Abstract
Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia. The etiology of IPH is not known and its diagnosis may be difficult due to the variable clinical courses. The most helpful signs for identifying IPH are iron deficiency anemia and recurrent or chronic cough, hemoptysis, dyspnea, wheezing. We report here 5 pediatric cases of IPH presenting with iron deficiency anemia and without pulmonary symptoms. Mean corpuscular volume was low in all patients; iron was low in 4 out of 5 cases; total iron binding capacity was high in all of them; ferritin was low in 3 patients. At follow up, none of them had responded successfully to the iron therapy. Although they didn’t present with pulmonary symptoms, chest radiographs incidentally revealed diffuse reticulonoduler shadows in all of them. Computed tomography revealed diffuse ground-glass opacities, consolidation, increased density. The diagnosis was confirmed by the detection of hemosiderin-laden macrophages in bronchoalveolar lavage fluid and gastric aspirate. If patients with iron deficiency anemia don’t respond to iron therapy, they should be examined for IPH. Chest radiographs should be taken even in absence of pulmonary symptoms. Early diagnosis is important for a timely management of IPH.
Highlights
N diffuse ground-glass opacities, consolidation, increased density
The diagnosis was confirmed by the detection of hemosiderinladen macrophages in bronchoalveolar lavage fluid and gastric aspirate
If patients with iron deficiency anemia don’t respond to iron therapy, they should be examined for Idiopathic pulmonary hemosiderosis ly (IPH)
Summary
We have reported to clinical findings and laboratory of our five cases below (Table 1). Despite the patients had pallor, iron deficiency anemia at diagnosis, they had not pulmonary symptoms such as hemoptysis, dyspnea, wheezing. The common feature of all our patients is having not pulmonary symptoms and following for a long time due to iron deficiency anemia before determining to pulmonary hemosiderosis. Mean Corpuscular Volume (MCV) was low at all of them. Total Iron Binding Capacity was high at all of them. ©Copyright S.A. Koker et al, 2017 Licensee PAGEPress, Italy Hematology Reports 2017; 9:7048 doi:10.4081/hr.2017.7048 siderosis was confirmed, patients were administered oral prednisolone at a dose of 2 mg/kg/day for 2 weeks if they presented during acute phase. Minimum dose of steroid was maintained during having their normal hemoglobin value, follow up
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