# OnCo record lch-multisystem (cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)". Whole corpus: https://onco.cc/api/v1/onco.nt
@prefix schema: <https://schema.org/> .
@prefix onco: <https://onco.cc/ns#> .
@prefix xsd: <http://www.w3.org/2001/XMLSchema#> .

<https://onco.cc/cancers/lch-multisystem/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "lch-multisystem" ;
  schema:name "Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement)"@en ;
  schema:alternateName "Multisystem LCH"@en, "MS-LCH"@en, "Risk-organ-positive LCH"@en, "Letterer-Siwe disease (obsolete)"@en, "Hand-Schüller-Christian disease (obsolete)"@en ;
  schema:description "Multisystem Langerhans cell histiocytosis is the severe form of this rare histiocytosis, in which the abnormal cells involve several organs at once, most dangerously the liver, spleen and bone marrow of infants. It is treated with a year of vinblastine and prednisone, with stronger drugs or BRAF-targeted tablets for children who do not respond quickly; survival is now high but late effects remain."@en ;
  schema:url <https://onco.cc/cancers/lch-multisystem/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis> ;
  schema:citation <https://doi.org/10.1182/blood-2012-09-455774>, <https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq>, <https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis> ;
  onco:tag "subtype-page", "paediatric" ;
  onco:related <https://onco.cc/cancers/lch-single-system/>, <https://onco.cc/cancers/langerhans-cell-histiocytosis/>, <https://onco.cc/cancers/erdheim-chester-disease/> ;
  onco:technologies <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/allogeneic-hsct/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/ultrasound/>, <https://onco.cc/technologies/liquid-biopsy/>, <https://onco.cc/technologies/survivorship-care-plan/> ;
  onco:targets <https://onco.cc/targets/braf/> ;
  onco:drugs <https://onco.cc/drugs/vinblastine/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/drugs/cobimetinib/> ;
  onco:terms <https://onco.cc/terms/late-effects/>, <https://onco.cc/terms/braf-v600-mutation/>, <https://onco.cc/terms/cytopenias/> ;
  onco:trials <https://onco.cc/trials/lch-iii/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-lch-iii-therapy-prolongation-multisystem-lch-blood-2013/>, <https://onco.cc/key-papers/paper-donadieu-vemurafenib-refractory-multisystem-lch-jco-2019/>, <https://onco.cc/key-papers/paper-badalian-very-braf-mutations-lch-blood-2010/> ;
  onco:pipeline <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/trials/lch-iii/>, <https://onco.cc/drugs/cobimetinib/> ;
  onco:standardOfCare <https://onco.cc/terms/braf-v600-mutation/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/ultrasound/>, <https://onco.cc/technologies/histopathology-ihc/>, <https://onco.cc/drugs/vinblastine/>, <https://onco.cc/trials/lch-iii/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/technologies/allogeneic-hsct/>, <https://onco.cc/drugs/vinblastine/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/terms/late-effects/>, <https://onco.cc/technologies/survivorship-care-plan/>, <https://onco.cc/technologies/mri/> .