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Abstract
Patients who present with typical features of mast cell activation with laboratory
confirmation and without evidence of a clonal mast cell disorder or other medical
condition should be initiated on medical treatment to block mast cells and their mediators.
If a major response is achieved, a diagnosis of nonclonal mast cell activation syndrome
(NC-MCAS) is likely and treatment should be optimized, including management of any
associated conditions. In this review, the latest evidence with regard to the diagnosis
and treatment of NC-MCAS is presented.