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80年代前,对免疫球蛋白轻链相关肾脏病(Monoclonal light chain-related renaldiseases,MLCRRD)的认识还仅仅局限于骨髓瘤肾病。近年来,随着肾组织免疫球蛋白轻链常规染色镜检,以及免疫电镜的广泛开展,MLCRRD已具有了更为广泛的含义。目前认为,MLCRRD是免疫球蛋白的轻链成分在肾小球、肾间质、肾小管、肾血管广泛沉积而造成的一组病症的总称(见表1)。尽管MLCRRD病理变化多样,但现有的诊断手段(免疫固定电泳、骨髓免疫学检查,以及肾组织免疫球蛋白轻链染色镜检等)已可早期诊断该病。通过检测肾组织中免疫球蛋白轻链成分的沉积,也可从肾脏病角度首先认识浆细胞异常增生的疾病。现就MLCRRD肾脏损害的病理类型及其近年研究进展作一概述。
Before the 1980s, the understanding of MLCRRD was limited to myeloma nephropathy. In recent years, with renal tissue immunoglobulin light chain conventional staining microscopy, and immunoelectron microscopy widely carried out, MLCRRD has a more extensive meaning. It is currently believed that MLCRRD is a collective term for a group of disorders caused by the extensive deposition of immunoglobulin light chain components in glomeruli, renal interstitium, renal tubules, and renal vessels (see Table 1). Although MLCRRD pathological changes, but the existing diagnostic tools (immunoaffinity electrophoresis, bone marrow immunology, and renal tissue immunoglobulin light chain staining, etc.) have been early diagnosis of the disease. By detecting the deposition of immunoglobulin light chain components in the kidney tissue, it is also possible to first recognize the diseases of the abnormal proliferation of plasma cells from the perspective of the kidney disease. Now on the pathological types of MLCRRD kidney damage and its recent progress in research are summarized.