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Article in Japanese
Abstract
Case Report
Autoimmune pulmonary alveolar proteinosis with myelofibrosis: a case report
Takako Kuroki Hiroki Ninomiya Yoshiya Tsunoda Kyoko Kondo Takeshi Kawakami Mao Sasamoto
Department of Respiratory Medicine, Kobari General Hospital
ABSTRACT
A 42-year-old woman with myelofibrosis (MF) was admitted to our hospital complaining of dyspnea. Chest radiography showed diffuse consolidation, and chest computed tomography revealed a typical crazy-paving pattern. The bronchoalveolar lavage fluid was yellowish-white in appearance and sera were positive for anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody. She was therefore diagnosed with autoimmune pulmonary alveolar proteinosis (aPAP). We treated her with whole lung lavage (WLL) twice, but she died after the second treatment. Her diagnosis was primary (autoimmune) pulmonary alveolar proteinosis with positive anti GM-CSF antibody. However, the coexistence of MF, HRCT features, pathological findings, and prognosis show characteristics of secondary pulmonary alveolar proteinosis.
KEYWORDS
Pulmonary alveolar proteinosis (PAP) Myelofibrosis (MF) Anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody Whole lung lavage (WLL)
Received 5 Mar 2020 ／ Accepted 23 Oct 2020
AJRS, 10(2): 183-186, 2021
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