Langerhans cell histiocytosis

URI: https://gptkb.org/entity/Langerhans_cell_histiocytosis
GPTKB entity
Statements (48)
Predicate Object
gptkbp:instanceOf gptkb:disease
gptkb:histiocytic_disorder
rare disease
gptkbp:affects adults
children
gptkbp:affectsOrgan gptkb:bone
gptkb:skin
gptkb:pituitary_gland
lungs
liver
lymph nodes
spleen
gptkbp:associatedWith gptkb:BRAF_V600E_mutation
MAP2K1 mutation
gptkbp:category autoimmune disease
immune system disease
neoplastic disease
gptkbp:causedBy proliferation of Langerhans cells
gptkbp:firstDescribed gptkb:Paul_Langerhans
1868
gptkbp:hasCellTypeInvolved gptkb:Langerhans_cell
gptkbp:hasDiagnosticMethod imaging
blood test
biopsy
gptkbp:hasEpidemiology more common in children under 15
gptkbp:hasOrphanetID ORPHA:535
gptkbp:hasRelatedDisorder gptkb:Erdheim-Chester_disease
gptkb:Rosai-Dorfman_disease
https://www.w3.org/2000/01/rdf-schema#label Langerhans cell histiocytosis
gptkbp:ICD-10_code C96.6
gptkbp:incidence 1 in 200,000 children per year
gptkbp:MeSH_ID D006391
gptkbp:OMIM 604856
gptkbp:prognosis variable
gptkbp:symptom gptkb:diabetes_insipidus
fever
weight loss
bone pain
skin rash
enlarged lymph nodes
gptkbp:synonym gptkb:Histiocytosis_X
gptkb:LCH
gptkbp:treatment surgery
radiation therapy
chemotherapy
steroids
gptkbp:bfsParent gptkb:BRAF_gene
gptkbp:bfsLayer 5