The First Definition of Pulmonary Component of Hypereosinophilic Syndrome: Bronchial Casts

Abstract

Hypereosinophilic syndrome is a heterogeneous disease characterized by eosinophilic tissue inflammation and eosinophilia. Pulmonary involvement could be seen in up to 55% among children with hypereosinophilic syndrome. A 3-year-old boy with chronic hypereosinophilia and respiratory complaints was diagnosed with idiopathic hypereosinophilic syndrome. Atelectasis was detected in the radiological evaluation, and bronchial casts with eosinophilic structures were removed by bronchoscopy. Steroid, inhaled hypertonic saline, inhaled bronchodilator, inhaled corticosteroid, and leukotriene receptor antagonist were used for 1 year in the management of hypereosinophilic syndrome, and related eosinophilic casts and repetitive bronchoscopies were administered for removal of the casts. The patient was successfully managed with an inhaled N-acetyl cysteine treatment. In children, the long-term prognosis of hypereosinophilic syndrome is uncertain. Comprehensive diagnostic tests are required for the early diagnosis and management of pediatric hypereosinophilic syndrome. In the presented case, the rare occurrence of pulmonary involvement of hypereosinophilic syndrome in a 3 year-old-boy with recurrent hypereosinophilic casts and its management were discussed.

Figure 1.

Left lower lobe atelectasis in the chest x-ray on admission (A). Right upper lobe and left lower lobe atelectasis on chest x-ray before the first bronchoscopy (B). Control chest x-ray a day after bronchoscopy and removal of the eosinophilic casts and a significant improvement of the atelectasis was observed (C).

Figure 2.

The appearance of the dense, mucoid structure which was in dirty yellow color at the entrance of the right lung upper lobe apical segment during fiberoptic bronchoscopy (A). The macroscopic view of eosinophilic casts removed from the right upper lobe apical segment (B). The appearance of dirty yellow tough crusty structure in the left lower lobe bronchus (C) during fiberoptic bronchoscopy and the macroscopic view after removal (D). Microscopic view of eosinophilic casts at 100× magnification with hematoxylin–eosin staining (E).

Figure 3.

Figure showing the relationship between the patient’s treatment scheme, peripheral blood eosinophil counts, and bronchoscopic interventions.