장기간 추적 관찰한 특발성 폐 혈철 침착증 1례

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology; is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. The clinical and paraclinical findings consist of recurrent hemoptysis, pulmonary infiltrates, and iron deficiency anemia. Examinations of sputum disclose large numbers of hemosiderin-laden alveolar macrophage. Pulmonary interstitial fibrosis may develop. We experienced a case of recurrent IPH in an eight and half year-old girl who had been initially admitted at the age of 12 months old for the same complaints: cough, fever, and recurrent episodes of hemoptysis and iron deficiency anemia. Hemosiderin laden macrophages were demonstrated in smears of material obtained from gastric juices. There were no specific causes for the pulmonary hemorrhage. We reported a case of IPH with sequential findings on plain chest radiograph and high resolution computed tomography.