PDF(Pubmed)
Abstract：
Kidney disease is a frequent complication of multiple myeloma and other malignancies associated with monoclonal gammopathies. Additionally, dysproteinemia-related kidney disease can occur independently of overt multiple myeloma or hematologic malignancies. Monoclonal gammopathy of renal significance (MGRS) is a spectrum of disorders in which a monoclonal immunoglobulin produced by a benign or premalignant B-cell or plasma cell clone causes kidney damage. MGRS-associated renal disease manifests in various forms, including immunoglobulin-associated amyloidosis, monoclonal immunoglobulin deposition diseases (light chain, heavy chain, and combined light and heavy chain deposition diseases), proliferative glomerulonephritis with monoclonal immunoglobulin deposits, C3 glomerulopathy with monoclonal gammopathy, and light chain proximal tubulopathy. Although MGRS is a nonmalignant or premalignant hematologic condition, it has significant renal implications that often lead to progressive kidney damage and, eventually, end-stage kidney disease. This review discusses the epidemiology, pathogenesis, and management of MGRS and focuses on the perspective of nephrologists.
摘要：
肾脏疾病是多发性骨髓瘤和其他与单克隆丙种球蛋白相关的恶性肿瘤的常见并发症。此外,异常蛋白血症相关的肾脏疾病可以独立于明显的多发性骨髓瘤或血液系统恶性肿瘤而发生。具有肾脏意义的单克隆丙种球蛋白病（MGRS）是一系列疾病，其中由良性或癌前B细胞或浆细胞克隆产生的单克隆免疫球蛋白会导致肾脏损伤。MGRS相关的肾脏疾病表现为多种形式，包括免疫球蛋白相关性淀粉样变性,单克隆免疫球蛋白沉积疾病(轻链，沉重的链条，以及轻链和重链沉积疾病的组合)，增殖性肾小球肾炎与单克隆免疫球蛋白沉积，C3肾小球病伴单克隆丙种球蛋白病，和轻链近端肾小管病。尽管MGRS是非恶性或恶性前血液学疾病，它具有显著的肾脏影响，通常导致进行性肾脏损害，最终,终末期肾病.这篇综述讨论了流行病学，发病机制，和MGRS的管理，并侧重于肾病学家的观点。
