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中年男性,病程3年,临床表现为中等量蛋白尿、少量镜下血尿、高血压、肾功能不全,伴贫血、白细胞减低,血清免疫固定电泳见λ型IgA单克隆免疫球蛋白条带,骨髓细胞学检查浆细胞比例5%。组织学改变为肾小球结节样病变,免疫荧光染色IgA及单一λ轻链均呈线状沉积于肾小球毛细血管袢及肾小管基膜,超微结构见肾小球基膜内侧缘及肾小管基膜外侧缘细沙状、高电子密度的致密物沉积。最终诊断为轻-重链沉积病(IgA-λ型)。
Middle-aged men, duration of 3 years, the clinical manifestations of moderate proteinuria, a small amount of microscopic hematuria, hypertension, renal insufficiency, with anemia, leukopenia, serum immunoelectrophoresis electrophoresis see λ IgA monoclonal immunoglobulin bands, Bone marrow cytology test 5% of plasma cells. Histological changes to glomerular nodular-like lesions, immunofluorescence staining of IgA and a single lambda light chain were linear deposition in the glomerular capillary loop and tubular basement membrane, the ultrastructure of glomerular basement membrane within the lateral margin And the outer side of the tubular basement membrane sand-shaped, high-density electron density deposition. The final diagnosis was light-heavy chain deposition disease (IgA-λ type).