Browse > Article
http://dx.doi.org/10.4046/trd.2009.66.2.98

Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea  

Kim, Chul (Department of Internal Medicine, Soonchunhyang University College of Medicine)
Jeong, Sung Hwan (Department of Internal Medicine, Gachon University Gil Medical Center)
Shim, Jae Jeong (Department of Internal Medicine, Guro Hospital, Korea University Medical Center)
Cha, Seung-Ick (Department of Internal Medicine, Kyungpook National University School of Medicine)
Son, Choonhee (Department of Internal Medicine, Dong-A University College of Medicine)
Chung, Man Pyo (Department of Internal Medicine, Sungkyunkwan University School of Medicine)
Park, Hye Yoon (Department of Internal Medicine, Sungkyunkwan University School of Medicine)
Kim, Young Whan (Department of Internal Medicine, Seoul National University College of Medicine)
Park, Jong Sun (Department of Internal Medicine, Seoul National University College of Medicine)
Uh, Soo-Taek (Department of Internal Medicine, Soonchunhyang University College of Medicine)
Park, Choon Sik (Department of Internal Medicine, Soonchunhyang University College of Medicine)
Kim, Dong Soon (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine)
Cho, Kyung Wook (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine)
Song, Jin Woo (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine)
Jegal, Yang Jin (Department of Internal Medicine, Ulsan University College of Medicine)
Park, Moo Suk (Department of Internal Medicine, Yonsei University College of Medicine)
Park, Byung Hoon (Department of Internal Medicine, Yonsei University College of Medicine)
Lee, Jin Hwa (Department of Internal Medicine, Ewha Womans University School of Medicine)
Hur, Jin Won (Department of Internal Medicine, College of Medicine, Inje University)
Yum, Ho-Kee (Department of Internal Medicine, College of Medicine, Inje University)
Lee, Hong-Lyeol (Department of Internal Medicine, College of Medicine, Inha University)
Park, Yong Bum (Department of Internal Medicine, College of Medicine, Hallym University)
Publication Information
Tuberculosis and Respiratory Diseases / v.66, no.2, 2009 , pp. 98-103 More about this Journal
Abstract
Background: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. Methods: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. Results: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18-67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1-180 months) and only two patients died during this period. Conclusion: This study provides a national survey of the patients with PLCH during a long follow-up period.
Keywords
Langerhans; Langerhans-cell histiocytosis; Pulmonary diseases; Histiocytosis;
Citations & Related Records

Times Cited By SCOPUS : 1
연도 인용수 순위
1 Zeid NA, Muller HK. Tobacco smoke induced lung granulomas and tumors: association with pulmonary Langerhans cells. Pathology 1995;27:247-54   DOI   ScienceOn
2 Sulica R, Teirstein A, Padilla ML. Lung transplantation in interstitial lung disease. Curr Opin Pulm Med 2001;7:314-22   DOI   PUBMED   ScienceOn
3 Watanabe R, Tatsumi K, Hashimoto S, Tamakoshi A, Kuriyama T. Clinico-epidemiological features of pulmonary histiocytosis X. Intern Med 2001;40:998-1003   DOI   ScienceOn
4 Crausman RS, Jennings CA, Tuder RM, Ackerson LM, Irvin CG, King TE Jr. Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 1996;153:426-35   DOI   PUBMED   ScienceOn
5 Minghini A, Trogdon SD. Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. Am Surg 1998;64:1040-2   PUBMED
6 Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004;125:1028-32   DOI   ScienceOn
7 Hwang YI, Park GM, Yim JJ, Yoo CG, Lee CT, Kim YW, et al. Clinical characteristics of pulmonary histiocytosis X. Tuberc Respir Dis 2001;51:346-53   DOI
8 Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med 2002;346:484-90   DOI   ScienceOn
9 Tazi A. Adult pulmonary Langerhans' cell histiocytosis. Eur Respir J 2006;27:1272-85   DOI   ScienceOn
10 Boehler A. Lung transplantation for cystic lung diseases: lymphangioleiomyomatosis, histiocytosis X, and sarcoidosis. Semin Respir Crit Care Med 2001; 22:509-16   DOI   PUBMED   ScienceOn
11 Colby TV, Lombard C. Histiocytosis X in the lung. Hum Pathol 1983;14:847-56   DOI   PUBMED
12 Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 2000;342:1969-78   DOI   ScienceOn
13 Sundar KM, Gosselin MV, Chung HL, Cahill BC. Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest 2003;123:1673-83   DOI   ScienceOn
14 Gabbay E, Dark JH, Ashcroft T, Milne D, Gibson GJ, Healy M, et al. Recurrence of Langerhans' cell granulomatosis following lung transplantation. Thorax 1998;53:326-7   DOI   ScienceOn
15 Habib SB, Congleton J, Carr D, Partridge J, Corrin B, Geddes DM, et al. Recurrence of recipient Langerhans' cell histiocytosis following bilateral lung transplanta tion. Thorax 1998;53:323-5   DOI   ScienceOn
16 Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 1999;85:2278-90   DOI   ScienceOn
17 Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in the adult. Medicine (Baltimore) 1981;60:385-96   DOI   ScienceOn
18 Gaensler EA, Carrington CB. Open biopsy for chronic diffuse infiltrative lung disease: clinical, roentgenographic, and physiological correlations in 502 patients. Ann Thorac Surg 1980;30:411-26   DOI   ScienceOn
19 Travis WD, Borok Z, Roum JH, Zhang J, Feuerstein I, Ferrans VJ, et al. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases. Am J Surg Pathol 1993;17:971-86   DOI   ScienceOn
20 Basset F, Corrin B, Spencer H, Lacronique J, Roth C, Soler P, et al. Pulmonary histiocytosis X. Am Rev Respir Dis 1978;118:811-20   PUBMED
21 Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37:104-9   DOI   ScienceOn
22 Von Essen S, West W, Sitorius M, Rennard SI. Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X. Chest 1990;98:765-7   DOI   ScienceOn
23 Kim YM, Park YI, Choi YK, Lee JS, Lee WC, Hong JH, et al. A case of primary pulmonary histiocytosis-X associated with central diabetes insipidus. Tuberc Respir Dis 1999;46:110-5   DOI
24 Schonfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H, et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993;60:38-44   PUBMED
25 Delobbe A, Durieu J, Duhamel A, Wallaert B. Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Soci$\t$\ de Pathologie Thoracique du Nord. Eur Respir J 1996;9:2002-6   DOI   ScienceOn
26 Tazi A, Soler P, Hance AJ. Adult pulmonary Lan gerhans' cell histiocytosis. Thorax 2000;55:405-16   DOI   ScienceOn
27 Moore AD, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, et al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989;172:249-54   DOI   PUBMED
28 Youkeles LH, Grizzanti JN, Liao Z, Chang CJ, Rosenstreich DL. Decreased tobacco-glycoprotein-induced lymphocyte proliferation in vitro in pulmonary eosinophilic granuloma. Am J Respir Crit Care Med 1995;151:145-50   DOI   PUBMED   ScienceOn
29 Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest 1999;115:1452-5   DOI   ScienceOn
30 Hong SJ, Ahn KH, Lee WY, Kong SJ, Yong SJ, Shin KC. A case of pulmonary histiocytosis-X associated with bilateral, recurrent, and spontaneous pneumo thorax. Tuberc Respir Dis 1994;41:152-7   DOI
31 Etienne B, Bertocchi M, Gamondes JP, Thevenet F, Boudard C, Wiesendanger T, et al. Relapsing pulmonary Langerhans cell histiocytosis after lung transplan tation. Am J Respir Crit Care Med 1998;157:288-91   DOI   PUBMED   ScienceOn