Scientific Online Resource System

Scripta Scientifica Vox Studentium

Pulmonary Langerhans cell histiocytosis - a rare type of a rare disease

Nikolai Kalvachev, Diana Petkova Gospodinova-Vylkova, Rumen Nenkov, Boyan Balev, Lilyana Petkova


Introduction: Langerhans cell histiocytosis is a rare disease (0.4 in 100,000) with a still highly debated aetiology - either a reactive or a neoplastic process. It is most commonly divided into single-system and multisystem disease. The pulmonary form is part of the former group and occurs mostly in adolescent and adult smokers (2nd through 4th decade of life).

Aim: The aim of this report is to provide more clinical data of one rarely documented disorder.

Materials and Methods: We present a 42-year-old male patient with a respiration-associated pain of unknown origin. His physical examination was unremarkable. A CT scan was indicated, revealing multiple thin-walled cystic lesions and multiple small nodules in the pulmonary parenchyma, predominantly in the middle and upper lung segments, which was suggestive of pulmonary Langerhans cell histiocytosis. Through a video assisted thoracoscopy, several of the nodules were resected and sent for biopsy.

Results: The patient was discharged on the day after the operation. The biopsy confirmed the diagnosis of Langerhans cell histiocytosis. Since systemic corticosteroids were contraindicated in this particular case, the patient was counseled to cease smoking. On the follow-up CT a tendency for a reversal of the condition was observed.

Conclusion: The pulmonary form of Langerhans cell histiocytosis is a rare disease, most commonly seen in relatively young smokers with non-specific presentation. The biopsy is the only definitive diagnostic procedure. The disease may resolve spontaneously or after smoking cessation. In more severe cases corticosteroids, chemotherapy or lung transplantation may be considered.


pulmonary langerhans cell histiocytosis, pulmonology, treatment, diagnosis

Full Text


Kumar V, Abbas AK, Aster JC. Robbins and Cotran Pathologic Basis of Disease. 9th edition. Canada: Elsevier; 2015. p. 621.

Komp DM. Langerhans cell histiocytosis. N Engl J Med. 1987;316(12):747-8. doi: 10.1056/NEJM198703193161211.

Coury F, Annels N, Rivollier A, Olsson S, Santoro A, Speziani C, et al. Langerhans cell histiocytosis reveals a new IL-17A-dependent pathway of dendritic cell fusion. Nat Med. 2008;14(1):81-7. doi: 10.1038/nm1694.

Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116(11):1919-23. doi: 10.1182/blood-2010-04-279083.

Brown NA, Furtado LV, Betz BL, Kiel MJ, Weigelin HC, Lim MS, et al. High prevalence of somatic MAP2K1 mutations in BRAF V600E negative Langerhans cell histiocytosis. Blood. 2014;124(10):1655-8. doi: 10.1182/blood-2014-05-577361.

Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175-84. doi: 10.1002/pbc.24367.

Lorillon G, Tazi A. How I manage pulmonary Langerhans cell histiocytosis. Eur Respir Rev. 2017; 26(145):170070. doi: 10.1183/16000617.0070-2017.

Watanabe R, Tatsumi K, Hashimoto S, Tamakoshi A, Kuriyama T, Respiratory Failure Research Group of Japan. Clinico-epidemiological features of pulmonary histiocytosis X. Intern Med. 2001;40(10):998-1003. doi: 10.2169/internalmedicine.40.998.

Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. N Engl J Med. 2002;346(7):484-90. oi: 10.1056/NEJMoa012087.

Suri HS, Yi ES, Nowakowski GS, Vassalo R. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 2012; 7:16. doi: 10.1186/1750-1172-7-16.

Juvet SC, Hwang D, Downey GP. Rare lung diseases III: Pulmonary Langerhans’ cell histiocytosis. Can Respir J. 2010; 17(3): e55–e62. DOI: 10.1155/2010/216240.

Castoldi MC, Verrioli A, De Juli E, Vanzulli A. Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan. Insights Imaging. 2014; 5(4): 483–92. doi: 10.1007/s13244-014-0338-0.

Grana N. Langerhans cell histiocytosis. Cancer Control. 2014; 21(4):328-34. doi: 10.1177/107327481402100409.

Grobost V, Khouatra C, Lazor R, Cordier JF, Cottin V. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis. Orphanet J Rare Dis. 2014;9:191. doi: 10.1186/s13023-014-0191-8.

Gwenael L. Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med. 2012;186(9):930-2. doi: 10.1164/ajrccm.186.9.930.

Abdellatif T, Gwenaël L, Julien H, Antoine N, Stéphane D, Achille A, et al. Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study. Orphanet J Rare Dis. 2017; 12(1):95. doi: 10.1186/s13023-017-0651-z.

Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, et al. Lung transplantation for pulmonary langerhans’ cell histiocytosis: a multicenter analysis. Transplantation. 2006;81(5):746-50. doi: 10.1097/



Font Size