Aggressive systemic mastocytosis

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.

Disease data

Classification

Disease

ORPHA code

98850

OMIM code

ICD10 code

C96.2

ICD11 code

*Soruce