| Objective: Analyze the clinical features of pulmonary langerhans'cell histiocytosis for reviewing our experience and improving the strategy of diagnosis and treatment.Method: Retrospectively review of 2 cases of PLCH administrated in the first affiliated hospital of Guangxi Medical University from Jan 2009 to Jan 2011.Both 2 cases proved by pathology.Result:①2 patients are all male, age of onset is 23, 27 separately. Both patients have smoking history, but have no previous pulmonary disease. Both 2 patients are all single-system (lung) involved.②Clinical features: respiratory symptom including cough and dyspnea. Physical examinations of chest are normal, except one patient has signs of pneumothorax.③Imaging: PLCH presents of small nodules, cysts lesions varying of sizes, wall thickness and interstitial changes which includes ground-glass and fiber cord-like lesions. Hilar and meditational lymph nodes are not enlarged. After smoking cessation and steroids treatment, nodes and lesions partially absorb.④Pathology: 2 cases show langerhans cells infiltrated. 1 case shows S-100(+), CD-1a (+), some cells presents CD68.⑤Other laboratory examinations: Blood tests show normal except increase of white blood cells, neutrophilicgranulocyte, ESR and CRP. Lung functions all show mixed pattern ( mainly present obstructive-restrictive) and diffusion dysfunction⑥Treatment: After smoking cessation and steroids therapy, 2 patients all improved in symptom,CT scans and lung function.Conclusion:①PLCH is more commonly fund in young smokers, which often present with cough, laboring dyspnea and pneumothorax.②The abnormalities present nodules, cystic degeneration and interstitial lesions on chest resolution CT scan. Pulmonary function present mixed pattern(obstructive pattern mainly). Pathological features are infiltration of langerhans cells.③Smoking cessation and steroids therapy are effective.
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