Systemic mastocytosis with associated hematologic neoplasm
ORPHA:98849· ICD-10 D47.0
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.
- Prevalence
- 1-9 / 100 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly