[一例IgA κ轻链沉积病合并成人范科尼综合征，浆细胞和近端肾小管细胞内有奥氏小体样胞浆内包涵体]
[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].
作者信息
Kahng Jimin, Kim Jeana, Shin Suk Joon, Han Kyungja
机构信息
Department of Laboratory Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
出版信息
Korean J Lab Med. 2007 Aug;27(4):248-52. doi: 10.3343/kjlm.2007.27.4.248.
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.
我们报告了一例54岁女性的IgA κ轻链沉积病合并成人范科尼综合征，其浆细胞和近端肾小管细胞中存在Auer小体样胞质内包涵体。细胞化学染色显示包涵体具有强酸性磷酸酶活性，过碘酸希夫反应弱阳性，而过氧化物酶和α-萘乙酸酯酶反应阴性。免疫染色证实浆细胞内存在IgA-κ。肾活检显示为本周氏蛋白管型肾病，κ轻链阳性，刚果红染色阴性。电子显微镜显示肾小管上皮细胞中有针状晶体。
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