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.
10.3343/kjlm.2007.27.4.248
- Author:
Jimin KAHNG
1
;
Jeana KIM
;
Suk Joon SHIN
;
Kyungja HAN
Author Information
1. Department of Laboratory Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea. jmkahng@catholic.ac.kr
- Publication Type:Case Report ; English Abstract
- Keywords:
Auer rod-like inclusion;
Light chain deposition disease;
Fanconi syndrome
- MeSH:
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
- From:The Korean Journal of Laboratory Medicine
2007;27(4):248-252
- CountryRepublic of Korea
- Language:Korean
-
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.
