心脏淀粉样变性的临床特点和病理学研究

发布时间：2018-03-15 09:28

本文选题：淀粉样变性　切入点：心肌　出处：《吉林大学》2015年硕士论文　论文类型：学位论文

【摘要】：研究目的：总结心肌淀粉样变的患者临床表现及辅助检查的特点，以提高临床对心肌淀粉样变的认识。提高其诊断率。研究方法：回顾分析2007年9月--2014年11月就诊于吉林大学第一医院心内科的患者4例，年龄为50—76岁，所有的患者均排除其他严重心、肝、肾、肺等疾病。所有患者均进行详细的临床资料收集，包括：一般情况、临床症状及体格检查等，行血、尿、肝、肾等常规实验室检查，及超声心动图、心电图、免疫固定电泳、24h尿轻链检测、骨髓穿刺+活检、心肌、肾脏、肝脏、腹壁、颈部等部位活组织检查结果，考虑是否合并有其他浆膜腔积液等情况，为患者进行心衰美国纽约心脏病学会分级，并经过病理学检测：镜检：HE染色，电镜检测，特殊染色：刚果红染色，偏振光及免疫组化(Max Vision法等)、流式细胞仪技术等病理学检查。研究结果：1、所有患者均行病理学检测：⑴活检组织经过HE染色呈粉红色、无定形、蜡样、有特征性裂纹；电镜下可见随机排列的原纤维结构；沉积物周围浆细胞增多并可能表达与淀粉样沉积物一致的轻链限制性。⑵刚果红染色：呈砖红色、偏振光下呈特征性的双色性和苹果绿双折射。⑶免疫组化检：λ轻链阳性。经过流式细胞仪检测：骨髓浆细胞的免疫球蛋白轻链限制性及异常表型特征检测异常浆细胞克隆。2、4例病例中均因胸闷、呼吸困难起病，，且均有右心功能不全的表现（颈静脉怒张、双下肢水肿等），及蛋白尿等表现，其中因淀粉样变所致的2例患者有肝脏增大，心律失常等特点。少数患者出现皮肤瘀点、瘀斑等临床表现的。心肌淀粉样变性为多系统疾病，单纯抗心力衰竭治疗无效，常联合肾脏损害和肺脏、肝脏等多器官损害；3、2例患者存在中有心包积液，2例患者腹腔积液，2例患者胸腔积液，其中有3例患者同时合并多浆膜腔积液。表明多数患者有合并多浆膜腔积液的情况。4、所有患者均出现左房增大，其中2例患者合并右房增大，3例患者出现室间隔厚度增厚、左室后壁增厚，尚无患者出现左室增大。超声心动图多显示，单纯的心房增大，而心室腔不大或心室壁出现闪耀的颗粒状回声。多出现房间隔增厚和室间隔增厚；4例患者均出现24h尿轻链改变。结论：1、心脏淀粉样变性的患者临床特点多表现为舒张性心力衰竭。2、心脏淀粉样变性的患者心电图多表现为肢体导联低电压，超声心动图可出现心肌回声增强(颗粒闪耀)，或表现为心肌肥厚。心电图的表现与心脏彩超表现矛盾。3、心脏淀粉样变性的患者多合并蛋白尿等多器官功能损害。4、淀粉样变性的临床特征的病理基础是淀粉样蛋白的沉积导致占位和组织损伤了。
[Abstract]:Objective: to summarize the clinical manifestations of myocardial amyloidosis and the characteristics of auxiliary examination in order to improve the clinical understanding of myocardial amyloidosis and improve the diagnostic rate of myocardial amyloidosis. Methods: from September 2007 to November 2014, 4 patients, aged 50-76 years, in Department of Cardiology, first Hospital of Jilin University, were retrospectively analyzed. All patients were excluded from other severe heart, liver and kidney. All patients underwent detailed clinical data collection, including general conditions, clinical symptoms and physical examination, routine laboratory examinations such as blood, urine, liver, kidney, and echocardiography, electrocardiogram, and so on. Immunofixation electrophoresis for 24 hours urinary light chain detection, bone marrow biopsy, myocardial, kidney, liver, abdominal wall, neck and other living tissue examination results, to consider whether there are other serous cavity effusion, etc. The heart failure patients were graded by the New York College of Cardiology and examined by pathology: microscopic examination: he staining, electron microscope examination, special staining: Congo red staining, polarizing light and immunohistochemical Vision method, flow cytometry and other pathological examination. Results: 1. All the patients underwent pathological examination. The biopsy tissues were stained pink, amorphous, waxy, with characteristic cracks by HE staining, and randomly arranged fibrillar structures were observed under electron microscope. The proliferation of plasma cells around the sediment and the possible expression of light chain restricted .2 Congo red staining consistent with amyloid sediments: brick red, Characteristic bichromatic and apple green birefringence immunohistochemical examination: 位 light chain positive. Flow cytometry: immunoglobulin light chain restriction and abnormal phenotypic characteristics of bone marrow plasmacyte detected abnormal plasma fine. In the 4 cases of cell clone, all of them were due to chest tightness. The symptoms of dyspnea and right ventricular insufficiency (jugular vein irritation, edema of both lower limbs, proteinuria, etc.) were found in all patients, among whom 2 patients with amyloidosis had liver enlargement. Arrhythmia and other characteristics. A few patients with skin stasis, ecchymosis and other clinical manifestations. Myocardial amyloidosis is a multi-system disease, simple anti-heart failure treatment, often combined with kidney damage and lung, There were 2 cases of pericardial effusion and 2 cases of peritoneal effusion in 2 patients with liver and other multiple organ damage, 2 patients with pleural effusion. There were 3 patients with polyserosal effusion at the same time, which indicated that most patients had multiple serous cavity effusion, all patients had left atrial enlargement, 2 patients with right atrial enlargement and 3 patients with ventricular septal thickness thickening. Left ventricular posterior wall thickened, no patient had left ventricular enlargement. However, there were small ventricular lumen or glittering granular echo in ventricular wall, and 4 patients with atrial septal thickening and ventricular septal thickening all had 24-hour urinary light chain changes. Conclusion the clinical features of patients with amylloidosis of heart are diastolic heart failure .2.The electrocardiogram of patients with cardiac amyloidosis is characterized by low voltage of limb lead. Echocardiography may show enhanced myocardial echo (granulosa blazing, or myocardial hypertrophy). The electrocardiogram (ECG) is inconsistent with the color Doppler echocardiography (CDFI), and the patients with cardiac amyloidosis may have multiple organ dysfunction, such as proteinuria, etc. The pathological basis of the clinical features of amyloidosis is that the deposition of amyloid protein leads to space occupying and tissue damage.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R597.2

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