PULMONARY LANGERHANS CELL HISTIOCYTOSIS - CASE REPORT

Abstract

Introduction: Langerhans cell histiocytosis (LCH) is a rare disorder of unknown origin. The disease can regress spontaneously or with glucocorticoid therapy, or is characterized by severe manifestations, recurrent pneumothorax, progressive respiratory failure, chronic corpulmonary and death. Over a 10-year period, only 4 cases of LCH have been identified at Pham Ngoc Thach (PNT) Hospital, Ho Chi Minh City, Vietnam. Case Presentation: We report a case of pulmonary Langerhans' cell histiocytosis diagnosed and treated at PNT Hospital. A 16-year-old young man presented at palliative care unit (department A4) with chief symptoms of dry cough and shortness of breath during the last 2 months. He reported a history of coughing and exertional dyspneoa during the last year. He is a pupil and has smoked half a packet of cigarettes daily for the last two and a half years. On physical examination, right pneumothorax was detected whereas the other organs were normal. No abnormalities were detected in blood tests. White blood cell count was 8,460/mm3 and haemoglobin level 14 g/dl. The other routine blood tests were all in normal ranges. Sputum smears for AFB and HIV testing were negative. Chest radiography showed right pneumothorax and reticulonodular shadows distributed in both lungs. Chest CT scan showed right pneumothorax together with multiple thin wall cysts with diameters of 1-1.5 cm and lesions of centrilobular nodules, predominately involving upper lobes in both lungs. Lung function test showed moderate restrictive pattern. Bronchoscopy was normal and transbronchial biopsy of the middle lobe was done but nothing specific was detected with the sample. Video-assisted thoracoscopic biopsy of the lung was performed and histological result showed hyperplasia of lung tissue including histiocytosis, langerhans cells with elongated shapes, bright and kidney-like nuclei forming clusters, and multinucleated giant cells. By immunohistochemical staining, monoclonal antibodies directed against LCA, CD68, CD1a and S.100 were observed. Clinical manifestations, chest CT scan, and pathological and immunohistochemical findings confirmed the diagnosis of Langerhans's cell histiocytosis. Drainage and pleurodesis were performed to manage pneumothorax. Smoking cessation was required. Prednisone at a starting dose of 30 mg was given daily and after 1 week of treatment, the patient got well and was transferred to palliative care clinics for follow-up. Conclusion: Pulmonary Langerhans' cell histiocytosis should be considered in male patients with history of heavy smoking, symptoms of dry cough and dyspneoa, and with specific images on chest radiography and CT scan. The disease is confirmed by biopsy of the lung with pathological characteristics.