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Lambda Light Chain Non-crystalline Proximal Tubulopathy with IgD Lambda Myeloma.
Seiji Kishi1, Fumiaki Obata1, Hirokazu Miki2
1Department of Nephrology, Graduate School of Biomedical Sciences, Tokushima University, Japan.
Internal Medicine (Tokyo, Japan)
|August 14, 2018
Summary
Light Chain Proximal Tubulopathy (LCPT) is a rare kidney disease caused by monoclonal free light chains. This case highlights successful treatment of IgD lambda myeloma with bortezomib and dexamethasone, achieving a very good partial response without worsening kidney function.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Light Chain Proximal Tubulopathy (LCPT) is a rare kidney disease characterized by monoclonal free light chain deposition and damage to proximal renal tubular epithelial cells.
- Paraprotein-related kidney diseases represent a spectrum of conditions where abnormal immunoglobulin proteins harm the kidneys.
Observation:
- A 78-year-old woman presented with anemia and kidney dysfunction.
- Serum and urine protein electrophoresis identified a monoclonal IgD and lambda (λ) free light chain abnormality.
- Kidney biopsy confirmed proximal tubular injury and λ light chain accumulation, with electron microscopy ruling out crystalline structures.
Findings:
- The patient was diagnosed with LCPT secondary to IgD lambda myeloma.
- Treatment with bortezomib and dexamethasone resulted in a very good partial response (VGPR).
- The patient's kidney function did not worsen during treatment.
Implications:
- This case underscores the importance of identifying monoclonal light chains in patients with unexplained kidney dysfunction.
- Effective treatment of the underlying plasma cell dyscrasia can lead to favorable outcomes in LCPT.
- Bortezomib and dexamethasone demonstrate efficacy in managing IgD lambda myeloma-associated LCPT.
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