Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome

Abstract

characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired PAP is divided into two forms based on clinical features: idiopathic PAP and secondary PAP. 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 In this paper, we report the first case of secondary PAP in China associated with myelodysplastic syndrome (MDS) with refractory anaemia and excess blasts (RAEB), which was diagnosed by histological findings of open lung biopsy. We retrospectively assessed his clinical feature and analyzed the published articles from PubMed in order to obtain further insight into secondary PAP that is associated with fatal complications in MDS patients.