轻重链沉积病合并轻链淀粉样变性  

Light-heavy chain deposition disease with light chain amyloidosis

作　　者：李晓梅[1] 曾彩虹[1] 

机构地区：[1]南京军区南京总医院肾脏科国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2016年第6期573-578,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：中年女性,临床表现为血清肌酐升高,中等量蛋白尿、大量镜下血尿,无高血压,轻度贫血,免疫固定电泳图谱见λ型Ig G单克隆免疫球蛋白条带,骨髓细胞学检查浆细胞比例为8%。肾活检示肾小球系膜区轻-中度增宽,肾血管壁刚果红染色阳性,免疫荧光染色Ig G及λ轻链呈线状沉积于肾小球毛细血管袢及肾小管基膜,间质血管壁λ轻链阳性,超微结构见肾小球基膜内侧缘、系膜区、肾小管基膜外侧缘细沙样高密度的电子致密物沉积,免疫电镜下见电子致密物Ig G、λ轻链染色胶体金颗粒阳性,间质动脉壁淀粉丝分布;皮肤脂肪活检刚果红染色阳性。最终诊断为轻重链沉积病（Ig G-λ型）合并AL型系统性淀粉样变性（累及肾脏、皮肤、心脏）。A 49-year-old woman presented with renal insufficiency,proteinuria,hematuria and serum monoclonal immunoglobulin Ig G-λ.Bone marrow aspirate revealed 8% plasma cells. Renal biopsy showed mild to moderate mesangial proliferation with positive Congo red staining in the vessels.Immunoflurerscence indicated linear staining along GBMs,TBMs and mesangium for λ light chains and Ig G.Ultrastructral examination showed granular electron dense deposits delineating the outer aspect of the TBMs and the endothelial aspect of the GBMs. The final diagnosis was light-heavy chain deposition disease（ Ig G-λ） with light chain amyloidosis,with kidney,skin and heart involved.

关 键 词：淀粉样变性 轻重链沉积病 肾活检 

分 类 号：R597.2[医药卫生—内科学]