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Table 1 Classification of mast cell activation disease (modified from [24]).

From: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

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)