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Idiopathic pulmonary hemosiderosis mimicking iron deficiency anemia: a delayed diagnosis?

Sultan Aydin Koker, Salih Gözmen, Yeşim Oymak, Tuba Hilkay Karapinar, Demet Can, Sinan Genç, Raziye Canan Vergin
  • Sultan Aydin Koker
    Division of Pediatric Hematology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey | drsultanaydin@hotmail.com
  • Salih Gözmen
    Division of Pediatric Hematology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
  • Yeşim Oymak
    Division of Pediatric Hematology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
  • Tuba Hilkay Karapinar
    Division of Pediatric Hematology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
  • Demet Can
    Division of Pediatric Allergy, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
  • Sinan Genç
    Division of Pediatric Radiology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
  • Raziye Canan Vergin
    Division of Pediatric Hematology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey

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 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 IPH. Chest radiographs should be taken even in absence of pulmonary symptoms. Early diagnosis is important for a timely management of IPH.

Keywords

Idiopathic pulmonary hemosiderosis, iron deficiency anemia, delayed diagnosis

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Submitted: 2017-01-13 05:43:49
Published: 2017-06-15 09:11:31
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Copyright (c) 2017 Sultan Aydin Koker, Salih Gözmen, Yeşim Oymak, Tuba Hilkay Karapinar, Demet Can, Sinan Genç, Raziye Canan Vergin

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