Isolated Pulmonary Langerhans Cell Histiocytosis in an Infant Diagnosed by CD1a Immunostaining of Bronchoalveolar Lavage Cells: A Case Report and Literature Review / 임상소아혈액종양

ABSTRACT

Isolated pulmonary Langerhans cell histiocytosis (LCH) is a very rare disease in childhood. We report a case of a 5-month-old girl with isolated pulmonary LCH, who was transferred due to incidental chest x-ray finding of multiple cystic lesions without any clinical symptoms. Chest computed tomography (CT) finding suggested that pulmonary LCH was likely, but evaluations including lung biopsy were negative. At a follow-up visit three months later, we performed bronchoalveolar lavage (BAL) fluid analysis and confirmed the presence of CD1a-positive cells, thereby confirming diagnosis of pulmonary LCH. After completing eight months of chemotherapy, yearly follow-up evaluations were performed and there has been no evidence of reactivation of the disease for four years. Based on our case, we suggest that BAL with immunohistochemical staining can be a valuable modality to eliminate the possibility of infection and other infiltrating disorders, and diagnose pulmonary LCH in case of suspicious pulmonary lesions.