Non-smokıng patient with pulmonary langerhans cell histiocytosis x diagnosed with the help of simultaneous bilateral spontaneous pneumothorax

Abstract

Introduction: Pulmonary Langerhans cell histiocytosis X (PLCHx) is a rare interstitial lung disease of unknown aetiology and occurs mostly in young smokers. Lung involvement alone accounts for >85% of the cases; other organs may be involved too (5%–15%). Case Report: A 19-year-old boy was presented to the emergency service with sudden-onset, sharp, stabbing chest pain and dyspnoea. The patient had no history of smoking. His medical and family histories were negative for any disease. Thoracic radiography showed increased reticulonodular density with a near-total bilateral pneumothorax at the left and partially at the right. Hence, bilateral tube thoracostomy was performed. High-resolution computed tomography (HRCT) showed pronounced thin-walled cystic structures smaller than 10 mm and small millimetre-sized nodules in the upper and middle zones. Extrapulmonary Langerhans cell histiocytosis was excluded based on the radiological and laboratory findings. Based on the clinical and tomography findings, diagnosis of PLCHx was made. Conclusion: Our case highlights that PLCHx should be suspected in non-smokers. Further studies elucidating the aetiology of PLCHx are needed.

Keywords

• Histiocytosis X,
• spontaneous pneumothorax
• interstitial lung disease,

References

1. 1. Kaya A, Savaş İ, Şen E, Sak SD, Güngör A, Gönüllü U. Pulmonary Histiocytosis X and Lung Carcinoma: Case Report. Tuberk Toraks 2001; 49(4): 500-503.
2. 2. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary langerhan’s cell histiocytosis. N Eng J Med 2000; 342: 1969-78.
3. 3. Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J 2006; 27: 1272-85.
4. 4. Vassallo R, Ryu JH. Pulmonary Langerhans' cell histiocytosis. Clin Chest Med 2004; 25:561.
5. 5. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonaryLangerhans’-cell histiocytosis in adults. N Engl J Med 2002; 346: 484–490.
6. 6. Minghini A, Trogdon SD. Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. Am Surg 1998;64:1040–1042.
7. 7. Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH. Pneumothorax in pulmonary langerhans cell histiocytosis.Chest 2004; 125: 1028–1032.
8. 8. Moore AD, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, et al. Pulmonary histiocytosis X: comparison of radiographic and CTfindings. Radiology 1989; 172: 249–254.

9. 9. Üzer F,Özbudak Ö, Arslan AG. Pulmonary langerhans cell histiocytosis case that not shows any progress although smoking. Akd Med J 2017;2:117-121.