伴多核巨细胞浸润的轻链型肾淀粉样变性  

Renal light chain amyloidosis with multinucleated giant cells

作　　者：梁丹丹 赵亮 黄湘华 曾彩虹 LIANG Dandan;ZHAO Liang;HUANG Xianghua;ZENG Caihong(National Clinical Research Center for Kidney Diseases,Jinling Hospital,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,南京210016

出　　处：《肾脏病与透析肾移植杂志》2024年第1期87-91,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金面上项目(82270767)。

摘　　要：64岁男性患者,临床表现为肾病综合征,无高血压及肾功能损害,血免疫固定电泳检查示λ型IgA单克隆免疫球蛋白条带,血游离轻链检查示κ/λ比值下降;骨髓流式细胞学见4.52%单克隆增生的浆细胞,外院肾活检诊断轻链型肾淀粉样变性。患者化疗后临床达非常好的部分缓解,经重复肾活检后诊断为罕见的伴多核巨细胞浸润的轻链型肾淀粉样变性。A 64⁃year⁃old male patient presented as nephrotic syndrome without hypertension and renal function impairment.Blood immunofixation electrophoresis showed λ IgA monoclonal immunoglobulin bands,and blood free light chain examination showed a decreased freeκ/λratio.Bone marrow flow cytometry showed 4.52% monoclonal plasma cells.He was diagnosed with AL type renal amyloidosis by renal biopsy.The patient achieved clinical VGPR after chemotherapy and repeat renal biopsy showed renal AL amyloidosis with prominent multinuclear giant cell formation.

关 键 词：轻链型淀粉样变性 多核巨细胞 肾活检 

分 类 号：R692[医药卫生—泌尿科学]