Pulmonary alveolar proteinosis (PAP) IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquire...Pulmonary alveolar proteinosis (PAP) IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquired PAP is divided into two forms based on clinical features: idiopathic PAP and secondary PAE Secondary PAP is reported to be associated with haematological malignancies, Pneumocystis carinii pneumonia and inhalation of silica or titanium, and the most frequent underlying disease of secondary PAP is haematological malignancy. The exact incidence of PAP in haematological malignancies is still obscure, since there have been only sporadic reports of secondary PAP.^ 2, 3展开更多
文摘Pulmonary alveolar proteinosis (PAP) IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquired PAP is divided into two forms based on clinical features: idiopathic PAP and secondary PAE Secondary PAP is reported to be associated with haematological malignancies, Pneumocystis carinii pneumonia and inhalation of silica or titanium, and the most frequent underlying disease of secondary PAP is haematological malignancy. The exact incidence of PAP in haematological malignancies is still obscure, since there have been only sporadic reports of secondary PAP.^ 2, 3