AL型淀粉样变性病20例临床特点分析  

Analysis of Clinical Characteristics in 20 Patients with AL Amyloidosis

作　　者：刘丁[1] 刘刚[2] 

机构地区：[1]北京监狱管理局中心医院内科 [2]北京大学第一医院肾内科

出　　处：《首都医科大学学报》2010年第3期416-419,共4页Journal of Capital Medical University

摘　　要：目的本研究旨在深入了解淀粉样变性病患者多系统受累的临床特点,提高对AL型淀粉样变性病的认识。方法研究对象为1999年1月至2009年1月在北京监狱管理局中心医院及北京大学第一医院确诊的20例AL型肾淀粉样变性病患者,所有患者均经过活检病理检查刚果红染色阳性确诊(舌、皮肤、肾、直肠黏膜)淀粉样变性病,经免疫荧光或免疫组化确定为AL型。对其临床表现及实验室检查资料进行了回顾性分析。结果 1)90%患者发病年龄>40岁,男女比例3∶2。90%的患者有2个或以上系统受累,3个系统以上受累占30%(6/20)。一般表现乏力者占45%(9/20),体质量下降者占40%(8/20)。2)循环系统呈现低血压趋势;超声心动图检查左室壁厚度(1.12±0.34)cm,室间隔厚度或左室壁厚度>1.1cm者占35%(7/20),40%(8/20)患者有左室舒张功能受损表现,15%(3/20)患者发现颗粒样心肌回声增强。心电图异常者占80%(16/20)。3)消化系统症状达75%;消化道出血者占30%(6/20);舌体肥大者占25%(5/20);肝脏肿大者占35%(7/20)。4)肾脏病变:90%(18/20)的患者表现为肾病综合征;85%(17/20)患者有肾小管损伤;肾脏增大者(长径>12cm)占40%(8/20)。5)血液系统病变:85%(17/20)患者血和尿中免疫固定电泳(immunofixation electrophoresis,IFE)发现同类型的单克隆免疫球蛋白轻链,其中λ轻链15例,κ轻链2例。6)神经系统病变:25%(5/20)患者出现神经系统表现。结论 AL型淀粉样变性病临床呈多系统受累,消化器官、肾脏、心脏为常见的靶器官,本病临床表现复杂但有临床特点可循。Objective To realize the clinical characteristics of multi-system involvement in patients with AL amyloidosis. Methods The clinical data of 20 patients who were diagnosed as having renal AL amyloidosis,by immunohistochemical method on renal biopsy sections were retrospectively analyzed. Results Of the 20 patients,90% of were over 40 years old. The ratio of male to female was 3：2. Two or more systems involvement was found in 90% of patients and 30% had more then three systems involved. In this group kidney involvement was found in 100%（nephrotic syndrome 90%）,hypotension in 45%,thickened ventricular walls in 35%,abnormal electrocardiogram in 80%,hepatomegaly in 35%,glossal hypertrophy in 25%,body weight loss in 40%,monoclonal light chain in serum or urine in 85% of patients. Conclusion AL amyloidosis often involves multiple systems,including digestive system,kidney and heart were the common target organs.The clinical characteristics of the disease are complicated.

关 键 词：AL淀粉样变性病 单克隆免疫球蛋白轻链 临床特点 

分 类 号：R5[医药卫生—内科学]