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Systemic mastocytosis

Disease definition

Systemic mastocytosis (SM) comprises a heterogeneous group of rare acquired and chronic hematological malignancies that are related to an abnormal proliferation of mast cells in tissue, including bone marrow, with or without skin involvement.

ORPHA:2467

  • Synonym(s):
    • Mast cell disease
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: C96.2
  • OMIM: -
  • UMLS: C0221013
  • MeSH: D034721
  • GARD: 8616
  • MedDRA: 10042949

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