Background: Idiopathic pulmonary hemosiderosis(IPH) is a rare disease with a variable outcome. Itis characterized by recurrent episodes of diffusealveolar hemorrhage and iron deficiency anemia.
Case Report: A 6-year-old boy, was hospitalizedfor severe anemia. At the time of the diagnosis hepresented with progressive fatigue and pallor. Thephysical examination revealed mucocutaneous pallor, a grade III/VI systolic murmur and inspiratorycrackles. Laboratory investigation revealedmicrocytic hypochromic anemia (hemoglobin5.7 g/dL, mean corpuscular volume of 58.9 fL, reticulocyte index 2.18) with iron deficiency, normalrenal and liver function, and normal coagulationprofile. Admitted under iron suplementationwith progressive rise in hemoglobin. Infectiouscauses were excluded. Bone marrow biopsy wasnormal. After 1 month he developed fever, coughand dispnoea, without hemoptysis. The chestradiograph showed diffuse alveolar-type infiltrates. Bronchoscopy with bronchoalveolar lavage reveleadover 75% haemosiderin -stained macrophages. Lung biopsy specimen was negative for vasculitis, immune complex deposition or granulomatousdisease. The immunological study was negative, including anti-GBM, ANA, c-ANCA, IgE cow's milkantibodies and reumatoid factor. Echocardiographyrevealed no evidence of pulmonary hypertension. The diagnosis of IPH was considered and thepatient was started on prednisolone (2 mg/kg/day)with improvement in respiratory symptoms after 1week. Despite corticosteroids he kept having IPHexacerbations and chronic anemia and initiatedhydroxichloroquine with good clinical response.
Comments: In this case the association of irondeficiency anemia refractory to iron supplementationwith the sudden onset of respiratory symptoms andthe suggestive radiological presentation establishedthe diagnosis of IPH.
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Corujeira, S., Santos-Silva, R., Ferreira, F. et al. 1294 Iron Deficiency Anemia in a Child with Idiopathic Pulmonary Hemosiderosis. Pediatr Res 68, 640–641 (2010). https://doi.org/10.1203/00006450-201011001-01294