall deposits with IgG of 1+ intensity on a scale of 0–4+; non-descript pattern with kappa light chain (2+) in the crystals and negative lambda light chain reaction (not shown).
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SFU025F3: Crystals showing kappa light chain restriction (A) and negative lambda light chain (B) (×40, immunoperoxidase). Immunofluorescence revealed linear capillary wall deposits with IgG of 1+ intensity on a scale of 0–4+; non-descript pattern with kappa light chain (2+) in the crystals and negative lambda light chain reaction (not shown).

Mentions: The biopsy revealed a membranoproliferative glomerulonephritis ‘pattern’ (Figure 1) with numerous eosinophilic crystalloid structures of variable shapes within the cytoplasm of endothelial cells and a few in the podocytes (Figure 2). The tubules showed very few foci with crystalloid structures indicating proximal tubulopathy. Immunofluorescence with IgG, IgA, IgM, C3 and C1q were negative. Immonoperoxidase staining for kappa and lambda light chain showed a strong reaction with kappa light chains and negative reaction with lambda (Figure 3). Ultrastructure study of endothelial cells showed rhomboid to hexagonal shaped crystals with sharp edges within the cytoplasm. A diagnosis of crystalloid glomerulopathy with an membranoproliferative glomerulonephritis (MPGN) pattern and proximal tubulopathy consistent with Fanconi syndrome was made.Fig. 1.


Crystalloid glomerulopathy in monoclonal gammopathy of renal significance (MGRS).

Vankalakunti M, Bonu R, Shetty S, Siddini V, Babu K, Ballal SH - Clin Kidney J (2014)

Crystals showing kappa light chain restriction (A) and negative lambda light chain (B) (×40, immunoperoxidase). Immunofluorescence revealed linear capillary wall deposits with IgG of 1+ intensity on a scale of 0–4+; non-descript pattern with kappa light chain (2+) in the crystals and negative lambda light chain reaction (not shown).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4377747&req=5

SFU025F3: Crystals showing kappa light chain restriction (A) and negative lambda light chain (B) (×40, immunoperoxidase). Immunofluorescence revealed linear capillary wall deposits with IgG of 1+ intensity on a scale of 0–4+; non-descript pattern with kappa light chain (2+) in the crystals and negative lambda light chain reaction (not shown).
Mentions: The biopsy revealed a membranoproliferative glomerulonephritis ‘pattern’ (Figure 1) with numerous eosinophilic crystalloid structures of variable shapes within the cytoplasm of endothelial cells and a few in the podocytes (Figure 2). The tubules showed very few foci with crystalloid structures indicating proximal tubulopathy. Immunofluorescence with IgG, IgA, IgM, C3 and C1q were negative. Immonoperoxidase staining for kappa and lambda light chain showed a strong reaction with kappa light chains and negative reaction with lambda (Figure 3). Ultrastructure study of endothelial cells showed rhomboid to hexagonal shaped crystals with sharp edges within the cytoplasm. A diagnosis of crystalloid glomerulopathy with an membranoproliferative glomerulonephritis (MPGN) pattern and proximal tubulopathy consistent with Fanconi syndrome was made.Fig. 1.

Bottom Line: The immunoperoxidase staining showed kappa light chain restriction.Subsequently, the bone marrow showed 6% plasma cells which confirmed the diagnosis of monoclonal gammopathy of renal significance.He responded well to bortezomib treatment with resolution of the nephrotic syndrome and normalization of renal function after 7 months.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology , Manipal Hospital , Bangalore , India.

ABSTRACT
We report a case of monoclonal gammopathy of renal significance in a 63-year-old man who presented with nephrotic-range proteinuria and renal insufficiency. The kidney biopsy showed a membranoproliferative glomerulonephritis pattern with extensive crystalloid deposits in the glomerular capillary endothelial cells and very few in the tubular epithelial cells. The immunoperoxidase staining showed kappa light chain restriction. Subsequently, the bone marrow showed 6% plasma cells which confirmed the diagnosis of monoclonal gammopathy of renal significance. He responded well to bortezomib treatment with resolution of the nephrotic syndrome and normalization of renal function after 7 months.

No MeSH data available.


Related in: MedlinePlus