目的:探讨轻链型淀粉样变性(AL型)淀粉样物质在肾组织沉积部位及程度与临床表现及预后的关系。方法:回顾分析经肾活检确诊的AL型淀粉样变性患者的临床病理资料,利用全切片扫描技术计算淀粉样物质占组织的百分比,分析其与临床及预后的关系。结果:AL型淀粉样变性患者259例,男性147例,中位年龄57岁,肾脏均受累,肠道受累71.06%,心脏受累43.22%。中位病程7月,蛋白尿4.80±3.15g/24h,19.31%的患者肾功能不全。中位随访时间22.3月,患者平均生存时间24.24月,1、2、3和5年人生存率分别为81.8%、64.1%、52.4%和29.6%;肾脏生存率分别为90.8%、82.3%、76.8%和47.8%。淀粉样物质累及肾小球(99.61%),肾血管(92.66%)和肾间质(50.19%)。淀粉样物质占肾小球、肾血管、肾间质及整个肾组织的百分比分别为11.81%±11.38%、14.14%±14.05%、3.34%±5.36%和4.25%±5.77%,且与多项临床指标具有相关性。淀粉样物质占肾小球百分比、年龄、尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG)及尿视黄醇结合蛋白(RBP)为患者预后的独立危险因素。淀粉样物质占肾血管、肾间质及整个肾组织百分比,血清肌酐,尿RBP为肾脏预后的独立危险因素。结论:全切片扫描定量分析是观察淀粉样物质在肾脏不同部位沉积及其程度的有效方法,并证实蛋白尿、血清肌酐等与淀粉样物质肾脏不同部位沉积有一定相关性。肾脏不同部位淀粉样物质沉积与患者预后和肾脏预后相关。 Objective: To investigate the relationship between light chain amyloid (AL) amyloid deposition location and degree in renal tissue and clinical manifestations and prognosis. Methods: The clinicopathological data of patients with AL amyloidosis diagnosed by renal biopsy were retrospectively analyzed. The percentages of amyloid in the tissue were calculated by the whole slice scanning technique, and their relationship with clinical and prognosis was analyzed. Results: There were 259 cases of AL amyloidosis in 147 males, with a median age of 57 years. All patients had renal involvement, 71.06% of intestinal tract involvement and 43.22% of cardiac involvement. Median duration of July, albuminuria 4.80 ± 3.15g / 24h, 19.31% of patients with renal insufficiency. The median follow-up time was 22.3 months. The average survival time of patients was 24.2 months. The survival rates of patients with 1, 2, 3 and 5 years were 81.8%, 64.1%, 52.4% and 29.6% respectively. The renal survival rates were 90.8% and 82.3% 76.8% and 47.8%. Amyloid involved glomeruli (99.61%), renal vessels (92.66%) and renal interstitium (50.19%). The percentages of amyloid in glomeruli, renal vessels, interstitium and whole kidney tissue were 11.81% ± 11.38%, 14.14% ± 14.05%, 3.34% ± 5.36% and 4.25% ± 5.77%, respectively, Clinical indicators are relevant. The percentage of amyloid in glomeruli, age, urinary N-acetylglucosaminidase (NAG) and urinary retinol binding protein (RBP) were independent prognostic factors in patients. The amyloid accounted for renal vascular, interstitial and the percentage of renal tissue, serum creatinine and urine RBP were independent risk factors for renal prognosis. Conclusion: Quantification of whole-slice scanning is an effective method to observe the deposition and extent of amyloid in different parts of kidney and confirm that proteinuria, serum creatinine and so on are related to deposition of different parts of amyloid kidneys. Amyloid deposition in different parts of the kidney correlates with patient prognosis and kidney prognosis.