Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disorder in wich lesions contain pathologic Langerhans cells (PLC). A quarter of patients with LCH has pulmonary lesions. Patients with pulmonary involvement are either asymptomatic or manifest adry unproductive cough with dyspnea and chest pain. The basic lesions of LCH is formed by collections of PLC. The presence of Birbeck granules by electron microscopy or the expression of the CD1a antigen on the cell surface are necessary for a definitive diagnosis of LCH. High resolution CT (HRCT) can show reticular, reticulonodular, nodular opacities and diffuse cysts and cavity. When the characteristic nodular, cavitary and cystic lesions are together they are highly suggestive of LCH, especially when they predominate in the upper to mile lung fields.

LCH must be considered in the diferansial diagnosis of interstitial lung disease associated with cavitary and cystic lesions.

Akciðer tutulumu semptomlarý ile baþvuran bir Langerhens hücreli histiyositoz olgusu