肾意义单克隆免疫球蛋白血症的诊治进展

Research Progress of the Diagnosis and Treatment in Monoclonal Gammopathy of Renal Significance

肾意义单克隆免疫球蛋白血症(MGRS)是IKMG(国际肾病和单克隆免疫球蛋白病研究组)在2012年定义的一组单克隆免疫球蛋白相关肾损害的疾病,其克隆来源于低度增殖的B细胞或浆细胞克隆。研究发现其进展为多发性骨髓瘤(MM)的风险高于MGUS,经过针对其相应克隆化疗后肾功能可得到改善。MGRS不同于意义未明的单克隆免疫球蛋白血症(MGUS),也不符合淋巴瘤或MM的诊断标准。2019年IKMG发表了关于MGRS的诊断共识,提出对小于50岁存在M蛋白血症和无法解释肾脏疾病的患者,建议进行肾活检,以明确MGRS的诊断。关于MGRS的治疗决策应遵循包括血液学家、肾病学家和病理学家参与的多学科合作模式来制定,通常采用可以清除引起肾损害的单克隆Ig的B细胞或浆细胞克隆的靶向药物基础的化疗,符合条件的患者造血干细胞移植治疗值得尝试。

Monoclonal gammopathy of renal significance(MGRS) is a group of monoclonal immunoglobulin(Ig)-associated renal lesions defined by International Nephrology and Monoclonal Immunoglobulin Disease Research Group(IKMG) in 2012. Clones of MGRS are derived from low proliferating B cell or plasma cell clones. Studies have found that the risk of progression to multiple myeloma(MM) is higher than that of monoclonal gammopathy of undetermined significance(MGUS),and their renal function can be improved after treatment with anti-corresponding clones.MGRS does not meet the diagnostic criteria for lymphoma or MM. In 2019,IKMG proposed a diagnostic consensus on MGRS,suggesting that the diagnostic workup is recommended to determine the diagnosis of MGRS,for patients younger than 50 years with monoclonal M proteinemia and unexplained kidney disease,especially for renal biopsy,it is essential for the diagnosis of MGRS.There is currently no consensus on the treatment of MGRS. However,treatment decisions should be based on a multidisciplinary model of cooperation involving hematologists,nephrologists,and pathologists.It is commonly used to protect kidney function and prevent recurrence for targeted chemotherapy that removes B cells or plasma cell clones of monoclonal Ig that cause kidney damage. In eligible patients,hematopoietic stem cell transplantation therapy is worth trying.