肾轻链沉积病伴多浆膜腔积液1例  

A case of renal light chain deposition disease complicated with multiple plasma chamber effusion

作　　者：徐田田 Xu Tiantian(Department of Hematology,The First Affiliated Hospital of Jinan University,Guangzhou 510630,China)

机构地区：[1]暨南大学附属第一医院血液内科,广州510630

出　　处：《国际医药卫生导报》2024年第9期1552-1555,共4页International Medicine and Health Guidance News

摘　　要：轻链沉积病(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全身性疾病。肾脏受累几乎涉及所有LCDD患者,肾外受累则相对较少,其中心脏、肝脏、胃肠道、周围神经和自主神经受累较为常见,肺部受累非常罕见。LCDD脏器功能受损多数呈进行性发展,罕有自发缓解。肾外轻链沉积对患者生存率及预后有明确的独立影响。Light chain deposition disease(LCDD)is the most common form of monoclonal immunoglobulin deposition disease,in which plasma cells or B-lymphocytes secrete excessive amounts of monoclonal immunoglobulin light chains,which are deposited in tissues and cause organ dysfunction.LCDD is a systemic disease with multiple organ involvement,mainly in the kidney.Renal involvement is common in almost all patients with LCDD,while extra-renal involvement is less common,with cardiac,hepatic,gastrointestinal,peripheral,and autonomic nerve involvement being more common,and pulmonary involvement being very rare.Most of the organ impairment in LCDD is progressive,and spontaneous remission is rare.Extra-renal light chain deposition has clear independent effect on the patients'survival and prognosis.

关 键 词：肾功能衰竭 肾轻链沉积病 多浆膜腔积液 硼替佐米 诊断及治疗 

分 类 号：R692.5[医药卫生—泌尿科学]