白细胞趋化因子2型肾淀粉样变性病1例  

Leukocyte chemotactic factor 2 associated renal amyloidosis: one case report

作　　者：王妍[1,2] 王素霞[1,2] 章友康[1,2] 

机构地区：[1]北京大学第一医院肾内科 [2]北京大学肾脏疾病研究所卫生部肾脏疾病重点实验室慢性肾脏病防治教育部重点实验室,北京100034

出　　处：《北京大学学报（医学版）》2015年第2期349-351,共3页Journal of Peking University:Health Sciences

摘　　要：淀粉样变性病是由于某种蛋白质发生构象改变，聚合形成具有β片层结构的不溶性的纤维丝，沉积于细胞外间隙，使组织结构破坏导致器官功能障碍并逐渐进展的疾病［1］。淀粉样蛋白可以来源于不同的前体蛋白，从而可将淀粉样变性病分为不同的类型。目前已知的淀粉样变前体蛋白已有26种，在我国约90％为轻链型淀粉样变性病（ light chain amyloidosis， AL）［2］，而白细胞趋化因子2（ leukocyte chemotactic factor 2，LECT2）型淀粉样变性病（ leukocyte chemotac-tic factor 2-associated amyloidosis， ALect2）是最近发现的一种新的淀粉样变类型［3］。目前国内尚未见该类型淀粉样变的病例报道，现将北京大学第一医院肾内科诊断的1例ALect2病例报道如下。SUMMARY Here we report a case of leukocyte chemotactic factor 2 （ LECT2 ）-associated renal amyloi-dosis （ ALect2） in our hospital.A 68-year-old male presented with massive proteinuria, hematuria, and hypertension.The renal function was normal.Light microscopy of the renal biopsy revealed glomeruli, interstitium and arteriole with amorphous pink acellular deposits on hematoxylin and eosin stain.The de-posits were strongly stained for Congo red and presented apple green birefringence viewed with polarized light.Ultrastructural examination revealed nonbranching fibrils （ diameters ranging from 8 nm to 12 nm） distributed in glomerular mesangium, subendothelia and renal interstitium.Immunohistochemistry and immunoelectron microscopy using a polyclonal anti-LECT2 antibody showed that the amyloid deposits and the fibrils were stained positively.ALect2 presented proteinuria,with or without acute/chronic renal dys-function clinically and all compartments of the kidney were involved.

关 键 词：肾 淀粉样变性 趋化因子类 

分 类 号：R597.2[医药卫生—内科学]