Disease recurrence after lung transplantation for idiopathic pulmonary hemosiderosis

Abstract

Idiopathic pulmonary hemosiderosis is characterized by the triad of hemoptysis, iron deficiency anemia and pulmonary infiltrates. Though idiopathic pulmonary hemosiderosis has classically been described as a childhood disease, survival into adulthood is possible. Treatment options for advanced and/or refractory disease is limited, and in our unique case of idiopathic pulmonary hemosiderosis with precapillary pulmonary hypertension, lung transplantation has had a favorable short-term outcome. We also demonstrate that disease recurrence of idiopathic pulmonary hemosiderosis following lung transplantation is possible.

Keywords: Disease recurrence; Hemoptysis; Idiopathic pulmonary hemosiderosis; Lung transplant; Pulmonary hypertension.

Fig. 1

Pre-transplant computed tomography images showing a dilated pulmonary artery suggestive of pulmonary hypertension (left) and extensive, basal-predominant, centrilobular ground glass opacities with smooth septal thickening (right).

Fig. 2

Gross (left) and microscopic (right) pathology from explanted native lungs revealing diffuse homogeneous alveolar septal fibrosis with clusters of alveolar hemosiderin-laden macrophages and interstitial hemosiderin deposition.