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Diffuse Alveolar-Septal Form of Isolated Pulmonary Amyloidosis Mimicking Lymphangitic Carcinomatosis: A Case Report

侷限性肺部肺泡间隔型類澱粉沈著症以癌性淋巴管炎影像表現-病例報告

摘要


肺部類澱粉沈著症可以是侷限性或是全身性類澱粉沈著症所影響之其中一個器官。它可以三種型態來表現:氣管支氣管型,肺實質結節型與廣泛肺泡間隔型。在此我們報告一位64歲男性因持續乾咳與呼吸急促長達兩個月時間來本院就診。胸腔影像檢查呈現局部肺泡浸潤與廣泛性肺間隔紋路增厚疑似癌性淋巴管炎,經氣管肺切片檢查,病理結果發現切片組織呈現一些無定形嗜伊紅性物質沈積於細胞外,並用剛果紅染色再以偏光鏡下檢查呈現特異性蘋果綠的顏色,隨後再作骨髓穿刺切片並無此發現,其他器官在相關檢查亦無受侵犯。最後診斷為少見之“侷限性肺部肺泡間隔型類澱粉沈著症”。

並列摘要


Pulmonary amyloidosis may be isolated or a part of systemic amyloidosis. It appears in 3 patterns: tracheobronchial, parenchymal nodular, and a diffuse alveolar-septal form. A 64-year-old man presented complaining of a chronic dry cough and dyspnea that had persisted for 2 months. Chest radiographs and a high-resolution computed tomography (HRCT) scan revealed patchy areas of air-space consolidative lesions in the right middle and left lingual lobes of his lungs. There were also diffuse thickening of bronchovascular bundles, intralobular and interlobular septa, and centrilobular opacities in both lungs, mimicking lymphangitic carcinomatosis. Histopathology of a transbronchial biopsy showed some amorphous eosinophilic depositions, and Congo red staining revealed apple-green birefringence under polarized light. After a series of examinations, the deposition of amyloid was found to be limited to the lungs. The final diagnosis was a rare, isolated pulmonary diffuse alveolar-septal form of amyloidosis.

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