Indolent systemic mastocytosis
ORPHA:98848· ICD-10 D47.0
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.
- Prevalence
- 1-5 / 10 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly