Orphanet: Systemic mastocytosis with an associated clonal hematologic non mast cell lineage disease

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Systemic mastocytosis with associated hematologic neoplasm

Disease definition

An advanced form of systemic mastocytosis (SM) characterized by the abnormal accumulation of neoplastic mast cells (MCs) in one or more extracutaneous organs, mainly the bone marrow, associated with another hematologic neoplasm of non MC nature.


Classification level: Disorder
  • Synonym(s):
    • SM-AHN
    • SM-AHNMD
    • Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: Elderly, Adult
  • ICD-10: C96.2
  • OMIM: -
  • UMLS: C1301365
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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