Park Kootae, Kwon Soon Hyo
Division of Nephrology, Hyonam Kidney Laboratory, Soonchunhyang University Hospital, 59 Daesagwan-Ro, Yongsan-Gu, Seoul, South Korea.
Blood Res. 2024 Aug 12;59(1):28. doi: 10.1007/s44313-024-00027-5.
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.
肾脏疾病是多发性骨髓瘤和其他与单克隆丙种球蛋白病相关的恶性肿瘤的常见并发症。此外，与异常蛋白血症相关的肾脏疾病可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相关的肾脏疾病有多种表现形式，包括免疫球蛋白相关淀粉样变性、单克隆免疫球蛋白沉积病（轻链、重链以及轻链和重链联合沉积病）、伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎、伴有单克隆丙种球蛋白病的C3肾小球病以及轻链近端肾小管病。尽管MGRS是一种非恶性或癌前血液系统疾病，但它对肾脏有重大影响，常常导致进行性肾脏损害，并最终发展为终末期肾病。本综述讨论了MGRS的流行病学、发病机制和管理，并重点从肾病学家的角度进行阐述。
