Langerhans-Cell Histiocytosis (LCH) malady
Rare diseases, Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases, Immune diseases categories
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8Disease Ontology, 21Genetics Home Reference, 42NIH Rare Diseases, 65Wikipedia, 46OMIM, 32MalaCards
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NIH Rare Diseases:42 Langerhans cell histiocytosis (lch) is a disorder that primarily affects children, but is also found in adults of all ages. people with lch produce too many langerhans cells orÂ histiocytes, a form of white blood cell found inÂ healthy people that is supposedÂ to protect the body from infection. in people with lch,Â these cells multiply excessively and build up in certain areas of the body, causing tumors calledÂ granulomas to form. the symptoms vary among affected individuals, and the cause of lch is unknown.Â in most cases, this condition is not life-threatening. some people do experience life-long problems associated with lch. last updated: 10/18/2013
MalaCards based summary: Langerhans-Cell Histiocytosis, also known as letterer-siwe disease, is related to histiocytosis and sarcoma, and has symptoms including anomalies of tongue, gingiva and oral mucosa, anomalies of teeth and dentition and premature eruption of teeth/natal teeth. An important gene associated with Langerhans-Cell Histiocytosis is CD207 (CD207 molecule, langerin). The drug thalidomide has been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and spleen.
Disease Ontology:8 A histiocytosis that is characterized by clonal proliferation of langerhans cells.
Genetics Home Reference:21 Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. However, Langerhans cell histiocytosis is not generally considered to be a form of cancer.
Wikipedia:65 Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans... more...
Descriptions from OMIM:46 246400,604856
Langerhans-Cell Histiocytosis, Aliases & Descriptions:
Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases, Cancer diseases
Anatomical: Respiratory diseases, Bone diseases, Blood diseases, Immune diseases
Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue
Rare respiratory diseases
Rare systemic and rhumatological diseases
Rare haematological diseases
Characteristics (Orphanet epidemiological data):48
Prevalence: 1-9/1000000; Age of onset: Neonatal/infancy
Symptoms by clinical synopsis from OMIM:246400
Clinical features from OMIM:246400,604856
Symptoms:48 (show all 31)
HPO human phenotypes related to Langerhans-Cell Histiocytosis:(show all 46)
MalaCards organs/tissues related to Langerhans-Cell Histiocytosis:32
Lymph node, Bone, Spleen, Skin, Lung, Bone marrow, Liver, Thyroid, Pituitary, Myeloid, T cells, Tongue, Colon, Testes, Thymus, Spinal cord, Breast, B cells, Heart, Tonsil, Monocytes
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Search GEO for disease gene expression data for Langerhans-Cell Histiocytosis.
26ICD10 via Orphanet
35MESH via Orphanet
47OMIM via Orphanet
58SNOMED-CT via Orphanet
63UMLS via Orphanet