Pulmonary alveolar proteinosis(PAP) is a rare disease in which the alveolar spaces are filled with an eosinophilic, PAS-positive material, whereas the interstitial architecture of the lung usually remains unaffected. Although a definitive diagnosis is usually made by an open lung biopsy, bronchoalveolar lavage(BAL) cytology may play a decisive role in the diagnosis and therapy of these patients and may spare a patient a more invasive diagnostic procedure. The author presents a patient in whom BAL cytology specimen contained the characteristic globules of amorphous proteinaceous PAS-positive material accompanied by background of rare macrophages and inflammatory cells.
Ultrastructural study using BAL specimen can confirm the diagnosis of PAP.
The authors experienced a case of pulmonary alveolar proteinosis. A 58-year-old woman presented with 6 months duration of cough sputum and multiple patch mottled densities in both lung fields. Major histologic finding was filling of the alveoli by Periodic-Acid-Schiff-positive proteinaceous material with maintenance of normal alveolar architecture. Osseous metaplasia was seen in the alveolar space, focally. Ultrastructural study revealed numerous lamellar bodies in alveolar spaces. The immunohistochemical study using antibody to surfactant apoprotein revealed positive reaction in proteinaceous material.
A case of pulmonary alveolar proteinosis is reported. Most of the alveolar spaces were filled with amorphous deep eosinohilic material which revealed strong positive reaction to periodic acid-Schiff staining. Electron microscopic observation of this material showed numerous lamellar bodies in the alveolar spaces and cytoplasms of alveolar macrophages. A part of them were concentric multilamellated type A lamellar bodies and the other were finger printlike type B bodies. Combined type A and type B lamellar bodies were rarely present. From the above features it is suggested that both type A and B lamellar bodies could be transformed one another and those lamellar bodies may be originated from pulmonary surfactant.