Abstract

Background. Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH.

Methods. We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022.

Results. 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%.

Conclusions. Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.

Keywords: Langerhans cell histiocytosis, children, pulmonary involvement

How to cite

1.
Coşkun Ç, Kutluk T, Yalçın B, et al. Pulmonary involvement in children with Langerhans cell histiocytosis. Turk J Pediatr 2024; 66: 323-331. https://doi.org/10.24953/turkjpediatr.2024.4515

References

  1. Wang D, Cui L, Li ZG, et al. Clinical research of pulmonary Langerhans cell histiocytosis in children. Chin Med J (Engl) 2018; 131: 1793-1798. https://doi.org/10.4103/0366-6999.237400
  2. Barclay M, Devaney R, Bhatt JM. Paediatric pulmonary Langerhans cell histiocytosis. Breathe (Sheff) 2020; 16: 200003. https://doi.org/10.1183/20734735.0003-2020
  3. Ronceray L, Pötschger U, Janka G, Gadner H, Minkov M; German Society for Pediatric Hematology and Oncology, Langerhans Cell Histiocytosis Study Group. Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr 2012; 161: 129-133. https://doi.org/10.1016/j.jpeds.2011.12.035
  4. Della Valle V, Donadieu J, Sileo C, et al. Chest computed tomography findings for a cohort of children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer 2020; 67: e28496. https://doi.org/10.1002/pbc.28496
  5. Bano S, Chaudhary V, Narula MK, et al. Pulmonary Langerhans cell histiocytosis in children: a spectrum of radiologic findings. Eur J Radiol 2014; 83: 47-56. https://doi.org/10.1016/j.ejrad.2013.04.044
  6. Phan M, Birkemeier KL, Crisp M, Santiago J, McGregor NW. Evolution and regression of imaging findings in treated pulmonary Langerhans cell histiocytosis. Proc (Bayl Univ Med Cent) 2020; 34: 156-158. https://doi.org/10.1080/08998280.2020.1823790
  7. Kamath S, Arkader A, Jubran RF. Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution. J Pediatr Orthop 2014; 34: 825-830. https://doi.org/10.1097/BPO.0000000000000218
  8. Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, Lange M. Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol 2018; 35: 6-17. https://doi.org/10.5114/pdia.2017.67095
  9. Tillotson CV, Anjum F, Patel BC. Langerhans cell histiocytosis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024. Available at: https://www.ncbi.nlm.nih.gov/books/NBK430885/
  10. Baig N, Raza MR, Zia N, Maqsood S, Yaqoob N, Ashraf MS. Childhood Langerhans cell histiocytosis: a ten-year study from Pakistan. Pediatric Hematology Oncology Journal 2022; 7: 177-181. https://doi.org/10.1016/j.phoj.2022.10.001
  11. Le Louet S, Barkaoui MA, Miron J, et al. Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study. Orphanet J Rare Dis 2020; 15: 241. https://doi.org/10.1186/s13023-020-01495-5
  12. Eckstein OS, Nuchtern JG, Mallory GB, et al. Management of severe pulmonary Langerhans cell histiocytosis in children. Pediatr Pulmonol 2020; 55: 2074-2081. https://doi.org/10.1002/ppul.24822
  13. Odame I, Li P, Lau L, et al. Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer 2006; 47: 889-893. https://doi.org/10.1002/pbc.20676
  14. Halder R, Anantharaman MB, Seth T, Mahapatra M, Saxena R. Pulmonary Langerhans cell histiocytosis: a not so benign lesion. J Pediatr Hematol Oncol 2019; 41: e122-e124. https://doi.org/10.1097/MPH.0000000000001371
  15. Radzikowska E. Update on pulmonary Langerhans cell histiocytosis. Front Med (Lausanne) 2021; 7: 582581. https://doi.org/10.3389/fmed.2020.582581
  16. Li CW, Li MH, Li JX, Tao RJ, Xu JF, Cao WJ. Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review. J Thorac Dis 2016; 8: 1283-1289. https://doi.org/10.21037/jtd.2016.04.64
  17. Elia D, Torre O, Cassandro R, Caminati A, Harari S. Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review. Eur J Intern Med 2015; 26: 351-356. https://doi.org/10.1016/j.ejim.2015.04.001
  18. Braier J, Latella A, Balancini B, et al. Outcome in children with pulmonary Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43: 765-769. https://doi.org/10.1002/pbc.20112
  19. Varkki S, Tergestina M, Bhonsle VS, Moses PD, Mathai J, Korula S. Isolated pulmonary Langerhans cell histiocytosis. Indian J Pediatr 2013; 80: 700-703. https://doi.org/10.1007/s12098-012-0866-x
  20. Aydoğdu K, Günay E, Fındık G, et al. Pulmonary Langerhans cell histiocytosis; characteristics of 11 cases. Tuberk Toraks 2013; 61: 333-341. https://doi.org/10.5578/tt.5944
  21. Baqir M, Vassallo R, Maldonado F, Yi ES, Ryu JH. Utility of bronchoscopy in pulmonary Langerhans cell histiocytosis. J Bronchology Interv Pulmonol 2013; 20: 309-312. https://doi.org/10.1097/LBR.0000000000000021
  22. Türk M, Türktaş H, Akyürek N. A Case of Langerhans cell histiocytosis with atypical radiological presentation. Turk Thorac J 2015; 16: 154-156. https://doi.org/10.5152/ttd.2014.4110
  23. Sharma S, Dey P. Childhood pulmonary Langerhans cell histiocytosis in bronchoalveolar lavage: a case report along with review of literature. Diagn Cytopathol 2016; 44: 1102-1106. https://doi.org/10.1002/dc.23561
  24. Harari S, Torre O, Cassandro R, Taveira-DaSilva AM, Moss J. Bronchoscopic diagnosis of Langerhans cell histiocytosis and lymphangioleiomyomatosis. Respir Med 2012; 106: 1286-1292. https://doi.org/10.1016/j.rmed.2012.06.012
  25. Simko SJ, Tran HD, Jones J, et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease. Pediatr Blood Cancer 2014; 61: 479-487. https://doi.org/10.1002/pbc.24772
  26. Prakash A, Durugappa T, Ramachandran P, Lahitta AV. Pleurodesis in pulmonary langerhans cell histiocytosis in children: a life-saving measure. Indian J Med Paediatr Oncol 2019; 40: 160-162. https://doi.org/10.4103/ijmpo.ijmpo_250_17
  27. Soyer T, Özyüksel G, Türer ÖB, et al. Bilateral pulmonary Langerhans’s cell histiocytosis is surgical challenge in children: a case report. European J Pediatr Surg Rep 2019; 7: e8-e11. https://doi.org/10.1055/s-0039-1688771
  28. Verma S, Jondhale S, Bansal D, Radotra BD, Singhi SC. Iodopovidone pleurodesis for isolated pulmonary Langerhan’s cell histiocytosis in a two year old child. Indian J Pediatr 2014; 81: 715-718. https://doi.org/10.1007/s12098-013-1181-x
  29. How CH, Hsu HH, Chen JS. Chemical pleurodesis for spontaneous pneumothorax. J Formos Med Assoc 2013; 112: 749-755. https://doi.org/10.1016/j.jfma.2013.10.016
  30. Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: history, classification, pathobiology, clinical manifestations, and prognosis. J Am Acad Dermatol 2018; 78: 1035-1044. https://doi.org/10.1016/j.jaad.2017.05.059