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

Abstract

Background: Monoclonal gammopathy of renal significance (MGRS) is a recently defined group of renal diseases caused by monoclonal immunoglobulin secreted by nonmalignant proliferative B cell or plasma cell. Monoclonal immunoglobulin can form different types of structures deposited in renal tissue, including fibrils, granules, microtubules, crystals and casts, and has mostly been reported in multiple myeloma patients. Here we report a rare case with κ light chain crystals in both podocytes and tubular epithelial cells associated with MGRS, which adds more information to the spectrum of MGRS-related renal diseases.

Case presentation: A 53-year old woman presented with albumin-predominant moderate proteinuria and renal failure. She had monoclonal IgGκ in the serum and monoclonal IgGκ plus free κ in the urine. Multiple myeloma and lymphoproliferative disorders were excluded. Renal biopsy confirmed κ-restricted crystal-storing renal disease involving the podocytes and proximal tubular epithelial cells. The patient was treated with bortezomib followed by lenalidomide-based chemotherapy, and renal function was stable after 1 year of follow-up.

Conclusions: This is a rare case of combined crystalline podocytopathy and tubulopathy associated with MGRS, in which diagnosis was dependent on electron and immuno-electron microscopy.

Keywords: Crystal deposition; MGRS; Monoclonal gammopathy; Podocytopathy.

Fig. 1

Patient renal biopsy findings. a Light microscopy showed some global sclerosis and segmental glomerular sclerosis along with focal tubular atrophy and interstitial inflammatory infiltration. (periodic methenamine silver and Masson trichrome staining, × 40). b Glomerular segmental sclerosis with cytoplasmic vacuolization of podocytes. (periodic acid-Schiff staining, × 200). c Cytoplasmic vacuolization and eosinophilic granules of proximal tubular epithelial cells. (hematoxylin and eosin, × 200). d The rod-like crystals in the cytoplasm of podocytes on EM (× 15,000). e Crystals in the proximal tubular epithelial cell on EM (× 12,000). f Immuno-electron microscopy (labeled by colloid gold particles with a diameter of 10 nm) indicated κ light chain deposition in the crystals without λ light chain (× 40,000)