摘要
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 describe the clinicopathologic features and diagnostic algorithm of pulmonary alveolar proteinosis （PAP）. Methods Nine biopsy cases of PAP were studied by hematoxylin-eosin（HE）stains and light microscopy, and histochemical staining using periodic acid-Schiff with digestion （D-PAS） and muciearmine. Results HE stained sections showed that the affected lung tissue displayed the following characteristic features： （ 1 ） alveoli and some of the small bronchioles were filled with eosinophilic and fine granular proteinaceons material; （2） proteinaceous material was admixed with various numbers of degenerated and sometimes exfoliated pneumoeytes; （3） pneumoeytes were hyperplastie; （4） alveolar capillaries and alveolar septa had become hyperemie; （5） compensating emphysema was noted in the surrounding lung parenchyma. Histochemical staining showed that the proteinaceous material was stained purple by D-PAS, and mucicarmine was negative. Conclusion PAP is a rare and it is easily misdiagnosed or neglected. Identification of homogeneous, eosinophilic, finely granular and D-PAS -positive proteinaceous material in alveolar spaces is of diagnostic importance in PAP. Fiber bronchoscopy and videoassised thoracoscopic lung biopsy is the main way to obtain the specimens.
出处
《诊断病理学杂志》
CSCD
2009年第6期437-439,共3页
Chinese Journal of Diagnostic Pathology
关键词
肺疾病
肺泡蛋白沉积症
临床病理特征
Lung diseases
Pulmonary alveolar proteinosis
Clinicopathologic features
