Aggressive systemic mastocytosis
ORPHA:98850· ICD-10 C96.2
Definition
A rare, aggressive form of advanced systemic mastocytosis (advSM) characterized by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukemia or another hematologic neoplasm.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Antenatal, Elderly, Neonatal