The diagnosis of Langerhans cell histiocytosis (LCH) a rare disease, characterized by a wide clinical spectrum has improved owing to the recent introduction of immunological and histochemical markers. Our study aimed at presenting the clinical and laboratory data of 10 Greek patients, and emphasize the role of Bronchoalveolar Lavage (BAL) as a simple and useful diagnostic tool.
Clinical and laboratory data for 10 adult patients with LCH was retrospectively analyzed.
Five males and 5 females, aged 18 to 79 years ( mean age 34 years) and 8 being smokers presented most frequently with cough (7 patients), exertional dyspnea (7), thoracic pain (2), fatigue (3), weight loss (2) and fever (3) while one patient was asymptomatic and in 2 patients spontaneous pneumothorax was the first presentation of the disease (diabetes insipidus was subsequently noted in one). Mean symptom duration was 11 months. Other organ involvement in 4 cases included skin and bone lesions, lymph node enlargement and adenoma of pituitary. The typical radiological and HRCT diffuse nodular pattern with small cysts was common to all patients two of which had advanced disease with extensive lung damage. Spirometry and DLCO were normal in 4 patients, whereas 4 patients with an obstructive disorder and 2 with a restrictive pattern had a decreased DLCO. Diagnosis was made by open lung biopsy in one case, VATS in 2 and transbronchial biopsy (TBB) in 2 cases. BAL was diagnostic in all patients, by detection of CD1a and S-100 protein positive hystiocytes. Follow up data was available in 5 patients.
LCH is a rare disease not necessarily exclusive to young smokers. Lung involvement is usual and HRCT findings as well as BAL with detection of CD1a cells and S-100 protein are characteristic in all cases.
LCH is also seen in old age with the characteristic radiological findings and typical immunological markers being present. BAL is a simple and safe diagnostic procedure with a 100% sensitivity and specificity.
V. Polychronopoulos, None.