Sanders P W, Herrera G A
Department of Medicine, University of Alabama, Birmingham 35294-0007.
Semin Nephrol. 1993 May;13(3):324-41.
The spectrum of renal lesions associated with deposition of monoclonal light chains is varied and extensive, yet the simultaneous occurrence of two or more of these lesions in the same patient is unusually recognized. In addition, patients may excrete large amounts of monoclonal light chains and have no clinically evident renal dysfunction. Thus, the type of renal lesion that occurs appears to depend on the physiochemical properties of the unique monoclonal light chain produced. The particular nephrotoxic properties responsible have not yet been identified but are currently under intensive examination. For example, various studies suggest that cast-forming Bence Jones proteins have isoelectric points above 5.1. Finally, along with these intrinsic properties, environmental factors combine to promulgate the lesion or prevent clinical expression of renal injury. Attention to the subtle morphological renal manifestations of MLCRRD is essential to diagnose these diseases early in the course. Characterization of the morphological pattern and degree of renal affectation is important because therapeutic modalities may vary accordingly. As understanding of the molecular events responsible for the renal alterations improves, additional therapeutic approaches may emerge to reverse renal failure in MLCRRD.
与单克隆轻链沉积相关的肾脏病变范围广泛且多样，但同一患者同时出现两种或更多种此类病变的情况却不常被认识到。此外，患者可能排泄大量单克隆轻链但并无明显的临床肾功能障碍。因此，所发生的肾脏病变类型似乎取决于所产生的独特单克隆轻链的理化性质。造成这种情况的特定肾毒性特性尚未确定，但目前正在深入研究。例如，各种研究表明，形成管型的本-周蛋白等电点高于5.1。最后，除了这些内在特性外，环境因素也共同促使病变发生或阻止肾损伤的临床表现。关注轻链沉积相关肾病（MLCRRD）细微的肾脏形态学表现对于在病程早期诊断这些疾病至关重要。肾脏形态学模式和受累程度的特征描述很重要，因为治疗方式可能会因此而有所不同。随着对导致肾脏改变的分子事件的理解不断提高，可能会出现更多治疗方法来逆转MLCRRD中的肾衰竭。
