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.
폐포단백증은 비전형적 림프절 종대를 포함한 다양한 방사선 소견을 보일 수 있으며 특히 다른 폐질환과 병발하여 발생하는 경우에는 진단에 각별한 주의가 필요하다. 또한 기저 질환에 속발하여 발생하는 이차성 폐포단백증의 경우에는 기저 질환의 치료가 무엇보다도 중요함을 인식하여야 할 것이다.
