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Abstract

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Article in Japanese

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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