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Joseph Junewick, MD FACR
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Pulmonary Hemosiderosis

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 3 years ago
Updated: over 3 years ago
Tags: PEDS
Modality/Study Types: CR CT
Activities:
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History

9 year old male with iron-deficiency anemia and hemoptysis.


Case Images


Diagnosis

Pulmonary Hemosidersois

Clinical Notes

Bronchoalveolar lavage with hemosiderin laden macrophages.

Findings

CT – Extensive peripheral and lower lower lobe ground glass opacification with tiny subpleural blebs.

CR – Chest radiograph obtained 2 months after CT shows improved mixed alveolar and interstitial disease.

Discussion

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon disease of unknown origin characterized by recurrent or intermittent hemoptysis, pulmonary infiltrates due to alveolar hemorrhage, iron deficiency anemia, and the presence of hemosiderin-laden alveolar macrophages in the bronchoalveolar lavage fluid. The typical radiographic findings of IPH are areas of airspace consolidation or ground-glass opacities, frequently with a perihilar and lower lung zone predominance. CT findings include nodules and patchy areas of ground-glass attenuation in the subacute phase and diffuse homogeneous areas of ground-glass attenuation during an exacerbation. MR finding include relatively decreased lung parenchymal signal intensity on T2-weighted images due to the paramagnetic effect of ferric iron. The differential diagnosis of pulmonary hemosiderosis in childhood includes Goodpasture syndrome, Heiner syndrome and pulmonary hemosiderosis in association with cardiac or pancreatic involvement. Lung biopsy is confirmatory. IPH is occasionally associated with celiac disease, known as Lane-Hamilton syndrome. IPH occurs most commonly between ages 1 and 7 years and occurs with equal frequency in boys and girls. Although the disease is usually lethal, aggressive therapy can permit survival longer than 10 years.

Reference

Rubin et al. Diagnosis of pulmonary hemosiderosis by MR imaging. AJR 1989; 152:573-574.

Nishino et al. Case 155: Lane-Hamilton syndrome. Radiology 2010; 254: 955-958.

Contributor

Chirag Patel, MD



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