ABSTRACT
Objective To study the clinical features of pulmonary Langerhans cell histiocytosis.Methods Restrospectively review of 7 cases of pulmonary Langerhans cell histiocytosis proved by pathology with the clinical,physical,radiological,pathological and pulmonary function presentation.Results Seven cases were all male,aged from 17 to 37.Four of them are cigarette smokers.The major symptoms were cough and laboring dyspnea,and pneumothorax presented in five cases.As to pulmonary function presentation,obstructive pattern presented in three cases,restrictive pattern and mixed pattern presented in two cases respectively,and the carbon monoxide diffusing capacity was decreased in four cases.The abnormalities present predominantly in the upper and middle lung fields on chest high-resolution CT scan.Lung cysts and reticular changes presented in six cases and nodules presented only in one case.The diagnosis was established by surgical lung biopsy.The positive cases of staining for the S-100,CD1a antigens,and CD68 were 7/7,4/5 and 5/6 respectively.Conclusion PLCH patients are typically young adults,often present with cough,laboring dyspnea and pneumothorax.Pulmonary function presentation is nonspecific and the carbon monoxide diffusing capacity may be decreased.The characteristic features of HRCT are bilateral reticulonodular areas and cysts change that predominantly involved the upper and middle lung zones with relative sparing of the lung bases.The pathologic feathers are interstitial infiltration of bronchiole walls and epithelium by LCH cells and cyst formation.The staining for CD1a antigens and the S-100 proteins is positive by immunohistochemical analysis.