Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax

Abstract

We describe the outcome of a 20-month-old female and a 6-year-old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X-ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6-mercaptopurine). They had recurrent episodes of pneumothorax during follow-up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow-up, respectively. Pulmonary function tests done during follow-up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases.

Isolated Pulmonary Langerhans Cell Histiocytosis in a 3-Year-old Girl With Atypical Radiologic Findings

“…Advanced pulmonary LCH patients may exhibit CT evidence of fibrosis and honeycombing. [4][5][6][7][8][9] In our case, the CT findings differed to the adult pattern. Thin-walled cysts were found throughout the lung parenchyma not sparing the lung bases.…”

“…Pleural effusions occur infrequently but may be observed in association with pneumothorax or after diagnostic thoracotomy. [4][5][6][7][8][9] CT, especially high-resolution CT (HRCT), is superior to radiography in demonstrating the morphology and distribution of lung abnormalities in patients with pulmonary LCH.…”

Primary pulmonary Langerhans cell histiocytosis associated with smoking in an adolescent boy

“…12,13 Few cases of confirmed isolated PLCH have been reported in literature in the pediatric age group. 2,5,8,[14][15][16] In only two of them with eventually stabilized disease. Our patient was symptomatic at the beginning.…”

Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

“…The main complication of pulmonary LCH is the occurrence of SSP with subsequent recurrence and persistence despite conservative management 11. Patients usually present to the emergency department with shortness of breath associated with pleuritic chest pain of sudden onset.…”