Images in Clinical Medicine

Childhood Idiopathic Pulmonary Hemosiderosis

Charles Keller, M.D., and Claire Langston, M.D.

N Engl J Med 2000; 343:781September 14, 2000

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Figure 1 A three-year-old girl with a declining growth rate and recurrent iron-deficiency anemia presented with a five-day history of nonproductive cough, fever, and anorexia. In association with an upper respiratory tract infection two weeks earlier, she had had hemoptysis involving 3-cm globs of bright red blood. Examination revealed a temperature of 39°C (102.2°F), a heart rate of 150 per minute, a respiratory rate of 60 per minute, and an oxygen saturation of 88 percent while she was breathing room air. Her breathing was labored, and breath sounds were decreased but coarse bilaterally. Chest radiographs revealed bilateral patchy alveolar infiltrates. The hemoglobin level was 8.4 g per deciliter, the reticulocyte count was 1.2 percent, and the mean corpuscular volume was 73 μm³. Examination of a section of the right middle lobe obtained during an open-lung biopsy (Panel A) revealed numerous golden-brown, hemosiderin-laden macrophages, small amounts of fibrin, and occasional extravasated erythrocytes (hematoxylin and eosin, ×100). Iron staining of bronchoalveolar-lavage fluid (Panel B) showed iron-positive (blue) macrophages and smaller, free erythrocytes (×100). These findings are pathognomonic of pulmonary hemosiderosis. The disease was considered idiopathic because no cause was found. Treatment with high-dose corticosteroids was begun to control the bleeding. After seven months of treatment, the hemoglobin level was 15 g per deciliter and the mean corpuscular volume was 86 μm³; however, the child had a recurrent cough as well as hemosiderin-laden macrophages in her sputum — a finding that prompted the initiation of adjuvant therapy with hydroxychloroquine.

Charles Keller, M.D.
Claire Langston, M.D.
Baylor College of Medicine, Houston, TX 77030

Citing Articles (3)

Citing Articles

1. 1

   Shela Akbar Ali Hirani, Arshalooz Rahman. (2011) Child With Idiopathic Pulmonary Hemosiderosis: A Case Report From Pakistan With Multiple Ethical and Moral Issues. Journal of Pediatric Nursing
   CrossRef

2. 2

   X. Zhang, L. Wang, A. Lu, M. Zhang. (2010) Clinical Study of 28 Cases of Paediatric Idiopathic Pulmonary Haemosiderosis. Journal of Tropical Pediatrics 56:6, 386-390
   CrossRef

3. 3

   Dmitriy Vaysman, Wilfredo G. Veloira, Allan L. Schuss, Haesoon Lee. (2004) Idiopathic Pulmonary Hemosiderosis. Pediatric Asthma, Allergy & Immunology 17:2, 151-155
   CrossRef