Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review.
BACKGROUND
Pulmonary Langerhans cell histiocytosis (PLCH) is an orphan disease in respiratory medicine, which most affects adult smokers. The purpose of this article was to discuss the clinical features, especially the radiologic features of PLCH patients during their hospitalization through a retrospective analysis on clinical data. Furthermore, the current literature was also reviewed.


METHODS
Between December 2008 and June 2012, 14 patients with PLCH were assessed at Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. Among these patients, seven patients were diagnosed through tissue biopsy from the lung and one patient from enlarged cervical lymph nodes; the rest of six patients were diagnosed based on the clinical-radiological data. The data consisting of demographics, clinical presentation, smoking habits, pulmonary function tests (PFTs) and radiographic image from the medical records was analyzed retrospectively.


RESULTS
The average age of patients (11 males and 3 females) was 42.79 (±13.71) years old. All male patients and one female patient had a long smoking history. The common manifestations were cough and exertional dyspnea. Spontaneous pneumothorax was found in three patients. Varieties of pulmonary shadows such as nodular, cystic, patch-like and cord-like were revealed by chest computed tomography (CT) examination. Large Langerhans cells (LCs) were discovered in biopsy tissue by immunohistochemical stains.


CONCLUSIONS
PLCH is still an orphan disease and maybe related to smoking. Clinical symptoms such as cough and exertional dyspnea are non-specific. We shall pay attention to recurrent pneumothorax as clinically it is associated with PLCH. The characteristic radiological manifestation is cystic or nodular shadow in the lungs, which plays crucial roles in diagnosing PLCH.
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Cheng-Wei
CW
Li
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Man-Hui
MH
Li
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Jiang-Xiong
JX
Li
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Ru-Jia
RJ
Tao
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Jin-Fu
JF
Xu
1 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Soochow University, Suzhou 215006, China ; 2 Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China.
Wei-Jun
WJ
Cao
Pulmonary Langerhans cell histiocytosis (PLCH); radiological manifestation; recurrent pneumothorax