Orphanet: Mastocytose systémique indolente

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Indolent systemic mastocytosis

Disease definition

A rare, usually benign, chronic, form of systemic mastocytosis (SM) characterized by an abnormal accumulation of neoplastic mast cells (MCs) mainly in the bone marrow (BM) but also in other organs or tissues such as preferably the skin.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: D47.0
  • OMIM: -
  • UMLS: C0272203
  • MeSH: -
  • GARD: -
  • MedDRA: 10056452

Detailed information


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