A Case of Atypical Manifestation of Pulmonary Alveolar Proteinosis Associated with Myelodysplastic Syndrome.
- Author:
Jae Young KIM
1
;
Seok Chan KIM
;
Ji Young KANG
;
Hea Yon LEE
;
Yoo Jin KIM
;
Yae Ni KIM
;
Ji Won HAN
Author Information
- Publication Type:Case Report
- Keywords: Pulmonary alveolar proteinosis; Myelodysplastic syndromes; Extracorporeal membrane oxygenation; Tuberculosis
- MeSH: Biopsy; Bronchiolitis; Cough; Dyspnea; Extracorporeal Membrane Oxygenation; Hematologic Neoplasms; Humans; Leukemia, Myelogenous, Chronic, BCR-ABL Positive; Lung; Male; Middle Aged; Military Personnel; Myelodysplastic Syndromes*; Pulmonary Alveolar Proteinosis*; Rare Diseases; Thrombocytopenia; Tuberculosis
- From:Korean Journal of Medicine 2014;86(1):74-78
- CountryRepublic of Korea
- Language:Korean
- 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.
