系统性轻重链型肾淀粉样变性  

Heavy and light chain renal amyloidosois

作　　者：陈文萃 任贵生 梁丹丹 曾彩虹 黄湘华 CHEN Wencui;REN Guisheng;LIANG Dandan;ZENG Caihong;HUANG Xianghua(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing University School of Medicine,Nanjing 210016,China)

机构地区：[1]东部战区总医院、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》2021年第6期596-600,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：江苏省重点研发计划社会发展面上项目(BE2017721)。

摘　　要：48岁中年男性患者,因发现蛋白尿3年余入院,临床表现为中等量蛋白尿,无镜下血尿和肾功能损害,服泼尼松及环孢素治疗1年余尿检异常无缓解。肾活检病理提示肾脏淀粉样变性,κ轻链及IgG1染色阳性。骨髓浆细胞比例正常,免疫分型可见少量单克隆浆细胞,血游离轻链绝对值及比值均正常,尿游离κ轻链升高,血和尿均未检测到M蛋白,无心脏、胃肠道、皮肤等肾外脏器受累证据。诊断为轻重链型肾淀粉样变性。采用以硼替佐米为基础的方案治疗后尿蛋白及血清肌酐稳定,血清白蛋白升高,疗效仍有待进一步观察。A 48-year-old male patient was admitted to the hospital for proteinuria with more than 3 years. Clinical manifestations were moderate proteinuria, no microscopic hematuria or renal dysfunction Renal biopsy showed renal amyloidosis with positive staining of κ light chain and IgG1. The proportion of bone marrow plasma cells was normal, a small number of monoclonal plasma cells was detected in immunophenotyping, the absolute value and ratio of serum free light chain were normal, urinary free κ light chain increased, M protein was not detected in serum and urine, and there was no evidence of involvement of heart, gastrointestinal tract or skin. It was diagnosed as heavy and light chain renal amyloidosis. The patients were treated with bortezomib-based regimen.

关 键 词：肾淀粉样变性 轻重链型淀粉样变性 肾脏病理 

分 类 号：R597.2[医药卫生—内科学] R692[医药卫生—临床医学]