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中年男性,贫血、高黏血症、肾功能不全、尿检阴性,免疫球蛋白IgM升高、κ型IgM单克隆免疫球蛋白条带,骨髓活检提示华氏巨球蛋白血症。肾脏病理组织学见弥漫肾小管萎缩,小管基膜增厚明显;间质嗜酸性细胞浸润;肾小球病变较轻。免疫荧光染色免疫球蛋白和补体染色阴性。轻链染色显示肾小球毛细血管袢、肾小管基膜、血管壁κ轻链染色阳性。电镜下见肾小球毛细血管袢基膜内侧缘、包曼囊壁、肾小管基膜外侧缘、血管壁见泥沙样电子致密物沉积。结合临床及病理,最终诊断华氏巨球蛋白血症相关的κ型轻链沉积病伴急性间质性肾炎。
Middle-aged men, anemia, hyperviscosity, renal insufficiency, urinalysis, elevated IgM, κ-IgM monoclonal immunoglobulin bands, and bone marrow biopsies suggest FHM. Renal histopathology see diffuse tubular atrophy, tubulointerstitial thickening significantly; interstitial eosinophilic infiltration; glomerular lesions lighter. Immunofluorescence immunoglobulin and complement staining negative. Light chain staining showed glomerular capillary loop, tubular basement membrane, kappa light chain staining positive. Electron microscope, see glomerular capillaries 袢 basement membrane inside edge, Baoman capsule wall, the outer edge of the basement membrane of the renal tubular, vascular wall see sediment-like electron dense deposits. Combined with clinical and pathological diagnosis of Fahrenheit macroglobulinemia-related light chain deposition disease with acute interstitial nephritis.