PULMONARY ALVEOLAR PROTEINOSIS - A REPORT OF 2 CASES WITH DIAGNOSTIC FEATURES IN BRONCHOALVEOLAR LAVAGE SPECIMENS

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare condition t hat has been associated with myriad diseases and disorders. Alveolar s paces are progressively filled with a phospholipoproteinaceous materia l, presumably related to a derangement of surfactant production and/or catabolism. The cytologic features of PAP in bronchoalveolar lavage ( BAL) sediments are unique, and recognition of these characteristics ca n help guide clinical intervention. CASES: A 47-year-old male with a h istory of progressive dyspnea and recent pneumonia presented with a fi ve-lobe alveolar infiltrate and subsequently underwent bronchoscopic e xamination. A 31-year-old female with chronic myelogenous leukemia in blast transformation developed unresponsive pulmonary infiltrates nece ssitating bronchoscopy with lavage. Both BAL lavage fluid sediments co ntained a homogeneous, basophilic, granular material typical of PAP. T he material was composed of extracellular, multilamellated bodies when viewed by electron microscopy. Both patients required repented therap eutic whole lung lavage, and one died of the disease eight months afte r the diagnosis. CONCLUSION: Clinical presentation, grossly milky BAL fluid and fluid sediment with light microscopic findings Of basophilic , periodic acid-Schiff-positive, granular debris with cholesterol crys tals and a few alveolar macrophages suggest this process. The light mi croscopic findings can be confirmed by ultramicroscopic demonstration of extracellular multilamellated bodies. BAL with appropriate examinat ion of the effluent sediment facilitates the diagnosis of PAP.