Clin Pediatr Hematol Oncol.  2017 Oct;24(2):157-161. 10.15264/cpho.2017.24.2.157.

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

• Kim MJ
• Yoo JH
• Cho YW
• Yoo JW
• Kang SH
• Kim H
• Koh KN
• Im HJ
• Seo JJ

• KMID: 2396020
• DOI: http://doi.org/10.15264/cpho.2017.24.2.157

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.

Keyword

Pulmonary; Langerhans cell histiocytosis; Bronchoalveolar lavage; CD1a

MeSH Terms

Biopsy
Bronchoalveolar Lavage*
Diagnosis
Drug Therapy
Female
Follow-Up Studies
Histiocytosis, Langerhans-Cell*
Humans
Infant*
Lung
Rare Diseases
Thorax