Агресивний системний мастоцитоз
ORPHA:98850· ICD-10 C96.2· Aggressive systemic mastocytosis
Визначення(English summary)
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.
- Поширеність
- 1-9 / 1 000 000
- Успадкування
- Not applicable
- Вік початку
- Adult, Antenatal, Elderly, Neonatal