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目的了解轻链蛋白沉积造成肾损害的特点,提高对其发病的认识。方法回顾分析中国医科大学附属第一医院2006年9月至2007年12月多发性骨髓瘤(MM)和浆细胞肾损害(共9例),以及肾淀粉样变(3例,均为原发、AL)的临床特点与病理特征。结果MM导致肾功能衰竭4例,肾病综合征2例。浆细胞病3例(1例浆细胞性肾病)中,2例存在肾功能衰竭。大部分MM与浆细胞病血清和(或)尿液中存在kappa、lambda轻链蛋白代谢异常(7/9)。3例AL均表现为典型的肾病综合征,肾脏病理均显示lambda轻链蛋白主要沉积在肾小球系膜区、基底膜、肾小管基底膜及小动脉壁。结论轻链蛋白代谢异常均可引起不同程度的肾脏损伤,产生多样的临床表现。
Objective To understand the characteristics of light chain protein deposition caused by renal damage and improve their understanding of the disease. Methods Retrospective analysis of multiple myeloma (MM) and plasma cell renal damage (9 cases) in the First Affiliated Hospital of China Medical University from September 2006 to December 2007, and renal amyloidosis (3 cases, both primary , AL) clinical features and pathological features. Results MM led to renal failure in 4 cases, nephrotic syndrome in 2 cases. Plasma cell disease in 3 cases (1 cases of plasma cell nephropathy), 2 cases of renal failure. Most MM and plasma cell disease serum and (or) urine kappa, lambda light chain protein metabolism (7/9). Three cases of AL showed typical nephrotic syndrome, renal pathology showed that lambda light chain protein mainly deposited in the mesangial area, the basement membrane, the basement membrane and the small artery wall. Conclusion The abnormal light chain protein metabolism can cause different degrees of renal damage, resulting in a variety of clinical manifestations.