Aggressive systemic mastocytosis

Aggressive systemic mastocytosis is a rare, severe subtype of advanced systemic mastocytosis characterized by the uncontrolled clonal proliferation and infiltration of neoplastic mast cells into extracutaneous organ systems

Aggressive systemic mastocytosis is a rare, severe subtype of advanced systemic mastocytosis characterized by the uncontrolled clonal proliferation and infiltration of neoplastic mast cells into extracutaneous organ systems Predominantly affecting adults, the disorder is driven in most cases by activating mutations in the *KIT* gene (commonly *KIT* D816V) It is pathophysiologically defined by mast-cell-mediated tissue infiltration that causes clinically evident, non-hematologic organ damage—term...

Aggressive systemic mastocytosis is a rare, severe subtype of advanced systemic mastocytosis characterized by the uncontrolled clonal proliferation and infiltration of neoplastic mast cells into extracutaneous organ systems

Predominantly affecting adults, the disorder is driven in most cases by activating mutations in the *KIT* gene (commonly *KIT* D816V)

It is pathophysiologically defined by mast-cell-mediated tissue infiltration that causes clinically evident, non-hematologic organ damage—termed C-findings—such as cytopenias from bone marrow failure, hepatomegaly with liver dysfunction or ascites, portal hypertension, splenomegaly with hypersplenism, malabsorption, and osteolytic bone lesions with skeletal fractures

Unlike other advanced mast cell disorders, it manifests severe, progressive organopathy without fulfilling the diagnostic criteria for mast cell leukemia or an associated hematologic neoplasm