The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.

Langerhans cell histiocytosis (LCH) results from the proliferation of immunophenotypically and functionally immature, morphologically rounded Langerhans cells along with eosinophils, macrophages, lymphocytes, and commonly, multinucleated giant cells.Controversy exists regarding whether the clonal proliferation is sufficient to consider this a neoplasm or whether the immunologic abnormalities observed in LCH are the cause of the clonal proliferation of lesional Langerhans cells.[1] In either case, the primary treatment is with chemotherapeutic agents. Some of the chemotherapy drugs used also have immunomodulatory activity.

Langerhans cell histiocytosis is the terminology currently preferred over histiocytosis X, eosinophilic granuloma, Abt-Letterer-Siwe disease, Hand-Schuller-Christian disease or diffuse reticuloendotheliosis. This is because the pathologic Langerhans cell is the cell type common to all of these diagnoses and is a member of the dendritic cell family, a cell found intermittently in the dermal-epidermal junction of the skin.[2-4]

References

1. Laman JD, Leenen PJ, Annels NE, et al.: Langerhans-cell histiocytosis 'insight into DC biology'. Trends Immunol 24 (4): 190-6, 2003.  [PUBMED Abstract]
   
   
2. Nezelof C, Basset F, Rousseau MF: Histiocytosis X histogenetic arguments for a Langerhans cell origin. Biomedicine 18 (5): 365-71, 1973.  [PUBMED Abstract]
   
   
3. Coppes-Zantinga A, Egeler RM: The Langerhans cell histiocytosis X files revealed. Br J Haematol 116 (1): 3-9, 2002.  [PUBMED Abstract]
   
   
4. Arceci RJ, Longley BJ, Emanuel PD: Atypical cellular disorders. Hematology Am Soc Hematol Educ Program : 297-314, 2002.  [PUBMED Abstract]
   
   

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