Stompór Tomasz, Perkowska-Ptasińska Agnieszka, Wojciechowska Małgorzata, Zając Katarzyna, Chmielewska-Badziąg Agnieszka, Pawłowska Anna
Department of Nephrology, Hypertension and Internal Medicine, University of Warmia and Mazury , Olsztyn , Poland .
Ren Fail. 2014 Jun;36(5):795-9. doi: 10.3109/0886022X.2014.884446. Epub 2014 Feb 10.
Multiple myeloma (MM) is the second most common hematological malignancy, with an annual incidence in Europe and the USA of about 4-6 cases per 100,000. Several forms of renal disease are found in the course of MM, including: cast nephropathy, light chain (LC) deposition disease and primary amyloidosis. Less frequent forms include: acute and chronic tubulopathies, neoplastic plasma cell infiltration and interstitial nephritis. In this paper, we discuss a case of 53-year-old male patient with MM who presented with massive proteinuria (24 g/24 h), mild renal insufficiency (eGFR 43 mL/min), and Fanconi-like syndrome (as reflected by normoglycemic glycosuria). In kidney biopsy glomeruli were normal, whereas abundant AFOG-positive deposits were found in the cytoplasm of proximal tubular epithelial cells. These deposits were strongly positive for kappa light chains on immunofluorescence. Electron microscopy revealed electron-dense, intracytoplasmic crystalloid deposits of variable shape (needle-shaped, round and rectangular), and size in the proximal tubular cells. This unusual variant of microscopic renal lesions in the course of MM coupled with coincidence of Fanconi-like and nephrotic syndrome as a clinical manifestation has not been reported to date.
多发性骨髓瘤(MM)是第二常见的血液系统恶性肿瘤,在欧洲和美国的年发病率约为每10万人中有4 - 6例。在MM病程中可发现多种形式的肾脏疾病,包括:管型肾病、轻链(LC)沉积病和原发性淀粉样变性。较少见的形式包括:急性和慢性肾小管病变、肿瘤性浆细胞浸润和间质性肾炎。在本文中,我们讨论了一例53岁男性MM患者,其表现为大量蛋白尿(24g/24小时)、轻度肾功能不全(估算肾小球滤过率[eGFR]为43mL/分钟)以及范可尼样综合征(表现为血糖正常性糖尿)。肾活检显示肾小球正常,而在近端肾小管上皮细胞的细胞质中发现大量阿尔辛蓝 - 甲醛 - 橘黄G(AFOG)阳性沉积物。这些沉积物在免疫荧光检查中κ轻链呈强阳性。电子显微镜检查显示近端肾小管细胞内有电子致密的、形状(针状、圆形和矩形)和大小各异的胞质晶体样沉积物。MM病程中这种罕见的微观肾脏病变变异形式,以及范可尼样综合征和肾病综合征同时作为临床表现的情况,迄今为止尚未见报道。