肾脏轻-重链沉积病

肾脏病与透析肾移植杂志 ›› 2013, Vol. 22 ›› Issue (1): 90-94.

肾脏轻-重链沉积病

出版日期:2013-02-28 发布日期:2013-03-19

Light and Heavy Chain Deposition Disease

Online:2013-02-28 Published:2013-03-19

摘要/Abstract

摘要：

摘要中年男性，病程3年，临床表现中等量蛋白尿、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%。光镜改变为肾小球结节样病变，免疫病理IgA及单一λ轻链均呈线状沉积于肾小球血管袢及肾小管基膜，超微结构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）。

Abstract:

ABSTRACT A 43-year-old man presented with proteinuria, hematuria, hypoalbuminemia, hypertension, renal failure, and serum IgA λ monoclonal component. Bone marrow aspirate revealed 5% plasma cells. Renal biopsy showed nodular glomerulopathy. Immunoflurescence indicated linear staining along GBMs and TBMs and mesangial staining in glomeruli for λ light chains and IgA. Ultrastructral examination observedd the punctuate and “powdery” electron-dense deposits along GBMs and TBMs. The final diagnosis was Light and Heavy Chain Deposition Disease.

Key words: light and heavy chain deposition disease , monoclonal immunoglobulin deposition disease , renal biopsy

梁少姗，曾彩虹. 肾脏轻-重链沉积病[J]. 肾脏病与透析肾移植杂志, 2013, 22(1): 90-94.

LIANG Shao-shan，ZENG Cai-hong. Light and Heavy Chain Deposition Disease[J]. Chinese Journal of Nephrology, Dialysis & Transplantation, 2013, 22(1): 90-94.

ISSN 1006-298X CN 32-1425/R