[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].
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.
Kahng
Jimin
J
Department of Laboratory Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea. jmkahng@catholic.ac.kr
Kim
Jeana
J
Shin
Suk Joon
SJ
Han
Kyungja
K
D005198:Fanconi Syndrome / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D002479:Inclusion Bodies / Q000648:ultrastructure*; D007687:Kidney Tubules, Proximal / Q000473:pathology / Q000648:ultrastructure*; D008875:Middle Aged; D010265:Paraproteinemias / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology / Q000648:ultrastructure*
D007070:Immunoglobulin A; D007145:Immunoglobulin kappa-Chains