OBJECTIVETo improve the recognition of the clinical features and results of laboratory examination for isolated pulmonary Langerhans cell histiocytosis (PLCH) in children.

METHODThe information of one case with isolated PLCH was analyzed and reports of 11 cases with isolated PLCH were reviewed.

RESULTThe patient we report is only 2 years old with 1 month of course of disease, manifesting with prominent pulmonary involvement: cough and short of breath; CT scan of the chest showed punctiform, nodular and reticular high density opacities involving all lobes of both lungs. Biopsy of the lung tissue showed expression of CD1a, CD68, S-100, consistent with the diagnosis of LCH. He received prednisolone, VP16 and Vindesine with good response. Ten of 11 cases of isolated PLCH reported before manifesting with cough and dyspnea, CT scan of the chest showed interstitial lung changes (5/8), cystic changes (5/8), small nodules (2/8) and pneumothorax (2/8). Langerhans cells were found in 9 cases on lung biopsy, part of biopsy lung tissues were stained with anti-CD1a, the alveolar lavage fluid of the other 2 cases were stained with S-100 and anti-CD1a.

CONCLUSIONIsolated PLCH is rarely reported in children. It manifested with prominent pulmonary involvement: cough and short of breath, and CT scan of the chest showed interstitial lung changes, small nodules or cysts involving the lung, Langerhans cell could be found in lung biopsy, and the immunohistochemical staining in lung biopsy lung and alveolar lavage fluid stained with S-100 and anti-CD1a antibodies.