Langerhans-Cell Histiocytosis malady
Categories: Genetic diseases, Rare diseases, Respiratory diseases, Bone diseases, Blood diseases, Cancer diseases, Immune diseases
Aliases & Descriptions for Langerhans-Cell Histiocytosis:
Orphanet epidemiological data:51
Prevalence: 1-9/1000000 (Europe); Age of onset: Infancy,Neonatal
Inheritance: autosomal recessive inheritance
Global: Genetic diseases, Rare diseases, Cancer diseases
Anatomical: Respiratory diseases, Bone diseases, Blood diseases, Immune diseases
ICD10: 28 27
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
NIH Rare Diseases:45 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 or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. in people with lch, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. the symptoms vary among affected individuals, and the cause of lch is unknown. 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 eosinophilic granuloma, is related to skeletal tuberculosis and non-langerhans-cell histiocytosis, and has symptoms including bone pain, recurrent fractures and osteolysis. An important gene associated with Langerhans-Cell Histiocytosis is CD1A (CD1a Molecule), and among its related pathways are Thyroid cancer and Trk receptor signaling mediated by the MAPK pathway. The drug thalidomide has been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and lung, and related mouse phenotypes are integument and craniofacial.
Disease Ontology:10 A histiocytosis that is characterized by clonal proliferation of Langerhans cells.
Genetics Home Reference:23 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:68 Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans... more...
Symptoms:51 (show all 31)
HPO human phenotypes related to Langerhans-Cell Histiocytosis:(show all 43)
UMLS symptoms related to Langerhans-Cell Histiocytosis:hepatomegaly
Drugs for Langerhans-Cell Histiocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):(show top 50) (show all 104)
Interventional clinical trials:(show all 34)
Search NIH Clinical Center for Langerhans-Cell Histiocytosis
Inferred drug relations via UMLS65/NDF-RT43:
MalaCards organs/tissues related to Langerhans-Cell Histiocytosis:33
Lymph node, Bone, Lung, Spleen, Skin, Bone marrow, Liver
MGI Mouse Phenotypes related to Langerhans-Cell Histiocytosis:38
Articles related to Langerhans-Cell Histiocytosis:(show top 50) (show all 751)
Search GEO for disease gene expression data for Langerhans-Cell Histiocytosis.
Pathways related to Langerhans-Cell Histiocytosis according to GeneCards Suite gene sharing:(show all 11)
Biological processes related to Langerhans-Cell Histiocytosis according to GeneCards Suite gene sharing:(show all 12)
28ICD10 via Orphanet
37MESH via Orphanet
50OMIM via Orphanet
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
66UMLS via Orphanet