轻链沉积病肾损害的诊断与治疗  

Diagnosis and treatment of light chain deposition disease with renal involvement

作　　者：林珊[1] 贾俊亚[1] 

机构地区：[1]天津医科大学总医院肾内科,300052

出　　处：《中华肾病研究电子杂志》2015年第5期14-18,共5页Chinese Journal of Kidney Disease Investigation（Electronic Edition）

基　　金：国家自然科学基金(81270791);天津市卫生局重点攻关课题(11KG132);天津市应用基础及前沿计划(14JCYBJC27900)

摘　　要：轻链沉积病(LCDD)为单克隆免疫球蛋白轻链在肾脏、心脏、肝脏等组织的沉积。LCDD肾损害起病时多伴有慢性肾功能不全。典型的LCDD肾损害有相对特殊的临床表现,血、尿蛋白电泳及游离轻链定量有助于诊断,确诊需行肾组织病理学检查,包括光镜、荧光显微镜及电镜等检查。LCDD肾损害的治疗应综合考虑B细胞增殖程度和单克隆免疫球蛋白对肾功能造成的损害。应积极控制B细胞增殖,但治疗时应选择肾毒性较小的药物如硼替佐米,必要时改用大剂量马法兰+周围血干细胞移植(HDM/ASCT)方案。Light chain deposition disease （LCDD）, belonging to monoclonal gammopathy, refers to monoclonal immunoglobulin light chain deposition in kidney, heart, liver, and other tissues. Most LCDD patients with renal involvement at onset have chronic renal function insufficiency. Typical LCDD-related kidney damage presents relatively specific clinical manifestations. The serum or urine protein electrophoresis and quantitative analysis of free light chains are helpful in diagnosis of LCDD. LCDD-related kidney damage can be confirmed by light microscopy, fluorescence microscopy, and electron microscopy examinations. The treatment of LCDD-related renal impairment should be given in consideration of the degree of B cell proliferation and the renal function impairment caused by monoclonal immunoglobulins. B cell proliferation should be controlled actively with less toxic agents to the kidney such as bortezomib or, if necessary, with a high dose of melphalan plus peripheral blood stem cell transplantation （HDM/PBSCT） pro~＇am.

关 键 词：轻链沉积病 单克隆丙种球蛋白病 硼替佐米 

分 类 号：R692[医药卫生—泌尿科学]