Pulmonary langerhans’ cell histiocytosis: 3 cases of examination and treatment at National Lung Hospital
Main Article Content
Abstract
Pulmonary Langerhans cell histiocytosis is a rare disease, the incidence in children under 15 years old is 0.7 - 4.1/1 million, in adults is 1 - 2/1 million. If the disease is not diagnosed and treated promptly, it can cause serious life-threatening complications such as severe pulmonary hypertension, repeated pneumothorax and severe deadly respiratory failure. We report 3 cases of Langerhans cell histiocytic lung disease examined and treated at the National Lung Hospital. All patients were managed and monitored for 3 to 6 months. Langerhans cell histiocytosis can manifest in many different organs such as the skin, lungs, endocrine glands, liver, spleen, lymph nodes and bone marrow. Diagnosis is mainly based on high-resolution computed tomography and lung biopsy. This report is intended to assist doctors in the diagnosis, prognosis, treatment and monitoring of the disease. And we also discuss a form of interstitial lung disease which are diffuse air cysts in the lung parenchyma on both sides, which is easily misdiagnosed with other lung diseases such as bronchiectasis, emphysema, or chronic obstructive pulmonary disease (COPD).
Article Details
Keywords
Langerhan’s celll histiocytosis, interstitial lung disease, cysts, nodules, lung
References
2. Annels NE, Da Costa CE, Prins FA, et al. Aberrant chemokine receptor expression and chemokine production by Langerhans cells underlies the pathogenesis of Langerhans cell histiocytosis. J Exp Med. 2003;197(10):1385-90.
3. Colasante A, Poletti V, Rosini S, et al. Langerhans cells in Langerhans cell histiocytosis and peripheral adenocarcinomas of the lung. Am Rev Respir Dis. 1993;148(3):752-9.
4. Ryu K, Nam BD, Hwang JH, et al. Early and Atypical Radiologic Presentations of Pulmonary Langerhans Cell Histiocytosis: A Report of Two Cases. Taehan Yongsang Uihakhoe Chi. 2021; 82(3):756-763.
5. Abbott GF, Rosado-de-Christenson ML, Franks TJ, et al. From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. RadioGraphics. 2004;24(3):821-41.
6. Elia D, Torre O, Cassandro R, et al. Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review. Eur J Intern Med. 2015; 26(5):351-6.
7. Soler P, Bergeron A, Kambouchner M, et al. Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis? Am J Respir Crit Care Med. 2000; 162(1):264-70.
8. Castoldi MC, Verrioli A, De Juli E, et al. Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan. Insights Imaging. 2014;5(4):483-92.
9. Kalchiem DO, Paulk A, Kligerman SJ, et al. Development of pulmonary Langerhans cell histiocytosis in a patient with established adenocarcinoma of the lung. 2017. 2017; 9(12):E1079-E1083.
10. Seaman DM, Meyer CA, Gilman MD, et al. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol. 2011;196(6):1305-11.
11. Baldi BG, Carvalho CRR, Dias OM, et al. Diffuse cystic lung diseases: differential diagnosis. J Bras Pneumol. 2017;43(2):140-149.
12. Lee KC, Kang EY, Yong HS, et al. A Stepwise Diagnostic Approach to Cystic Lung Diseases for Radiologists. Korean J Radiol. 2019;20(9):1368-1380.
13. Hoag JB, Sherman M, Fasihuddin Q, et al. A comprehensive review of spontaneous pneumothorax complicating sarcoma. Chest. 2010;138(3):510-8.
14. Silva M, Sabato M, Valentino M, et al. Unusual findings of Langerhans cell histiocytosis in a young asymptomatic patient: case report. J Pulm Respir Med. 2014;4(3).