华氏巨球蛋白血症相关的轻链沉积病

肾脏病与透析肾移植杂志 ›› 2012, Vol. 21 ›› Issue (4): 390-394.

华氏巨球蛋白血症相关的轻链沉积病

出版日期:2012-08-28 发布日期:2012-09-03

Light chain deposition disease associated with Waldenström macroglobulinemia

Online:2012-08-28 Published:2012-09-03

摘要/Abstract

摘要：

摘要 46岁男性患者，贫血、高黏血症、肾功能不全、尿检基本阴性，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、基膜增厚明显，间质嗜酸性细胞浸润，肾小球病变较轻，肾组织免疫荧光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。

关键词: 华氏巨球蛋白血症 , 轻链沉积病 , 肾活检

Abstract:

ABSTRACT A 46-year-old male was characterized by anemia, hyperviscosity syndrome, renal failure, increased immunoglobulin IgM, and serum κ IgM monoclonal component. Bone marrow biopsies indicated Waldenstrom macroglobulinemia. Renal biopsy showed diffuse tubular atrophy, tubular basement membrane thickening and focal interstitial infiltration of eosinophils with unremarkable glomerular lesion. Immunofluorescence revealed linear staining for κ light chain along glomerular capillary wall, tubular basement membrane, and blood vessel wall, and all of negative for IgG, IgA, IgM, C3, and C1q. Electron microscopy showed punctate electron dense deposits along glomerular capillary wall, the Bowman capsule wall, tubular basement membrane and vessel wall. The final diagnosis was κ light chain deposition disease associated with Waldenström macroglobulinemia accompanied with acute interstitial nephritis.

Key words: Waldenström macroglobulinemia , light chain deposition disease , Renal biopsy

陈浩，曾彩虹，刘志红. 华氏巨球蛋白血症相关的轻链沉积病[J]. 肾脏病与透析肾移植杂志, 2012, 21(4): 390-394.

CHEN Hao, ZENG Cai-hong, LIU Zhi-hong. Light chain deposition disease associated with Waldenström macroglobulinemia[J]. Chinese Journal of Nephrology, Dialysis & Transplantation, 2012, 21(4): 390-394.

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