# OnCo record advanced-systemic-mastocytosis (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/advanced-systemic-mastocytosis/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "advanced-systemic-mastocytosis" ;
  schema:name "Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia)"@en ;
  schema:alternateName "AdvSM"@en, "Aggressive systemic mastocytosis"@en, "ASM"@en, "SM-AHN"@en, "Mast cell leukaemia"@en, "MCL"@en ;
  schema:description "Advanced systemic mastocytosis is the dangerous form of this rare blood cancer, in which KIT-mutant mast cells damage the marrow, liver, gut or bones, grow alongside a second blood cancer such as chronic myelomonocytic leukaemia, or flood the blood as mast cell leukaemia. The KIT-blocking tablets midostaurin and avapritinib have replaced older chemotherapy, and fit patients may have a transplant."@en ;
  schema:url <https://onco.cc/cancers/advanced-systemic-mastocytosis/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Mastocytosis> ;
  schema:citation <https://doi.org/10.1056/NEJMoa1513098>, <https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1478>, <https://en.wikipedia.org/wiki/Mastocytosis> ;
  onco:tag "subtype-page", "haematologic" ;
  onco:related <https://onco.cc/cancers/indolent-systemic-mastocytosis/>, <https://onco.cc/cancers/systemic-mastocytosis/>, <https://onco.cc/cancers/cmml/>, <https://onco.cc/cancers/aml-secondary/> ;
  onco:technologies <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/allogeneic-hsct/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/technologies/histopathology-ihc/>, <https://onco.cc/technologies/liquid-biopsy/> ;
  onco:targets <https://onco.cc/targets/kit/>, <https://onco.cc/targets/flt3/> ;
  onco:drugs <https://onco.cc/drugs/avapritinib/>, <https://onco.cc/drugs/midostaurin/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/interferon-alfa/>, <https://onco.cc/drugs/azacitidine/>, <https://onco.cc/drugs/bezuclastinib/> ;
  onco:terms <https://onco.cc/terms/cytopenias/>, <https://onco.cc/terms/molecular-response/>, <https://onco.cc/terms/driver-mutation/> ;
  onco:trials <https://onco.cc/trials/nct04996875/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-who-2022-myeloid-khoury-leukemia-2022/>, <https://onco.cc/key-papers/paper-gotlib-midostaurin-advanced-systemic-mastocytosis-nejm-2016/>, <https://onco.cc/key-papers/paper-pathfinder-avapritinib-advanced-systemic-mastocytosis-nat-med-2021/>, <https://onco.cc/key-papers/paper-explorer-avapritinib-advanced-systemic-mastocytosis-nat-med-2021/> ;
  onco:pipeline <https://onco.cc/drugs/avapritinib/>, <https://onco.cc/drugs/bezuclastinib/>, <https://onco.cc/trials/nct04996875/>, <https://onco.cc/technologies/allogeneic-hsct/> ;
  onco:standardOfCare <https://onco.cc/technologies/histopathology-ihc/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/targets/kit/>, <https://onco.cc/terms/cytopenias/>, <https://onco.cc/drugs/avapritinib/>, <https://onco.cc/drugs/midostaurin/>, <https://onco.cc/targets/kit/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/drugs/azacitidine/>, <https://onco.cc/drugs/midostaurin/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/interferon-alfa/>, <https://onco.cc/drugs/bezuclastinib/>, <https://onco.cc/trials/nct04996875/>, <https://onco.cc/technologies/allogeneic-hsct/> .