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

서지정보

· 발행기관 : 대한소아혈액종양학회
· 수록지 정보 : Clinical Pediatric Hematology-Oncology / 24권 / 2호 / 157 ~ 161페이지
· 저자명 : 김미진, 유진호, 조영욱, 유재원, 강성한, 김혜리, 고경남, 임호준, 서종진

초록

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.

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