本文選題：淀粉樣變性　切入點：心肌　出處：《吉林大學》2015年碩士論文　論文類型：學位論文

【摘要】：研究目的：總結心肌淀粉樣變的患者臨床表現及輔助檢查的特點，以提高臨床對心肌淀粉樣變的認識。提高其診斷率。 研究方法：回顧分析2007年9月--2014年11月就診于吉林大學第一醫(yī)院心內科的患者4例，年齡為50—76歲，所有的患者均排除其他嚴重心、肝、腎、肺等疾病。所有患者均進行詳細的臨床資料收集，包括：一般情況、臨床癥狀及體格檢查等，行血、尿、肝、腎等常規(guī)實驗室檢查，及超聲心動圖、心電圖、免疫固定電泳、24h尿輕鏈檢測、骨髓穿刺+活檢、心肌、腎臟、肝臟、腹壁、頸部等部位活組織檢查結果，考慮是否合并有其他漿膜腔積液等情況，為患者進行心衰美國紐約心臟病學會分級，并經過病理學檢測：鏡檢：HE染色，電鏡檢測，特殊染色：剛果紅染色，偏振光及免疫組化(Max Vision法等)、流式細胞儀技術等病理學檢查。 研究結果：1、所有患者均行病理學檢測：⑴活檢組織經過HE染色呈粉紅色、無定形、蠟樣、有特征性裂紋；電鏡下可見隨機排列的原纖維結構；沉積物周圍漿細胞增多并可能表達與淀粉樣沉積物一致的輕鏈限制性。⑵剛果紅染色：呈磚紅色、偏振光下呈特征性的雙色性和蘋果綠雙折射。⑶免疫組化檢：λ輕鏈陽性。經過流式細胞儀檢測：骨髓漿細胞的免疫球蛋白輕鏈限制性及異常表型特征檢測異常漿細胞克隆。2、4例病例中均因胸悶、呼吸困難起病，，且均有右心功能不全的表現（頸靜脈怒張、雙下肢水腫等），及蛋白尿等表現，其中因淀粉樣變所致的2例患者有肝臟增大，心律失常等特點。少數患者出現皮膚瘀點、瘀斑等臨床表現的。心肌淀粉樣變性為多系統疾病，單純抗心力衰竭治療無效，常聯合腎臟損害和肺臟、肝臟等多器官損害；3、2例患者存在中有心包積液，2例患者腹腔積液，2例患者胸腔積液，其中有3例患者同時合并多漿膜腔積液。表明多數患者有合并多漿膜腔積液的情況。4、所有患者均出現左房增大，其中2例患者合并右房增大，3例患者出現室間隔厚度增厚、左室后壁增厚，尚無患者出現左室增大。超聲心動圖多顯示，單純的心房增大，而心室腔不大或心室壁出現閃耀的顆粒狀回聲。多出現房間隔增厚和室間隔增厚；4例患者均出現24h尿輕鏈改變。 結論：1、心臟淀粉樣變性的患者臨床特點多表現為舒張性心力衰竭。2、心臟淀粉樣變性的患者心電圖多表現為肢體導聯低電壓，超聲心動圖可出現心肌回聲增強(顆粒閃耀)，或表現為心肌肥厚。心電圖的表現與心臟彩超表現矛盾。3、心臟淀粉樣變性的患者多合并蛋白尿等多器官功能損害。4、淀粉樣變性的臨床特征的病理基礎是淀粉樣蛋白的沉積導致占位和組織損傷了。
[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.
【學位授予單位】：吉林大學
【學位級別】：碩士
【學位授予年份】：2015
【分類號】：R597.2

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本文編號：1615428