It is possible that the main title of the report Langerhans Cell Histiocytosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Langerhans cell histiocytosis (LCH) is a spectrum of rare disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions). The lesions may include certain distinctive granule-containing cells (Langerhans cells) involved in certain immune responses, as well as other white blood cells (e.g., monocytes, eosinophils). Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement. Most often the bone lesions are painful. Skin rashes may itch or cause painful ulcers especially under the arms or groin area. The pathogenesis (medical cause) is not clearly understood and an ongoing debate continues regarding its cause as a reactive immunologic or neoplastic (cancer-like) process. No infectious agent (virus, bacteria, or fungus) has been associated with LCH. Patients often have a strong family history of immune diseases such as thyroid disease, arthritis, or lupus.
Most affected individuals have single or multiple bone lesions characterized by lytic lesions (holes in the bones). Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs. Affected individuals may have no apparent symptoms (asymptomatic), and may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord. In some cases, other tissues and organs may also be affected, including the skin, lungs, liver, spleen, bone marrow, thymus, thyroid, and brain. In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus or growth failure.
Langerhans cell histiocytosis was selected by the Histiocyte Society to replace the older, less specific term histiocytosis X. Histiocytosis X encompassed three entities known as eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease that were characterized by the accumulation of histiocytes. The "X" denoted that the cause and development of the disorder was not understood. Langerhans cell histiocytosis was chosen because it is now known that Langerhans cells play the central role in the development of these disorders.
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March of Dimes Birth Defects Foundation
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White Plains, NY 10605
Diabetes Insipidus Foundation, Inc.
c/o Mike Gandrud
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Ames, IA 50010
American Lung Association
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NIH/National Heart, Lung and Blood Institute
P.O. Box 30105
Bethesda, MD 20892-0105
MUMS National Parent-to-Parent Network
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Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Histiocytosis Association of Canada
Okanagan Mission RPO
Kelowan, BC, V1W 4A7
Histiocytosis Research Trust
PO Box 435
Leeds, LS17 1GE
Tel: 07850 740241
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Last Updated: 3/4/2009
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