Isolated Pulmonary Langerhans Cell Histiocytosis in an Infant Diagnosed by CD1a Immunostaining of Bronchoalveolar Lavage Cells: A Case Report and Literature Review
10.15264/cpho.2017.24.2.157
- Author:
Mi Jin KIM
1
;
Jin Ho YOO
;
Young Wook CHO
;
Jae Won YOO
;
Sung Han KANG
;
Hyery KIM
;
Kyung Nam KOH
;
Ho Joon IM
;
Jong Jin SEO
Author Information
1. Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. jjseo@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Pulmonary;
Langerhans cell histiocytosis;
Bronchoalveolar lavage;
CD1a
- MeSH:
Biopsy;
Bronchoalveolar Lavage;
Diagnosis;
Drug Therapy;
Female;
Follow-Up Studies;
Histiocytosis, Langerhans-Cell;
Humans;
Infant;
Lung;
Rare Diseases;
Thorax
- From:Clinical Pediatric Hematology-Oncology
2017;24(2):157-161
- CountryRepublic of Korea
- Language:English
-
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.
