【Bence Jones蛋白k型多发性骨髓瘤致肾Fanconi综合征1例】。

Nihon Jinzo Gakkai shi Pub Date : 2016-01-01
Miho Inomata, Koki Tokunaga, Mai Nakahara, Hiroyuki Deguchi, Takuma Kojyo, Masaharu Abe, Miki Oyamada, Man-Ei Oku, Akio Ido
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引用次数: 0

摘要

一位60岁男性,曾因慢性肾脏疾病及慢性乙型肝炎感染而接受治疗,因胸骨疼痛及蛋白尿及血尿加重而转介至我院。入院时,实验室检查结果显示有低磷血症、血糖和尿NAG和62MG水平升高的证据。该患者被诊断为范可尼综合征,检查结果显示存在泛氨基酸性尿症、尿中尿酸排泄量升高和磷重吸收率增加。此外,骨显像显示肋骨两侧放射性示踪剂的多重对称摄取增加,从而诊断为低磷血症相关性骨软化伴肾范可尼综合征。尿免疫电泳显示存在K - Bence Jones蛋白(BJP)。骨髓活组织检查显示血浆细胞比小于10%。然而,患者有超过lg/天的蛋白尿和血清IgM抑制(18mg/dL),因此根据SWOG标准诊断为多发性骨髓瘤。光镜检查显示肾小球硬化,小叶间动脉内膜增厚,近端上皮小管细胞细胞质中有嗜酸颗粒沉积。免疫荧光显示这些区域的抗k染色阳性。近端小管上皮细胞的电镜检查显示存在许多菱形和椭圆形晶体,被认为是BJP的K轻链。一般来说,石膏肾病、轻链沉积病(LCDD)和AL淀粉样变是公认的骨髓瘤引起的肾脏损伤。然而,很少有范可尼综合征合并多发性骨髓瘤的临床报道,比如我们在这里描述的病例研究。此外,活检样本的组织学检查提供了近端上皮小管细胞中K - BJP的进一步证据。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[A case of renal Fanconi syndrome due to Bence Jones' protein K-type multiple myeloma].

A 60-year-old man, who had been treated for chronic kidney disease and chronic hepatitis B infection, was referred to our hospital following presentation with thoracic bone pain and exacerbation of proteinuria and hematu- ria. On admission, laboratory test results showed evidence of hypophosphatemia, glucosuria and elevated levels of both urinary NAG and 62MG.The patient was diagnosed with Fanconi syndrome based on findings indicating the presence of pan-aminoaciduria, elevated urinary excretion of uric acid and an increased phosphorus reabsorption rate. Furthermore, bone scintigraphy showed increased multiple symmetric uptake of radiotracer in both sides of the ribs, leading to the diagnosis"of hypoposphatemia-related osteomalacia with renal Fanconi syndrome. Urinary immunoelectrophoresis indicated the presence of K Bence Jones' protein (BJP). A bone marrow biopsy examina- tion showed that the plasma-to-cell ratio was less than 10%. However, the patient had over lg/day of proteinuria and suppression of serum IgM (18mg/dL) and was, therefore, diagnosed with multiple myeloma based on SWOG criteria. Light microscopic examination showed evidence of glomerulosclerosis, intimal thickness of interlobular arteries and acidophilic granular deposits in the cytoplasm of the proximal epithelial tubular cells. Immunofluores- cence indicated positive anti-K staining in these regions. Electron microscopic examination of the proximal tubular epithelial cells revealed the presence of numerous diamond-shaped and oval crystals, thought to be the K light chain of BJP. In general, cast nephropathy, light chain deposition disease (LCDD) and AL amyloidosis are recog- nized renal injuries caused by myeloma. However, there have been few clinical reports of Fanconi syndrome with multiple myeloma, such as the case study we have described here. In addition, histological examination of a biopsy sample provided further evidence of K BJP in the proximal epithelial tubular cells.

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