A CASE OF MONOCLONAL GAMMOPATHY OF RENAL SIGNIFICANCE WITH A NOVEL VARIANT OF ULTRASTRUCTURAL STRIATED DEPOSITS

 

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https://storage.unitedwebnetwork.com/files/1099/128eea10bef61b9281f7d6bf2020469d.pdf
A CASE OF MONOCLONAL GAMMOPATHY OF RENAL SIGNIFICANCE WITH A NOVEL VARIANT OF ULTRASTRUCTURAL STRIATED DEPOSITS

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Shuichiro
Endo
Shuichiro Endo danielcraig0917@gmail.com Siga General Hospital Nephrology Siga Japan *
Tatsuya Sato t_satou_0216@yahoo.co.jp Siga General Hospital Nephrology Siga Japan -
Azusa Hoshina azutom106@gmail.com Siga General Hospital Nephrology Siga Japan -
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Monoclonal gammopathy of renal significance (MGRS) has been receiving increasing attention among hematologists, but its diagnosis remains challenging for nephrologists due to the wide variety of histological manifestations. Even after a diagnosis of MGRS is established, the rarity of the disease often delays aggressive treatment because of limited therapeutic evidence. Further accumulation of well-documented cases is therefore essential. 

we present a case of MGRS most closely resembling the immunotactoid glomerulopathy variant, but with histological features distinct from those described in previous reports of immunotactoid glomerulopathy.

A 74-year-old Japanese woman with hypertension and chronic heart failure was referred to our hospital for evaluation of stable chronic renal insufficiency, presenting without symptoms but with mild proteinuria. Laboratory data revealed a serum creatinine of 0.83 mg/dL and a urine protein-to-creatinine ratio of 1.53 g/gCr, without hematuria. Serum protein electrophoresis was positive for IgG κ and IgM κ, although serum IgG and IgM levels were within normal ranges, and the serum free light chain ratio was also normal. Bone marrow aspiration showed no evidence of hematological disorder, with only 1.2% plasma cells, consistent with monoclonal gammopathy of undetermined significance (MGUS).

Unexpectedly, kidney biopsy demonstrated membranoproliferative glomerulonephritis characterized by mesangial expansion and double contouring of the glomerular basement membrane. Immunofluorescence revealed IgG κ monotypic deposition along the glomerular tufts. Electron microscopy identified electron-dense deposits in the subendothelial and mesangial regions. At higher magnification, the deposits displayed randomly arranged tubular structures, 40–60 nm in diameter, with striations approximately 20 nm in width.

This case represents monoclonal gammopathy of renal significance (MGRS), most closely resembling the immunotactoid glomerulopathy variant. However, the ultrastructural features of the striated deposits differ distinctly from those described in previous reports of immunotactoid glomerulopathy. Furthermore, this case is unusual in that clinical manifestations and laboratory abnormalities were minimal, and diagnosis was achieved only through electron microscopy. We present this as a possible new variant of immunotactoid glomerulopathy.

This case highlights the diagnostic challenges of MGRS and underscores the importance of accumulating detailed case reports to better characterize its clinicopathological spectrum and guide therapeutic decision-making.

Kewords