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目的总结原发性淀粉样变的临床和病理改变,提高对该病的认识水平。方法回顾分析2002~2005年经肾活检证实为原发性淀粉样变(AL型)的14例患者的临床特点,实验室检查及肾脏病理检查结果。结果原发性淀粉样变性除累及肾脏外,还可累及肠道、骨髓、心脏等器官。常见的临床表现为蛋白尿(100%)、水肿(79%)、乏力(71%)、体重减轻(43%)、纳差(36%)、低血压(36%)等。辅助检查中主要表现为血浆白蛋白低(100%)、大量蛋白尿(>3.5g/24h)(71%)、血IgG异常(71%)、尿κ升高(100%)、尿λ升高(86%)、心脏彩超异常(82%)、双肾体积偏大(71%)等。肾脏病理:光镜均见肾小球、小管间质及血管淀粉样物质沉积,刚果红染色(+),偏光显微镜下呈苹果绿双折光,高锰酸钾处理后不褪色。免疫荧光肾小球见κ(67%)、λ(100%)沉积,并伴IgG(38%)、IgA(38%)、IgM(85%)、C3(38%)、C4(31%)、C1q(46%)的沉积。结论轻链型淀粉样变并非少见病,其可累及各个脏器。确诊需靠病理检查、刚果红染色、高锰酸钾处理试验。
Objective To summarize the clinical and pathological changes of primary amyloidosis and to raise the level of understanding of the disease. Methods The clinical features, laboratory tests and pathological findings of 14 patients with primary amyloidosis (AL type) confirmed by renal biopsy from 2002 to 2005 were analyzed retrospectively. Results Primary amyloidosis in addition to involving the kidneys, but also involving the intestine, bone marrow, heart and other organs. Common clinical manifestations are proteinuria (100%), edema (79%), fatigue (71%), weight loss (43%), anorexia (36%), hypotension (36%) and so on. Auxiliary examinations showed mainly low serum albumin (100%), massive proteinuria (> 3.5g / 24h) (71%), abnormal serum IgG (71%), elevated urinary kappa (100% High (86%), abnormal color Doppler ultrasound (82%), large kidney volume (71%) and so on. Renal pathology: Glomeruli, tubulointerstitial and vascular amyloid deposits were observed under light microscope. Congo red staining (+), apple green double refraction under polarizing microscope, and no discoloration after treatment with potassium permanganate. IgG (38%), IgA (38%), IgM (85%), C3 (38%) and C4 (31%) were deposited in the immunofluorescent glomeruli, , C1q (46%) deposition. Conclusion Light chain amyloidosis is not a rare disease, which can affect various organs. Confirmed by pathological examination, Congo red staining, potassium permanganate treatment test.