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Encyclopedia of Cancer pp 1–5Cite as

Langerhans Cell Histiocytosis

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Synonyms

Histiocytosis X; LCH

Definition

Langerhans cell histiocytosis (LCH), previously referred to as histiocytosis X, is a rare clonal disorder of Langerhans cell proliferation, involving the skin, bone, and other organs. The disease family consists of the syndromes originally described as eosinophilic granuloma, Hand–Schüller–Christian disease, and Letterer–Siwe disease, a fatal and disseminated form of Langerhans cell histiocytosis which is most commonly seen in children less than two years old. Modern classification of LCH consists of single system versus multisystem and unifocal versus multifocal.

Characteristics

Epidemiology

Most patients diagnosed with LCH are children with a peak percentage of diagnoses occurring between 1 and 3 years of age. The incidence of LCH has been estimated to be five cases per million per year in children. It appears to be more common in boys than in girls (1.2–2:1). The incidence of LCH in adults is thought to be one-half of that in children....

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References

  • Donadieu J, Egeler RM, Pritchard J (2005) Langerhans cell histiocytosis: a clinical update. In: Weitzman S, Egeler RM (eds) Histiocytic disorders of children and adults; basic science, clinical features and therapy. Cambridge University Press, Cambridge, pp 95–129

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  • Henter JI, Tondini C, Pritchard J (2004) Histiocyte disorders. Crit Rev Oncol Hematol 50(2):157–174

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  • McClain KL (2005) Drug therapy for the treatment of Langerhans cell histiocytosis. Expert Opin Pharmacother 6(14):2435–2441

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  • Morimoto A, Ikushima S, Kinugawa N et al (2006) Improved outcome in the treatment of pediatric multifocal Langerhans cell histiocytosis: results from the Japan Langerhans cell Histiocytosis Study Group-96 protocol study. Cancer 107(3):613–619

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  • Savasan S (2006) An enigmatic disease: childhood Langerhans cell histiocytosis in 2005. Int J Dermatol 45(3):182–188

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan

    Akira Morimoto

Authors
  1. Akira Morimoto
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Corresponding author

Correspondence to Akira Morimoto .

Editor information

Editors and Affiliations

  1. Abt. Tumorgenetik, Deutsches Krebsforschungszentrum, Heidelberg, Germany

    Manfred Schwab

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© 2014 Springer-Verlag Berlin Heidelberg

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Morimoto, A. (2014). Langerhans Cell Histiocytosis. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_3273-3

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_3273-3

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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