Kirchner J, Jacobi V, Kardos P, Kollath J
Institut für Allgemeine Röntgendiagnostik des Klinikums der Johann Wolfgang Goethe Universität, Theodor-Stern-Kai 7, D-60590, Frankfurt am Main, Germany.
Eur Radiol. 1998;8(3):352-4. doi: 10.1007/s003300050392.
Primary pulmonary amyloidosis is a rare disorder that appears in three forms: tracheobronchial, nodular parenchymal, and diffuse parenchymal. We report the case of a 46-year-old women with extensive tracheobronchial amyloidosis which presented with a 2-year history of dyspnea and with signs of severe fixed obstruction in pulmonary function tests. Computed tomography of the thorax demonstrated marked thickening of the trachea and the central bronchial tree with substantial narrowing of the main, lobar, and segmental bronchi. Transbronchial specimen showed typical birefringence under polarizing microscope after staining with Congo Red. We did not find hints for systemic amyloidosis.
原发性肺淀粉样变性是一种罕见疾病，有三种表现形式：气管支气管型、结节实质型和弥漫实质型。我们报告一例46岁女性，患有广泛的气管支气管淀粉样变性，有2年呼吸困难病史，肺功能检查显示严重固定性梗阻体征。胸部计算机断层扫描显示气管和中央支气管树明显增厚，主支气管、叶支气管和段支气管明显狭窄。经支气管活检标本经刚果红染色后在偏振显微镜下显示典型的双折射。我们未发现系统性淀粉样变性的迹象。
