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Monoclonal Immunoglobulin Crystalline Membranous Nephropathy
Salvatore E Mignano1, Virginie Pascal2, Nnaemezie E Odioemene3
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Summary
Monoclonal immunoglobulin crystalline nephropathies are rare. This study details a unique IgG lambda case presenting as membranous nephropathy, expanding the known spectrum of kidney diseases caused by protein crystal deposition.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Monoclonal immunoglobulin (MIg) crystalline nephropathies involve MIg precipitation in the kidney.
- These rare conditions can manifest with diverse kidney pathologies.
Observation:
- A patient with multiple myeloma (IgGλ) and diabetes presented with nephrotic proteinuria.
- Kidney biopsy showed membranous nephropathy superimposed on diabetic glomerulosclerosis, negative for common antibody targets.
- Electron microscopy revealed subepithelial crystals, positive for IgGλ by paraffin immunofluorescence.
Findings:
- Immunoglobulin repertoire sequencing identified a unique, highly mutated IgGλ clone.
- The atypical clone involved a mutated IGHV1-24 heavy chain and a rare IGLV10-54 light chain.
- This case represents a novel presentation of MIg crystalline nephropathy.
Implications:
- This expands the pathological spectrum of MIg crystalline nephropathies.
- Highlights the importance of considering crystalline deposits in unexplained nephropathies.
- Suggests advanced sequencing techniques aid in diagnosing rare immune-mediated kidney diseases.
