Langerhans Cell Histiocytosis

Important
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.

Synonyms

Langerhans-Cell Granulomatosis
Non-Lipid Reticuloendotheliosis
Systemic Aleukemic Reticuloendotheliosis
Type II Histiocytosis
LCH
Histiocytosis X
LC’s
Langerhans-Cell

Disorder Subdivisions

Abt-Letterer-Siwe Disease
Eosinophilic Granuloma
Hand-Schueller-Christian Syndrome
Hashimoto-Pritzker Syndrome
Letterer-Siwe Disease
Pure Cutaneous Histiocytosis
Self-Healing Histiocytosis

General Discussion

Langerhans cell histiocytosis (LCH) is a rare spectrum of 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. The pathogenesis (medical cause) is not clearly understood and an ongoing debate continues regarding its cause as a reactive or neoplastic (cancer causing) process.

Most affected individuals have single or multiple bone lesions characterized by degenerative changes and loss of the calcium of bone (osteolysis). 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, or other areas. In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus. The exact cause of Langerhans cell histiocytosis is unknown.

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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Resources

Histiocytosis Association of America
332 North Broadway
Pitman, NJ 08071
Tel: (856)589-6606
Fax: (856)589-6614
Tel: (800)548-2758
Email: association@histio.org
Internet: http://www.histio.org

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

Diabetes Insipidus Foundation, Inc.
5203 New Prospect Court
Ellicott City, MD 21043
United States
Tel: 4104800880
Email: info@diabetesinsipidus.org
Internet: http://www.diabetesinsipidus.org

American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
USA
Tel: 2123158700
Fax: 2123158870
Tel: 8005864872
Internet: http://www.lungusa.org

NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov

Histiocytosis Association of Canada
29095 Okanagan Mission RPO
Kelowan, BC, V1W 1K2
Canada
Tel: 250-764-6104
Fax: 250-764-6104
Email: histio.canada@shaw.ca
Internet: http://www.histio.org/ca

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 8/17/2007
Copyright 1987, 1989, 1992, 2002, 2003, 2004, 2007 National Organization for Rare Disorders, Inc.

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