单克隆免疫球蛋白相关肾损害的诊治进展

Advance in diagnostic and therapeutic strategies of monoclonal gammopathy of renal significance

单克隆免疫球蛋白(MIg)或其片段导致的肾脏损害,称为单克隆免疫球蛋白相关肾损害(MGRS)。过去,由于MGRS血液学改变更接近意义未明的单克隆免疫球蛋白病(MGUS),不主张对除AL淀粉样变性以外的MGRS进行治疗。但成功清除分泌MIg的细胞克隆能够保护肾功能,进展至终末期肾病的MGRS患者体内MIg水平与肾移植术后MGRS复发有关,获得血液学完全缓解能够避免移植肾失功,这些研究结果均表明MGRS并非意义未明,而必须积极治疗。本文主要就MGRS诊断及治疗的研究进展加以综述。

Monoclonal gammopathy of renal significance（ MGRS） is defined by the causal relationship between a small B-cell clone and renal disease,usually through deposition of the secreted monoclonal immunoglobulin（ MIg） or a fragment of MIg. In the past,for the hematologic disorder in patients with MGRS was consistent with monoclonal gammopathy of undetermined significance（ MGUS） and the treatment was not recommended for MGUS,therapy for patients with MGRS was commonly withheld. However,the facts that preservation and restoration of kidney function are possible with successful treatment targeting the responsible clone,that the persistence of the monoclonal gammopathy is associated with high rates of recurrence after kidney transplantation in patients with end stage renal disease,and that achievement of hematologic complete response has been shown to prevent recurrence after kidney transplantation. Those data demonstrate that MGRS is no longer undetermined and necessary to achieve appropriate therapy. Here,we summarize progression of diagnosis and treatment of MGRS.