肾淀粉样变性患者321例临床与病理分析  

Clinicopathological analysis of 321 patients with renal amyloidosis

作　　者：程佩瑶 张颖[1] 李远[1] 权松霞[1] 邢国兰[1] CHENG Pei-yao;ZHANG Ying;LI Yuan;QUAN Song-xia;XING Guo-lan(Department of Nephrology,the First Affiliated Hospital of Zhengzhou University»Zhengzhou,Henan 450052,China)

机构地区：[1]郑州大学第一附属医院肾内科,河南郑州450052

出　　处：《中华实用诊断与治疗杂志》2021年第6期588-591,共4页Journal of Chinese Practical Diagnosis and Therapy

基　　金：国家自然科学基金(U1604186)。

摘　　要：目的探讨肾淀粉样变性的分型、临床及病理特征,为临床诊断和治疗提供依据。方法321例肾淀粉样变性患者,采用常规病理技术与激光显微切割联合质谱蛋白组学进行分型诊断。根据分型结果分为单克隆免疫球蛋白组310例和非单克隆免疫球蛋白组11例。单克隆免疫球蛋白组又分为轻链型肾淀粉样变性(amyloid immunoglobulin light-chain,AL)组287例和重链型/重+轻链型肾淀粉样变性(amyloid immunoglobulin heavy-chain/amyloid immunoglobulin heavy-and light-chain,AH/AHL)组23例。比较各组临床资料。肾淀粉样变性患者肾组织行刚果红染色,对肾小球、肾间质和肾血管病理改变进行半定量评分,比较各组病理特征。结果321例肾淀粉样变性患者中,AL型287例(89.41%),AH/AHL型23例(7.17%),血清淀粉样蛋白A型4例(1.25%)、甲状腺素转运蛋白型2例(0.62%),凝溶胶蛋白型2例(0.62%),溶菌酶型1例(0.31%),白细胞趋化因子2型1例(0.31%),甲状腺素转运蛋白型合并轻链型1例(0.31%)。单克隆免疫球蛋白组患者肾功能不全(21.29%)、重度肾间质淀粉样物质沉积(6.45%)、重度肾间质纤维化伴肾小管萎缩(10.97%)和重度肾间质炎细胞浸润(11.61%)比率低于非单克隆免疫球蛋白组(54.55%、27.27%、45.45%、45.45%)(P<0.05);AH/AHL组患者血红蛋白[131.55(120.00,143.00)g/L]、白蛋白[27.93(23.10,37.20)g/L]水平高于AL组[122.28(110.00,137.00)g/L、23.70(19.40,28.40)g/L](P<0.05),补体C3[0.92(0.90,1.06)g/L]、补体C4[0.22(0.20,0.25)g/L]水平低于AL组[1.06(0.90,1.25)g/L、0.32(0.25,0.42)g/L](P<0.05)。结论肾淀粉样变性常见类型为单克隆免疫球蛋白相关淀粉样变性,其临床及病理损伤改变较非单克隆免疫球蛋白型淀粉样变性轻;激光显微切割联合质谱蛋白组学分析有助于明确肾淀粉样变性的分型诊断。Objective To investigate the classification and clinicopathological characteristics of renal amyloidosis,and to provide a basis for clinical diagnosis and therapy.Methods Totally 321 patients with renal amyloidosis were classified by using conventional pathological techniques and laser microdissection followed by mass spectrometry proteomics,and were divided into monoclonal immunoglobulin group(n=310)and non-monoclonal immunoglobulin group(n=11)according to the classification results.Monoclonal immunoglobulin group was redivided into amyloid immunoglobulin light-chain(AL)group(n=287)and amyloid immunoglobulin heavy-chain/amyloid immunoglobulin heavy-and light-chain(AH/AHL)group(n=23).The clinical data were compared between groups.The renal tissue from patients with renal amyloidosis was stained with Congo red.Semi-quantificative scoring of glomerular,interstitial and renal vascular pathological changes was performed to compare the pathological characteristics of each group.Results There were 287 patients(89.41%)of AL type,23 patients(7.17%)of AH/AHL type,4 patients(1.25%)of amyloid serum amyloid protein A type,2 patients(0.62%)of amyloid transthyretin type,2 patients(0.62%)of amyloid gelsolin type,1 patient(0.31%)of amyloid lysozyme type,1 patient(0.31%)of amyloid leukocyte chemotactic factor 2 type,and 1 patient(0.31%)of amyloid transthyretin and immunoglobulin light chainλtype.The percentages of renal insufficiency(21.29%),severe renal interstitial amyloid deposition(6.45%),severe renal interstitial fibrosis with tubule atrophy(10.97%)and severe interstital inflammatory cell infiltration(11.61%)in monoclonal immunoglobulin group were lower than those in non-monoclonal immunoglobulin group(54.55%,27.27%,45.45%,45.45%)(P<0.05).The levels of hemoglobin(131.55(120.00,143.00)g/L)and albumin(27.93(23.10,37.20)g/L)in AH/AHL group were higher than those in AL group(122.28(110.00,137.00)g/L,23.70(19.40,28.40)g/L)(P<0.05),and the levels of complement C3(0.92(0.90,1.06)g/L)and complement C4(0.22(0.20,0.25)g/L)in AH/AHL g

关 键 词：肾淀粉样变性 激光显微切割 质谱蛋白组学 

分 类 号：R692[医药卫生—泌尿科学]