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http://dx.doi.org/10.3904/kjm.2014.86.1.74

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

Kim, Jae Young (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Kim, Seok Chan (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Kang, Ji Young (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Lee, Hea Yon (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Kim, Yoo Jin (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Kim, Yae Ni (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Han, Ji Won (Department of Internal Medicine, The Catholic University of Korea College of Medicine)
Publication Information
The Korean Journal of Medicine / v.86, no.1, 2014 , pp. 74-78 More about this Journal
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.
Keywords
Pulmonary alveolar proteinosis; Myelodysplastic Syndromes; Extracorporeal Membrane Oxygenation; Tuberculosis;
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