kjlm The Korean Journal of Laboratory Medicine Korean J Lab Med 2383-5702 2383-5710 The Korean Society for Laboratory Medicine 10.3343/kjlm.2007.27.4.248 kjlm-27-248 Original Article 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 KahngJiminM.D. 1 KimJeanaM.D. 2 ShinSuk JoonM.D. 3 HanKyungjaM.D. 1 Department of Laboratory Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea Department of Hospital Pathology, The Catholic University of Korea College of Medicine, Seoul, Korea Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea 022007 10022007 27 4 248 252 26012007 19062007 20062007 Copyright © 2007 The Korean Society for Laboratory Medicine 2007 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 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 α-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.

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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).

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).

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.