Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Langerhans Cell Histiocytosis

  • Akira Morimoto
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_3273-3



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.



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....


Diabetes Insipidus Vinca Alkaloid Permanent Sequela Bile Duct Destruction Degenerative Central Nervous System 
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  1. 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 95–129Google Scholar
  2. Henter JI, Tondini C, Pritchard J (2004) Histiocyte disorders. Crit Rev Oncol Hematol 50(2):157–174CrossRefPubMedGoogle Scholar
  3. McClain KL (2005) Drug therapy for the treatment of Langerhans cell histiocytosis. Expert Opin Pharmacother 6(14):2435–2441CrossRefPubMedGoogle Scholar
  4. 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):613–619CrossRefPubMedGoogle Scholar
  5. Savasan S (2006) An enigmatic disease: childhood Langerhans cell histiocytosis in 2005. Int J Dermatol 45(3):182–188CrossRefPubMedGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of PediatricsKyoto Prefectural University of MedicineKyotoJapan