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Semin Respir Crit Care Med 2020; 41(02): 269-279
DOI: 10.1055/s-0039-1700996
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Langerhans Cell Histiocytosis

Brian Shaw
1   Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio
,
Michael Borchers
2   Medical Service, Veterans Affairs Medical Center, Cincinnati, Ohio
3   Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, Ohio
,
Dani Zander
4   Department of Pathology and Laboratory Medicine, University of Cincinnati, Cincinnati, Ohio
,
Nishant Gupta
2   Medical Service, Veterans Affairs Medical Center, Cincinnati, Ohio
3   Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, Ohio
› Author Affiliations
Further Information

Publication History

Publication Date:
12 April 2020 (online)

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Abstract

Pulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

Keywords

PLCH - pneumothorax - MAPK - BRAF - cladribine - smoking
 

  • References

  • 1 Wei P, Lu HW, Jiang S, Fan LC, Li HP, Xu JF. Pulmonary Langerhans cell histiocytosis: case series and literature review. Medicine (Baltimore) 2014; 93 (23) e141
    PubMedGoogle Scholar
  • 2 Emile JF, Abla O, Fraitag S. , et al; Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127 (22) 2672-2681
    CrossrefPubMedGoogle Scholar
  • 3 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 Société de Pathologie Thoracique du Nord. Eur Respir J 1996; 9 (10) 2002-2006
    CrossrefPubMedGoogle Scholar
  • 4 Tazi A. Adult pulmonary Langerhans' cell histiocytosis. Eur Respir J 2006; 27 (06) 1272-1285
    CrossrefPubMedGoogle Scholar
  • 5 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 (07) 484-490
    CrossrefPubMedGoogle Scholar
  • 6 Basset F, Corrin B, Spencer H. , et al. Pulmonary histiocytosis X. Am Rev Respir Dis 1978; 118 (05) 811-820
    PubMedGoogle Scholar
  • 7 Crausman RS, Jennings CA, Tuder RM, Ackerson LM, Irvin CG, King Jr TE. Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 1996; 153 (01) 426-435
    CrossrefPubMedGoogle Scholar
  • 8 Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in the adult. Medicine (Baltimore) 1981; 60 (06) 385-396
    PubMedGoogle Scholar
  • 9 Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 1999; 85 (10) 2278-2290
    CrossrefPubMedGoogle Scholar
  • 10 Schönfeld N, Frank W, Wenig S. , et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993; 60 (01) 38-44
    PubMedGoogle Scholar
  • 11 Travis WD, Borok Z, Roum JH. , et al. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases. Am J Surg Pathol 1993; 17 (10) 971-986
    CrossrefPubMedGoogle Scholar
  • 12 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
    CrossrefPubMedGoogle Scholar
  • 13 Haupt R, Minkov M, Astigarraga I. , et al; Euro Histio Network. 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 (02) 175-184
    CrossrefPubMedGoogle Scholar
  • 14 Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax 2017; 72 (10) 937-945
    CrossrefPubMedGoogle Scholar
  • 15 Kim T, Kwon HJ, Eom M, Kim SW, Sin MH, Jung SH. Bronchial washing cytology of pulmonary Langerhans cell histiocytosis: a case report. J Pathol Transl Med 2017; 51 (04) 444-447
    CrossrefPubMedGoogle Scholar
  • 16 Juvet SC, Hwang D, Downey GP. Rare lung diseases III: pulmonary Langerhans' cell histiocytosis. Can Respir J 2010; 17 (03) e55-e62
    PubMedGoogle Scholar
  • 17 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 (05) 411-426
    CrossrefPubMedGoogle Scholar
  • 18 Lorillon G, Tazi A. How I manage pulmonary Langerhans cell histiocytosis. Eur Respir Rev 2017; 26 (145) 170070
    CrossrefPubMedGoogle Scholar
  • 19 Thomeer M, Demedts M, Vandeurzen K. ; VRGT Working Group on Interstitial Lung Diseases. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg 2001; 56 (03) 163-172
    CrossrefPubMedGoogle Scholar
  • 20 Nagarjun Rao R, Chang CC, Tomashefski Jr JF. Lymphocyte sub-populations and non-Langerhans' cell monocytoid cells in pulmonary Langerhans' cell histiocytosis. Pathol Res Pract 2008; 204 (05) 315-322
    CrossrefPubMedGoogle Scholar
  • 21 Ito T, Carson IV WF, Cavassani KA, Connett JM, Kunkel SL. CCR6 as a mediator of immunity in the lung and gut. Exp Cell Res 2011; 317 (05) 613-619
    CrossrefPubMedGoogle Scholar
  • 22 Sallusto F, Schaerli P, Loetscher P. , et al. Rapid and coordinated switch in chemokine receptor expression during dendritic cell maturation. Eur J Immunol 1998; 28 (09) 2760-2769
    CrossrefPubMedGoogle Scholar
  • 23 Worbs T, Hammerschmidt SI, Förster R. Dendritic cell migration in health and disease. Nat Rev Immunol 2017; 17 (01) 30-48
    CrossrefPubMedGoogle Scholar
  • 24 Botelho FM, Nikota JK, Bauer CM. , et al. Cigarette smoke-induced accumulation of lung dendritic cells is interleukin-1α-dependent in mice. Respir Res 2012; 13: 81
    CrossrefPubMedGoogle Scholar
  • 25 Demedts IK, Bracke KR, Van Pottelberge G. , et al. Accumulation of dendritic cells and increased CCL20 levels in the airways of patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2007; 175 (10) 998-1005
    CrossrefPubMedGoogle Scholar
  • 26 Lommatzsch M, Bratke K, Knappe T. , et al. Acute effects of tobacco smoke on human airway dendritic cells in vivo. Eur Respir J 2010; 35 (05) 1130-1136
    CrossrefPubMedGoogle Scholar
  • 27 Tsoumakidou M, Demedts IK, Brusselle GG, Jeffery PK. Dendritic cells in chronic obstructive pulmonary disease: new players in an old game. Am J Respir Crit Care Med 2008; 177 (11) 1180-1186
    CrossrefPubMedGoogle Scholar
  • 28 Annels NE, Da Costa CE, Prins FA, Willemze A, Hogendoorn PC, Egeler RM. Aberrant chemokine receptor expression and chemokine production by Langerhans cells underlies the pathogenesis of Langerhans cell histiocytosis. J Exp Med 2003; 197 (10) 1385-1390
    CrossrefPubMedGoogle Scholar
  • 29 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 (04) 483-492
    CrossrefPubMedGoogle Scholar
  • 30 Leatherwood DL, Heitkamp DE, Emerson RE. Best cases from the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics 2007; 27 (01) 265-268
    CrossrefPubMedGoogle Scholar
  • 31 Stone S, Lynch D. National Jewish Health classical pulmonary radiology case reports: imaging features of Langerhans cell histiocytosis. QJM 2018; 111 (10) 739-740
    CrossrefPubMedGoogle Scholar
  • 32 Geissmann F, Lepelletier Y, Fraitag S. , et al. Differentiation of Langerhans cells in Langerhans cell histiocytosis. Blood 2001; 97 (05) 1241-1248
    CrossrefPubMedGoogle Scholar
  • 33 de Graaf JH, Tamminga RY, Dam-Meiring A, Kamps WA, Timens W. The presence of cytokines in Langerhans' cell histiocytosis. J Pathol 1996; 180 (04) 400-406
    CrossrefPubMedGoogle Scholar
  • 34 Hayashi T, Rush WL, Travis WD, Liotta LA, Stetler-Stevenson WG, Ferrans VJ. Immunohistochemical study of matrix metalloproteinases and their tissue inhibitors in pulmonary Langerhans' cell granulomatosis. Arch Pathol Lab Med 1997; 121 (09) 930-937
    PubMedGoogle Scholar
  • 35 Bernstrand C, Cederlund K, Sandstedt B. , et al. Pulmonary abnormalities at long-term follow-up of patients with Langerhans cell histiocytosis. Med Pediatr Oncol 2001; 36 (04) 459-468
    CrossrefPubMedGoogle Scholar
  • 36 Hashimoto M, Yanagisawa H, Minagawa S. , et al. TGF-β-dependent dendritic cell chemokinesis in murine models of airway disease. J Immunol 2015; 195 (03) 1182-1190
    CrossrefPubMedGoogle Scholar
  • 37 Soler P, Moreau A, Basset F, Hance AJ. Cigarette smoking-induced changes in the number and differentiated state of pulmonary dendritic cells/Langerhans cells. Am Rev Respir Dis 1989; 139 (05) 1112-1117
    CrossrefPubMedGoogle Scholar
  • 38 Vassallo R, Walters PR, Lamont J, Kottom TJ, Yi ES, Limper AH. Cigarette smoke promotes dendritic cell accumulation in COPD; a Lung Tissue Research Consortium study. Respir Res 2010; 11: 45
    CrossrefPubMedGoogle Scholar
  • 39 Bracke KR, D'hulst AI, Maes T. , et al. Cigarette smoke-induced pulmonary inflammation and emphysema are attenuated in CCR6-deficient mice. J Immunol 2006; 177 (07) 4350-4359
    CrossrefPubMedGoogle Scholar
  • 40 Marchal J, Kambouchner M, Tazi A, Valeyre D, Soler P. Expression of apoptosis-regulatory proteins in lesions of pulmonary Langerhans cell histiocytosis. Histopathology 2004; 45 (01) 20-28
    CrossrefPubMedGoogle Scholar
  • 41 Prasse A, Stahl M, Schulz G. , et al. Essential role of osteopontin in smoking-related interstitial lung diseases. Am J Pathol 2009; 174 (05) 1683-1691
    CrossrefPubMedGoogle Scholar
  • 42 Reibman J, Hsu Y, Chen LC, Bleck B, Gordon T. Airway epithelial cells release MIP-3alpha/CCL20 in response to cytokines and ambient particulate matter. Am J Respir Cell Mol Biol 2003; 28 (06) 648-654
    CrossrefPubMedGoogle Scholar
  • 43 Yousem SA, Colby TV, Chen YY, Chen WG, Weiss LM. Pulmonary Langerhans' cell histiocytosis: molecular analysis of clonality. Am J Surg Pathol 2001; 25 (05) 630-636
    CrossrefPubMedGoogle Scholar
  • 44 Willman CL, Busque L, Griffith BB. , et al. Langerhans'-cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med 1994; 331 (03) 154-160
    CrossrefPubMedGoogle Scholar
  • 45 Willman CL, McClain KL. An update on clonality, cytokines, and viral etiology in Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998; 12 (02) 407-416
    CrossrefPubMedGoogle Scholar
  • 46 Yu RC, Chu C, Buluwela L, Chu AC. Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet 1994; 343 (8900): 767-768
    CrossrefPubMedGoogle Scholar
  • 47 Eroglu Z, Ribas A. Combination therapy with BRAF and MEK inhibitors for melanoma: latest evidence and place in therapy. Ther Adv Med Oncol 2016; 8 (01) 48-56
    CrossrefPubMedGoogle Scholar
  • 48 Durham BH, Getta B, Dietrich S. , et al. Genomic analysis of hairy cell leukemia identifies novel recurrent genetic alterations. Blood 2017; 130 (14) 1644-1648
    CrossrefPubMedGoogle Scholar
  • 49 Kim WW, Ha TK, Bae SK. Clinical implications of the BRAF mutation in papillary thyroid carcinoma and chronic lymphocytic thyroiditis. J Otolaryngol Head Neck Surg 2018; 47 (01) 4
    CrossrefPubMedGoogle Scholar
  • 50 Allen CE, Parsons DW. Biological and clinical significance of somatic mutations in Langerhans cell histiocytosis and related histiocytic neoplastic disorders. Hematology (Am Soc Hematol Educ Program) 2015; 2015: 559-564
    CrossrefPubMedGoogle Scholar
  • 51 Mourah S, How-Kit A, Meignin V. , et al. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis. Eur Respir J 2016; 47 (06) 1785-1796
    CrossrefPubMedGoogle Scholar
  • 52 Roden AC, Yi ES. Pulmonary Langerhans cell histiocytosis: an update from the pathologists' perspective. Arch Pathol Lab Med 2016; 140 (03) 230-240
    CrossrefPubMedGoogle Scholar
  • 53 Brabencova E, Tazi A, Lorenzato M. , et al. Langerhans cells in Langerhans cell granulomatosis are not actively proliferating cells. Am J Pathol 1998; 152 (05) 1143-1149
    PubMedGoogle Scholar
  • 54 Senechal B, Elain G, Jeziorski E. , et al. Expansion of regulatory T cells in patients with Langerhans cell histiocytosis. PLoS Med 2007; 4 (08) e253
    CrossrefPubMedGoogle Scholar
  • 55 Caffrey PR, Altman RS. Pulmonary alveolar microlitbiasis occurring in premature twins. J Pediatr 1965; 66: 758-763
    CrossrefPubMedGoogle Scholar
  • 56 Chilosi M, Facchetti F, Caliò A. , et al. Oncogene-induced senescence distinguishes indolent from aggressive forms of pulmonary and non-pulmonary Langerhans cell histiocytosis. Leuk Lymphoma 2014; 55 (11) 2620-2626
    CrossrefPubMedGoogle Scholar
  • 57 Knudson Jr AG. Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci U S A 1971; 68 (04) 820-823
    CrossrefPubMedGoogle Scholar
  • 58 Singla A, Kopras EJ, Gupta N. Spontaneous pneumothorax and air travel in Pulmonary Langerhans cell histiocytosis: a patient survey. Respir Investig 2019; S2212-5345(19)30148-0
    PubMedGoogle Scholar
  • 59 Colby TV, Lombard C. Histiocytosis X in the lung. Hum Pathol 1983; 14 (10) 847-856
    CrossrefPubMedGoogle Scholar
  • 60 Minghini A, Trogdon SD. Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. Am Surg 1998; 64 (11) 1040-1042
    PubMedGoogle Scholar
  • 61 Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004; 125 (03) 1028-1032
    CrossrefPubMedGoogle Scholar
  • 62 Fartoukh M, Humbert M, Capron F. , et al. Severe pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med 2000; 161 (01) 216-223
    CrossrefPubMedGoogle Scholar
  • 63 Harari S, Brenot F, Barberis M, Simmoneau G. Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension. Chest 1997; 111 (04) 1142-1144
    PubMedGoogle Scholar
  • 64 Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 2000; 342 (26) 1969-1978
    CrossrefPubMedGoogle Scholar
  • 65 Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics 2004; 24 (03) 821-841
    CrossrefPubMedGoogle Scholar
  • 66 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 (02) 104-109
    CrossrefPubMedGoogle Scholar
  • 67 Brauner MW, Grenier P, Tijani K, Battesti JP, Valeyre D. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology 1997; 204 (02) 497-502
    CrossrefPubMedGoogle Scholar
  • 68 Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with high-resolution CT. Radiology 1989; 172 (01) 255-258
    CrossrefPubMedGoogle Scholar
  • 69 Seely JM, Salahudeen Sr S, Cadaval-Goncalves AT. , et al. Pulmonary Langerhans cell histiocytosis: a comparative study of computed tomography in children and adults. J Thorac Imaging 2012; 27 (01) 65-70
    CrossrefPubMedGoogle Scholar
  • 70 Tazi A, de Margerie C, Naccache JM. , et al. The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. Orphanet J Rare Dis 2015; 10: 30
    CrossrefPubMedGoogle Scholar
  • 71 Thurlbeck WM, Churg A. Thurlbeck's Pathology of the Lung, 3rd ed. New York: Thieme; 2005
    Google Scholar
  • 72 Lieberman PH, Jones CR, Steinman RM. , et al. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol 1996; 20 (05) 519-552
    CrossrefPubMedGoogle Scholar
  • 73 Kambouchner M, Basset F, Marchal J, Uhl JF, Hance AJ, Soler P. Three-dimensional characterization of pathologic lesions in pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med 2002; 166 (11) 1483-1490
    CrossrefPubMedGoogle Scholar
  • 74 Emile JF, Wechsler J, Brousse N. , et al. Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples. Am J Surg Pathol 1995; 19 (06) 636-641
    CrossrefPubMedGoogle Scholar
  • 75 Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest 1999; 115 (05) 1452-1455
    CrossrefPubMedGoogle Scholar
  • 76 Tazi A, Montcelly L, Bergeron A, Valeyre D, Battesti JP, Hance AJ. Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med 1998; 157 (6 Pt 1): 2007-2010
    CrossrefPubMedGoogle Scholar
  • 77 Von Essen S, West W, Sitorius M, Rennard SI. Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X. Chest 1990; 98 (03) 765-767
    CrossrefPubMedGoogle Scholar
  • 78 Tazi A, Lorillon G, Haroche J. , et al. Vinblastine chemotherapy in adult patients with Langerhans cell histiocytosis: a multicenter retrospective study. Orphanet J Rare Dis 2017; 12 (01) 95
    CrossrefPubMedGoogle Scholar
  • 79 Goh NS, McDonald CE, MacGregor DP, Pretto JJ, Brodie GN. Successful treatment of Langerhans cell histiocytosis with 2-chlorodeoxyadenosine. Respirology 2003; 8 (01) 91-94
    CrossrefPubMedGoogle Scholar
  • 80 Pardanani A, Phyliky RL, Li CY, Tefferi A. 2-Chlorodeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. Mayo Clin Proc 2003; 78 (03) 301-306
    CrossrefPubMedGoogle Scholar
  • 81 Saven A, Burian C. Cladribine activity in adult Langerhans-cell histiocytosis. Blood 1999; 93 (12) 4125-4130
    CrossrefPubMedGoogle Scholar
  • 82 Aerni MR, Aubry MC, Myers JL, Vassallo R. Complete remission of nodular pulmonary Langerhans cell histiocytosis lesions induced by 2-chlorodeoxyadenosine in a non-smoker. Respir Med 2008; 102 (02) 316-319
    CrossrefPubMedGoogle Scholar
  • 83 Epaud R, Ducou Le Pointe H, Fasola S. , et al. Cladribine improves lung cysts and pulmonary function in a child with histiocytosis. Eur Respir J 2015; 45 (03) 831-833
    CrossrefPubMedGoogle Scholar
  • 84 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
    CrossrefPubMedGoogle Scholar
  • 85 Lazor R, Etienne-Mastroianni B, Khouatra C, Tazi A, Cottin V, Cordier JF. Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy. Thorax 2009; 64 (03) 274-275
    CrossrefPubMedGoogle Scholar
  • 86 Lorillon G, Bergeron A, Detourmignies L. , et al. Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med 2012; 186 (09) 930-932
    CrossrefPubMedGoogle Scholar
  • 87 Weitzman S, Braier J, Donadieu J. , et al. 2′-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH). Results of the LCH-S-98 protocol of the Histiocyte Society. Pediatr Blood Cancer 2009; 53 (07) 1271-1276
    CrossrefPubMedGoogle Scholar
  • 88 Diamond EL, Subbiah V, Lockhart AC. , et al. Vemurafenib for BRAF V600-mutant Erdheim-Chester disease and Langerhans cell histiocytosis: analysis of data from the histology-independent, phase 2, open-label VE-BASKET study. JAMA Oncol 2018; 4 (03) 384-388
    CrossrefPubMedGoogle Scholar
  • 89 Diamond EL, Durham BH, Ulaner GA. , et al. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature 2019; 567 (7749): 521-524
    CrossrefPubMedGoogle Scholar
  • 90 Lorillon G, Jouenne F, Baroudjian B. , et al. Response to trametinib of a pulmonary Langerhans cell histiocytosis harboring a MAP2K1 deletion. Am J Respir Crit Care Med 2018; 198 (05) 675-678
    CrossrefPubMedGoogle Scholar
  • 91 Radzikowska E, Błasińska-Przerwa K, Wiatr E, Bestry I, Langfort R, Roszkowski-Śliż K. Pneumothorax in patients with pulmonary Langerhans cell histiocytosis. Lung 2018; 196 (06) 715-720
    CrossrefPubMedGoogle Scholar
  • 92 DiMaggio LA, Lippes HA, Lee RV. Histiocytosis X and pregnancy. Obstet Gynecol 1995; 85 (5 Pt 2): 806-809
    CrossrefPubMedGoogle Scholar
  • 93 King Jr TE. Restrictive lung disease in pregnancy. Clin Chest Med 1992; 13 (04) 607-622
    PubMedGoogle Scholar
  • 94 Boehler A. Lung transplantation for cystic lung diseases: lymphangioleiomyomatosis, histiocytosis x, and sarcoidosis. Semin Respir Crit Care Med 2001; 22 (05) 509-516
    Article in Thieme ConnectPubMedGoogle Scholar
  • 95 Etienne B, Bertocchi M, Gamondes JP. , et al. Relapsing pulmonary Langerhans cell histiocytosis after lung transplantation. Am J Respir Crit Care Med 1998; 157 (01) 288-291
    CrossrefPubMedGoogle Scholar
  • 96 Gabbay E, Dark JH, Ashcroft T. , et al. Recurrence of Langerhans' cell granulomatosis following lung transplantation. Thorax 1998; 53 (04) 326-327
    CrossrefPubMedGoogle Scholar
  • 97 Habib SB, Congleton J, Carr D. , et al. Recurrence of recipient Langerhans' cell histiocytosis following bilateral lung transplantation. Thorax 1998; 53 (04) 323-325
    CrossrefPubMedGoogle Scholar
  • 98 Sulica R, Teirstein A, Padilla ML. Lung transplantation in interstitial lung disease. Curr Opin Pulm Med 2001; 7 (05) 314-322
    CrossrefPubMedGoogle Scholar
  • 99 Dauriat G, Mal H, Thabut G. , et al. Lung transplantation for pulmonary Langerhans' cell histiocytosis: a multicenter analysis. Transplantation 2006; 81 (05) 746-750
    CrossrefPubMedGoogle Scholar
  • 100 Wajda N, Zhu Z, Jandarov R, Dilling DF, Gupta N. Clinical outcomes and survival following lung transplantation in patients with pulmonary Langerhans cell histiocytosis. Respirology 2019
    PubMedGoogle Scholar
  • 101 Girschikofsky M, Arico M, Castillo D. , et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis 2013; 8: 72
    CrossrefPubMedGoogle Scholar
  • 102 Suri HS, Yi ES, Nowakowski GS, Vassallo R. Pulmonary Langerhans cell histiocytosis. Orphanet J Rare Dis 2012; 7: 16
    CrossrefPubMedGoogle Scholar
  • 103 Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans' cell histiocytosis. Thorax 2000; 55 (05) 405-416
    CrossrefPubMedGoogle Scholar