Case Report: An Unusual Case of Idiopathic Pulmonary Hemosiderosis in a School Aged Nepali Girl

<ns3:p>Abstract Idiopathic pulmonary hemosiderosis (IPH) is a rare, but potentially serious, disease in children. It is thought to be an immune-mediated disorder, but its exact pathogenesis remains unknown. The disease usually shows three characteristic features: the classical triad of hemoptysis, iron deficiency anemia, and diffuse parenchymal consolidations on chest X-ray. However, children often do not have all three signs at the beginning of hospitalization, which can delay diagnosis and worsen outcomes. We report the case of a 6-year-old Nepali girl who had repeated episodes of hemoptysis, fatigue, shortness of breath on exertion, and progressive palllor for four months. In the past, she had multiple similar episodes of shortness of breath and severe anemia that were treated unsuccessfully with iron supplementation and repeated blood transfusions. Chest computed tomography showed scattered ground-glass opacities in both lungs, suggestive of pulmonary hemorrhage. IPH was confirmed by the presence of hemosiderin-laden macrophages in the bronchoalveolar lavage. The patient was treated with a combination of azathioprine and corticosteroids, and her condition improved. This case emphasizes that clinicians should suspect IPH in children with severe iron-deficiency anemia that does not improve on iron supplementation and repeated blood transfusions. The absence of hemoptysis does not exclude the diagnosis of IPH in children. This is because they may initially present with incomplete clinical features but may later develop the typical triad of IPH. Early and accurate diagnosis as well as appropriate therapy can be lifesaving.</ns3:p>