Abstract:

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. 

Key words:

Renal failure, Renal light chain deposition disease, Multiple plasma lumen effusion, Bortezomib, Diagnosis and treatment

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。

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