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目的探讨肺泡蛋白沉积症(PAP)的临床病理特征及其诊断方法。方法对9例PAP进行常规HE染色,光镜观察,并用淀粉酶消化后过碘酸希夫(D-PAS)及黏液卡红进行组织化学染色。结果PAP显示肺泡腔内及部分小支气管腔内充满嗜伊红性细颗粒状蛋白性物质;蛋白性物质中杂有多少不等退变及脱落的肺泡上皮细胞;肺泡Ⅱ型上皮细胞增生;肺泡间隔毛细血管充血;周围肺组织代偿性肺气肿。此外,肺泡腔内嗜伊红性细颗粒状磷脂类蛋白性物质D-PAS(+)(呈紫红色),黏液卡红(-)。结论PAP临床罕见,易误诊或被忽视。典型PAP为肺泡腔内出现嗜伊红性细颗粒状蛋白性物质,蛋白性物质D-PAS(+),黏液卡红(-),是确诊PAP的主要方法。纤支镜及胸腔镜肺活检是获取标本的主要途径。
Objective To investigate the clinicopathological features and diagnostic methods of pulmonary alveolar proteinosis (PAP). Methods Nine cases of PAP were stained with HE and observed with light microscope. The tissue sections were stained with D-PAS and mucardin by amylase digestion. Results PAP showed alveolar cavity and part of the bronchial lumen full of eosinophilic fine granular protein material; protein material in the number of heterogeneous degeneration and loss of alveolar epithelial cells; alveolar type Ⅱ epithelial cell proliferation; alveolar Interval capillary congestion; compensatory emphysema around the lung tissue. In addition, alveolar intracellular eosinophil fine granular phospholipid protein D-PAS (+) (was purple), mucus card red (-). Conclusion PAP clinical rare, easily misdiagnosed or overlooked. Typical PAP appears in the alveolar cavity of eosinophilic fine granular protein material, protein D-PAS (+), mucus card red (-), is the main method of diagnosis of PAP. Fiberoptic bronchoscopy and thoracoscopic lung biopsy is the main way to obtain specimens.