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Virtual Pediatric Hospital: Paediapaedia: Idiopathic Pulmonary Hemosiderosis Paediapaedia: Chest and Airway Diseases

Idiopathic Pulmonary Hemosiderosis

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Iron deficiency anemia and recurrent pulmonary symptoms including cough, hemoptysis, dyspnea, and wheezing. The radiological signs may precede the clinical symptoms.

Etiology/Pathophysiology:
Caused by diffuse alveolar hemorrhage with abnormal accumulation of hemosiderin in the lung. The etiology is unknown.

Pathology:
The diagnosis is made by seeing iron laden macrophages in the sputum, gastric washings, or bronchial washings.

Imaging Findings:
On film bilateral infiltrates that may be minimal or massive are seen.The infiltrates usually clear within a week. Atelectasis, emphysema, and hilar lymphadenopathy may also be present. The disease is progressive with repeated hemorrhagic episodes ultimately leading to the development of diffuse interstitial fibrosis in the lungs.

DDX:

References:
See References Chapter.

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