轻链沉积病的临床诊治进展  

Progress in clinical diagnosis and treatment of light chain deposition disease

作　　者：沈扬[1] 张丽红[1] SHEN Yang;ZHANG Lihong(Department of Hematology,the First Hospital of Hebei Medical University,Hebei Shijiazhuang 050031,China)

机构地区：[1]河北医科大学第一医院血液内科,河北石家庄050031

出　　处：《现代肿瘤医学》2023年第21期4050-4054,共5页Journal of Modern Oncology

基　　金：河北省卫生健康委医学科学研究课题(编号:20201150)。

摘　　要：轻链沉积病(light chain deposition disease,LCDD)是最多见的单克隆免疫球蛋白沉积病,由浆细胞或B淋巴细胞分泌过量单克隆免疫球蛋白轻链,沉积在组织中引起器官功能障碍。LCDD多表现为以肾脏为首的多器官受累的全身性疾病。本文由LCDD的内涵与外延引入,从器官系统受累的临床表现与评估、病理学特征与诊断、血液学特征与检测、治疗策略与方案等方面,就近年来LCDD临床诊断与治疗的进展作一综述。Light chain deposition disease(LCDD)is the most common monoclonal immunoglobulin deposition disease.Excessive monoclonal immunoglobulin light chain is secreted by plasma cells or B lymphocytes and deposited in tissues,resulting in organ dysfunction.LCDD is a systemic disease with multiple organ involvement led by kidney.Introduced from the connotation and extension of LCDD,this paper summarizes the progress of clinical diagnosis and treatment of LCDD in recent years from the aspects of clinical manifestations and evaluation of organ system involvement,pathological characteristics and diagnosis,hematological characteristics and detection,treatment strategies and protocols.

关 键 词：轻链沉积病 单克隆免疫球蛋白沉积病 具有肾脏意义的单克隆免疫球蛋白病 游离轻链 达雷妥尤单抗 CyBorD 诊断 治疗 

分 类 号：R730.5[医药卫生—肿瘤]