Clinical Analysis on Pulmonary Langerhans’ Cells Histiocytosis and Literatures Review_West China Medical Journal

Authors：

 WANGDan ,  LIUChun-tao

Department of Respiratory Medicine, West China Hospital, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

LIUChun-tao, Email: taosen666@vip.163.com

Keywords：

Pulmonary Langerhans' cells histiocytosis; Clinical features; High resolution CT

DOI：

10.7507/1002-0179.20150575

Video：

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Objective To investigate the clinical features of Pulmonary Langerhans' cells histiocytosis (PLCH). Methods Four cases of PLCH diagnosed by histopathologic examination between August 2004 and September 2013 were retrospectively analyzed. Results Four male patients aged from 19 to 46 year old, including three smokers. The main symptoms were chest tightness, cough, and dyspnea. Pneumothorax was presented in two cases, and tuberculosis was in one. The chest high resolution CT (HRCT) revealed lung cysts, nodles, and reticular changes predominantly in the upper and middle lung fields. The pathological Langerhans' cells infiltration were found in the histological biopsy of lesions of the 4 cases. All of the patients were positive in the immuno-histological staining for the S-100 and CD1a antigens. Two cases were positive in Langrin staining (other two patients didn't underwent the staining). Two of the 4 patients were given oral steroid, and the symptoms were improved in one of them. The case with pulmonary tuberculosis improved in symptoms and CT results showed the absorption of the lesion after anti-tuberculosis therapy. Three cases were not followed up. Conclusion PLCH patients were mainly young adults, often presented with chest tightness, cough, and dyspnea. The clinical features of chest HRCT are bilateral cysts, nodules and reticular changes. The disease may be defined by the finding of pathologic Langerhans' cells or the positive staining for CD1a antigens or Langerin.

Citation： WANGDan, LIUChun-tao. Clinical Analysis on Pulmonary Langerhans’ Cells Histiocytosis and Literatures Review. West China Medical Journal, 2015, 30(11): 2013-2018. doi: 10.7507/1002-0179.20150575 Copy

1.	Aricò M, Girschikofsky M, Généreau T, et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society[J]. Eur J Cancer, 2003, 39(16):2341-2348.
2.	Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans' cell his tiocytosis[J]. N Engl J Med, 2000, 342(26):1969-1978.
3.	Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults[J]. N Engl J Med, 2002, 346(7):484-490.
4.	Thomeer M, Demedts M, Vandeurzen K, et al. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders[J]. Acta Clin Belg, 2001, 56(3):163-172.
5.	Watanabe R, Tatsumi K, Hashimoto S, et al. Clinico-epidemiological features of pulmonary histiocytosis X[J]. Intern Med, 2001, 40(10):998-1003.
6.	Tazi A. Adult pulmonary Langerhans'cell histiocytosis[J]. Eur Respir J, 2006, 27(6):1272-1285.
7.	Schönfeld N, Frank W, Wenig S, et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X[J]. Respiration, 1993, 60(1):38-44.
8.	Suri HS, Yi ES, Nowakowski GS, et al. Pulmonary Langerhans cell histiocytosis[J]. Orphanet J Rare Dis, 2012, 19:7.
9.	Yousem SA, Colby TV, Chen YY, et al. Pulmonary Langerhans' cell histiocytosis:molecular analysis of clonality[J]. Am J Surg Pathol, 2001, 25(5):630-636.
10.	Yousem SA, Dacic S, Nikiforov YE, et al. Pulmonary Langerhans cell histiocytosis:profiling of multifocal tumors using next-generation sequencing identifies concordant occurrence of BRAF V600E mutations[J]. Chest, 2013, 143(6):1679-1684.
11.	Roden AC, Hu X, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis:clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases[J]. Am J Surg Pathol, 2014, 38(4):548-551.
12.	Satter EK, High WA. Langerhans cell histiocytosis:a review of the current recommendations of the Histiocyte Society[J]. Pediatr Dermatol, 2008, 25(3):291-295.
13.	Clarke BE. Cystic lung disease[J]. J Clin Pathol, 2013, 66(10):904-908.
14.	蔡柏蔷, 李龙芸. 协和呼吸病学[M]. 2版. 北京:中国协和医科大学出版社, 2005.
15.	Travis WD, Borok Z, Roum JH, et al. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases[J]. Am J Surg Pathol, 1993, 17(10):971-986.
16.	Kambouchner M, Basset F, Marchal J, et al. Three-dimensional characterization of pathologic lesions in pulmonary Langerhans cell histiocytosis[J]. Am J Respir Crit Care Med, 2002, 166(11):1483-1490.
17.	Weitzman S, Egeler RM. Histocytic disorders of children and adults[M]. Cambridge:Cambridge University Press, 2005:14-39.
18.	Bechan GI, Egeler RM, Arceci RJ. Biology of Langerhans cells and langerhans cell histiocytosis[J]. Int Rev Cytol, 2006, 254:1-43.
19.	Querings K, Starz H, Balda BR. Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases[J]. Acta Derm Venereol, 2006, 86(1):39-43.
20.	Geissmann F, Lepelletier Y, Fraitag S, et al. Differentiation of Langerhans cells in Langerhans cell histiocytosis[J]. Blood, 2001, 97(5):1241-1248.
21.	Valladeau J, Ravel O, Dezutter-Dambuyant C, et al. Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules[J]. Immunity, 2000, 12(1):71-81.
22.	Beverley PC, Egeler RM, Arceci RJ, et al. The nikolas symposia and histiocytosis[J]. Nat Rev Cancer, 2005, 5(6):488-494.
23.	Wong CY, Law GT, Wong KY, et al. A case of Langerhans' cell histiocytosis of the lung presenting with haemoptysis[J]. Hong Kong Med J, 2004, 10(1):49-51.
24.	Lad M, Duncan S, Patten DK. Pulmonary Langerhans cell histiocytosis[J]. Clin Adv Hematol Oncol, 2013, 11(9):602-603.
25.	Zeppa P, Cozzolino I, Russo M, et al. Pulmonary Langerhans cell histiocytosis (histiocytosis X) on bronchoalveolar lavage:a report of 2 cases[J]. Acta Cytol, 2007, 51(3):480-482.
26.	Mendez JL, Nadrous HF, Vassallo R, et al. Pneumothorax in pulmonary Langerhans' cell histiocytosis[J]. Chest, 2003, 125(3):1028-1032.
27.	Sundar KM, Gosselin MV, Chung HL, et al. Pulmonary Langerhans cell histiocytosis:emerging concepts in pathobiology, radiology, and clinical evolution of disease[J]. Chest, 2003, 123(5):1673-1683.
28.	Nair A, Hansell DM. High-resolution computed tomography features of smoking-related interstitial lung disease[J]. Semin Ultrasound CT MR, 2014, 35(1):59-71.
29.	Seely JM, Salahudeen S, Cadaval-Goncalves AT, et al. Pulmonary Langerhans cell histiocytosis:a comparative study of computed tomography in children and adults[J]. J Thorac Imaging, 2012, 27(1):65-70.
30.	Gadner H, Grois N, Arico M, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis[J]. J Pediatr, 2001, 138(5):728-734.
31.	Ng-Cheng-Hin B, O'hanlon-Brown C, Alifrangis C, et al. Langerhans cell histiocytosis:old disease new treatment[J]. QJM, 2011, 104(2):89-96.
32.	Von Essen S, West W, Sitorius M, et al. Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X[J]. Chest, 1990, 98(3):765-767.
33.	Mogulkoc N, Veral A, Bishop PW, et al. Pulmonary Langerhans' cell histiocytosis:radiologic resolution following smoking cessation[J]. Chest, 1999, 115(5):1452-1455.
34.	Dauriat G, Mal H, Thabut G, et al. Lung transplantation for pulmonary Langerhans' cell histiocytosis:a multicenter analysis[J]. Transplantation, 2006, 81(5):746-750.

1. Aricò M, Girschikofsky M, Généreau T, et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society[J]. Eur J Cancer, 2003, 39(16):2341-2348.
2. Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans' cell his tiocytosis[J]. N Engl J Med, 2000, 342(26):1969-1978.
3. Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults[J]. N Engl J Med, 2002, 346(7):484-490.
4. Thomeer M, Demedts M, Vandeurzen K, et al. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders[J]. Acta Clin Belg, 2001, 56(3):163-172.
5. Watanabe R, Tatsumi K, Hashimoto S, et al. Clinico-epidemiological features of pulmonary histiocytosis X[J]. Intern Med, 2001, 40(10):998-1003.
6. Tazi A. Adult pulmonary Langerhans'cell histiocytosis[J]. Eur Respir J, 2006, 27(6):1272-1285.
7. Schönfeld N, Frank W, Wenig S, et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X[J]. Respiration, 1993, 60(1):38-44.
8. Suri HS, Yi ES, Nowakowski GS, et al. Pulmonary Langerhans cell histiocytosis[J]. Orphanet J Rare Dis, 2012, 19:7.
9. Yousem SA, Colby TV, Chen YY, et al. Pulmonary Langerhans' cell histiocytosis:molecular analysis of clonality[J]. Am J Surg Pathol, 2001, 25(5):630-636.
10. Yousem SA, Dacic S, Nikiforov YE, et al. Pulmonary Langerhans cell histiocytosis:profiling of multifocal tumors using next-generation sequencing identifies concordant occurrence of BRAF V600E mutations[J]. Chest, 2013, 143(6):1679-1684.
11. Roden AC, Hu X, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis:clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases[J]. Am J Surg Pathol, 2014, 38(4):548-551.
12. Satter EK, High WA. Langerhans cell histiocytosis:a review of the current recommendations of the Histiocyte Society[J]. Pediatr Dermatol, 2008, 25(3):291-295.
13. Clarke BE. Cystic lung disease[J]. J Clin Pathol, 2013, 66(10):904-908.
14. 蔡柏蔷, 李龙芸. 协和呼吸病学[M]. 2版. 北京:中国协和医科大学出版社, 2005.
15. Travis WD, Borok Z, Roum JH, et al. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases[J]. Am J Surg Pathol, 1993, 17(10):971-986.
16. Kambouchner M, Basset F, Marchal J, et al. Three-dimensional characterization of pathologic lesions in pulmonary Langerhans cell histiocytosis[J]. Am J Respir Crit Care Med, 2002, 166(11):1483-1490.
17. Weitzman S, Egeler RM. Histocytic disorders of children and adults[M]. Cambridge:Cambridge University Press, 2005:14-39.
18. Bechan GI, Egeler RM, Arceci RJ. Biology of Langerhans cells and langerhans cell histiocytosis[J]. Int Rev Cytol, 2006, 254:1-43.
19. Querings K, Starz H, Balda BR. Clinical spectrum of cutaneous Langerhans' cell histiocytosis mimicking various diseases[J]. Acta Derm Venereol, 2006, 86(1):39-43.
20. Geissmann F, Lepelletier Y, Fraitag S, et al. Differentiation of Langerhans cells in Langerhans cell histiocytosis[J]. Blood, 2001, 97(5):1241-1248.
21. Valladeau J, Ravel O, Dezutter-Dambuyant C, et al. Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules[J]. Immunity, 2000, 12(1):71-81.
22. Beverley PC, Egeler RM, Arceci RJ, et al. The nikolas symposia and histiocytosis[J]. Nat Rev Cancer, 2005, 5(6):488-494.
23. Wong CY, Law GT, Wong KY, et al. A case of Langerhans' cell histiocytosis of the lung presenting with haemoptysis[J]. Hong Kong Med J, 2004, 10(1):49-51.
24. Lad M, Duncan S, Patten DK. Pulmonary Langerhans cell histiocytosis[J]. Clin Adv Hematol Oncol, 2013, 11(9):602-603.
25. Zeppa P, Cozzolino I, Russo M, et al. Pulmonary Langerhans cell histiocytosis (histiocytosis X) on bronchoalveolar lavage:a report of 2 cases[J]. Acta Cytol, 2007, 51(3):480-482.
26. Mendez JL, Nadrous HF, Vassallo R, et al. Pneumothorax in pulmonary Langerhans' cell histiocytosis[J]. Chest, 2003, 125(3):1028-1032.
27. Sundar KM, Gosselin MV, Chung HL, et al. Pulmonary Langerhans cell histiocytosis:emerging concepts in pathobiology, radiology, and clinical evolution of disease[J]. Chest, 2003, 123(5):1673-1683.
28. Nair A, Hansell DM. High-resolution computed tomography features of smoking-related interstitial lung disease[J]. Semin Ultrasound CT MR, 2014, 35(1):59-71.
29. Seely JM, Salahudeen S, Cadaval-Goncalves AT, et al. Pulmonary Langerhans cell histiocytosis:a comparative study of computed tomography in children and adults[J]. J Thorac Imaging, 2012, 27(1):65-70.
30. Gadner H, Grois N, Arico M, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis[J]. J Pediatr, 2001, 138(5):728-734.
31. Ng-Cheng-Hin B, O'hanlon-Brown C, Alifrangis C, et al. Langerhans cell histiocytosis:old disease new treatment[J]. QJM, 2011, 104(2):89-96.
32. Von Essen S, West W, Sitorius M, et al. Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X[J]. Chest, 1990, 98(3):765-767.
33. Mogulkoc N, Veral A, Bishop PW, et al. Pulmonary Langerhans' cell histiocytosis:radiologic resolution following smoking cessation[J]. Chest, 1999, 115(5):1452-1455.
34. Dauriat G, Mal H, Thabut G, et al. Lung transplantation for pulmonary Langerhans' cell histiocytosis:a multicenter analysis[J]. Transplantation, 2006, 81(5):746-750.

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Clinical Analysis on Pulmonary Langerhans’ Cells Histiocytosis and Literatures Review

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