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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.

Citation

Jimin Kahng, Jeana Kim, Suk Joon Shin, Kyungja Han. 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]. The Korean journal of laboratory medicine. 2007 Aug;27(4):248-52

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PMID: 18094584

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