47例肾淀粉样变性病的肾脏临床病理特点

Pathological analysis of forty-seven patients with renal amyloidosis

目的：探讨肾淀粉样变性病的肾脏临床病理特点,为临床诊治提供帮助.方法：回顾分析我院1998年1月～2012年5月收治的47例经肾活检明确诊断肾淀粉样变性病患者的肾脏临床表现,依据淀粉样蛋白沉积部位分型以及肾损害评分等.结果：47例肾淀粉样变性病患者平均年龄56.9±10.2岁（23岁～76岁）,男女比为2.4∶1.免疫球蛋白轻链型肾淀粉样变性病（AL）45例,其中8例伴发多发性骨髓瘤（MM）,血清淀粉样蛋白A型肾淀粉样变性病（AA）2例.AL-λ 41例,AL-κ 4例,两者之比为10.3∶1.AL中有免疫复合物沉积的患者8例,其中IgA肾病6例,膜增生性肾小球肾炎1例,Ⅲ期膜性肾病1例.8例伴发MM的患者均无免疫复合物沉积.尿蛋白定量、镜下血尿、血压与肾脏病理分型、分级无关;血肌酐升高患者15例,病理分型以Ⅳ、Ⅵ为主,共13例,占86.7％,分级均在2、3级组,其淀粉样蛋白在血管壁沉积主要集中在中、重度组,但与淀粉样蛋白在肾间质沉积程度无关.结论：肾淀粉样变性病患者中无免疫复合物沉积者MM的发生率高;尿蛋白定量不能反映肾脏病理的严重程度;血肌酐与病理分型、分级有一定相关.

Objective：To investigate the renal clinical and pathological features of renal amyloidosis,and to provide assistance for clinical diagnosis and treatment.nethods：A retrospective analysis of 47 patients with renal amyloidosis diagnosed in the hospital from January 1998 to May 2012.Patients were grouped according to amyloid deposition in the kidney.Renal histological lesions were scored and grading.Results：The average age of these patients was 56.9±10.2 years old （23-76 years old）.Male patients numbered 33 and female pa- tients numbered 14,and male to female ratio was 2.4：l.Renal immunoglobulin light chain amyloidosis（AL）had 45 cases,including 8 cases of multiple myeloma（MM）,and 2 cases of serum amyloid A-type renal amyloidosis （AA）.Forty one cases of AL-h,and 4 cases of AL-K,the ratio was 10.3：1.Immune complexes deposition was existed in 8 AL patients,including 6 cases of IgA nephropathy, 1 case of membrane nephropathy and 1 case of membranoproliferative glomerulonephritis.Eight eases of MM had no immune complex deposition.There was no relation between the degree of proteinuria and renal pathology.Elevated serum creatinine occurred in 15 cases,mainly including 6 cases of type Ⅳ ,7 cases of type Ⅵ ,including 8 cases of grade 2,7 cases of grade 3.The amyloid deposition on the vessel wall in patients with renal insufficiency was mainly in moderate to se- vere group.Conclusion：Patients with renal amyloidosis without immune complex deposition are associated with a high incidence of MM.The degree of proteinuria do not react to the severity of renal pathology.Serum crea- tinine has a certainly relevance with the renal pathology in patients with renal amyloidosis.