Abstract
Mast cell activation syndromes (MCAS) are divided into 3 groups based on mast cell (MC) clonality and activation triggers. Primary MCAS diseases are due to clonal expansion and MC proliferation that are typically associated with mutations in KIT as found in mastocytosis and monoclonal mast cell activation syndrome. Secondary MC disorders are in response to allergic or other inflammatory diseases causing MC activation. Idiopathic MCAS is diagnosed when no clonal MC disease and other etiologic mechanisms have been identified.
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