Table 1

Classification of mast cell activation disease (modified from [2-4]).

Mast cell activation disease (MCAD)


Mast cell activation syndrome (MCAS)


Systemic mastocytosis (SM) defined by the WHO criteria

• Indolent systemic mastocytosis

• Isolated bone marrow mastocytosis

• Smoldering systemic mastocytosis

• Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease

• Aggressive systemic mastocytosis


Mast cell leukemia (MCL)


Molderings et al. Journal of Hematology & Oncology 2011 4:10   doi:10.1186/1756-8722-4-10

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