Korean J Lab Med.  2007 Aug;27(4):248-252. 10.3343/kjlm.2007.27.4.248.

A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndrome with Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells

• Kahng J
• Kim J
• Shin SJ
• Han K

• KMID: 1781492
• DOI: http://doi.org/10.3343/kjlm.2007.27.4.248

Abstract

We report a case of IgA kappa light chain deposition disease and combined adult Fanconi syndrome with Auer rod-like intracytoplasmic inclusions in plasma cells and proximal renal tubular cells in a 54-yr-old female. Cytochemical stainings revealed a strong acid phosphatase activity of the inclusions and weak periodic acid-Schiff positivity, whereas the reactions for peroxidase and alpha-naphthyl acetate esterase were negative. An immunostaining verified IgA-kappa inside the plasma cells. Kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity, and Congo red staining was negative. Electron microscopy showed needle-shaped crystals located in tubular epithelial cells.

Keyword

Auer rod-like inclusion; Light chain deposition disease; Fanconi syndrome

MeSH Terms

Fanconi Syndrome/diagnosis/etiology/*pathology
Female
Humans
*Immunoglobulin A/analysis
Immunoglobulin kappa-Chains/analysis
Inclusion Bodies/*ultrastructure
Kidney Tubules, Proximal/pathology/*ultrastructure
Middle Aged
Paraproteinemias/*pathology
Plasma Cells/pathology/*ultrastructure

Figure

• 

  Fig. 1. Bone marrow plasma cell morphology and special stain. (A) Atypical plasma cells with spindle-shaped, Auer rod-like intracytoplasmic inclusions (Wright stain, ×400/window ×1,000). (B) Strong acid phosphatase activity of the inclusions. Note the lack of staining in surrounding cytoplasm (acid phosphatase, ×1,000). (C) Negative in non-specific esterase stain (Non-specific esterase, ×1,000). (D) and (E) Immunohistochemical stain showing kappa-positivity (D) and lambda-negativity (E) (×1,000).

• 

  Fig. 2. The kidney biopsy revealed Bence Jones cast nephropathy with kappa light chain positivity. (A) H-E stain, ×100. (B) Immunohistochemical stain showing IgA positivity (×1,000) and (C) kappa positivity (×1,000). (D) Congo red stain showing negativity (×100).

• 

  Fig. 3. Ultrastructure of tubular epithelial cells in a case of IgA myeloma kidney. The figure shows needle-shaped crystals located in tubular epithelial cells (TEM) (A) ×2,000 & (B) ×40,000.

Reference

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