单克隆免疫球蛋白相关肾小管间质疾病诊治进展  

Advances in the pathological diagnosis and therapy of monoclonal immunoglobulin-related tubulointerstitial diseases

作　　者：王素霞[1] WANG Su-xia(Laboratory of Electron Microscopy, Pathological Centre, Peking University First Hospital, Beijing 100034, China)

机构地区：[1]北京大学第一医院病理中心电镜室,北京100044

出　　处：《中国实用内科杂志》2018年第6期511-514,共4页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金面上项目(81470956)

摘　　要：单克隆免疫球蛋白相关肾小管间质疾病主要指单克隆轻链(κ,λ)所导致的肾小管间质病变,包括轻链相关肾小管损伤及肾小管坏死、轻链相关肾小管间质性肾炎、轻链近端肾小管病及轻链管型肾病等四类疾病。临床上以肾功能不全为主要特征,可伴有小分子为主的蛋白尿;病理以单克隆轻链在肾小管上皮胞浆、肾小管基底膜及管型沉积为共同特征;电镜与免疫电镜证实单克隆轻链在上皮胞质溶酶体及其结晶等包涵体内的蓄积,具有重要的诊断价值。轻链导致不同的病理损伤类型,可能与轻链的负荷量、轻链的特定基因亚型及其不同的结构变异有关,详细的发病机制有待进一步研究。Monoclonal immunoglobulin-related tubulointerstitial diseases is mainly caused by monoclonal light chains（κ,λ）, which includes light chain associated tubular injury and tubular necrosis, light chain-mediated tubulointerstitial nephritis, light chain proximal tubulopahty, and light chain cast nephropathy. The main clinical manifestation is renal insufficiency with small molecular proteinuria; the deposition of monoclonal light chain in the tubular epithelial cytoplasm, tubular basement membrane and casts is the common feature of all these diseases, the identification of monoclonal light chain in the cytoplasmic lysosome and crystal inclusions by electron microscopy and immmuno-electron labeling was of great value for the diagnosis. The diversity of renal lesions may be determined by the burden of light chains, the specific germline genotype and the structural alteration of light chains, and the pathogenetic mechanism underlying the different patterns of light chain-related tubulointerstitial diseases needs further study.

关 键 词：免疫球蛋白 轻链 近端肾小管病 管型肾病 免疫电镜 

分 类 号：R692.33[医药卫生—泌尿科学]