A Case of Atypical Manifestation of Pulmonary Alveolar Proteinosis Associated with Myelodysplastic Syndrome / 대한내과학회지

ABSTRACT

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of lipoproteinaceous material in the distal air spaces. Secondary forms of PAP are caused by underlying diseases, in particular tumorous conditions, the most common of which are hematologic malignancies, such as chronic myeloid leukemia and myelodysplastic syndrome (MDS). We herein describe a case of atypical manifestation of PAP caused by MDS, which initially mimicked military tuberculosis with severe thrombocytopenia because of radiologic features showing necrotic mediastinal lymphadenopathies and diffuse bronchiolitis. A 46-year-old male visited the hospital complaining of coughing and general weakness and was presumptively diagnosed with military tuberculosis combined with MDS. He was treated with antituberculous medication, but the lung lesions did not improve and dyspnea only progressed. Finally, he underwent lung biopsy and was diagnosed with atypical pattern of PAP associated with MDS.