Disordered mast cell activation occurs when mast cells are pathologically overproduced or if their activation is out of proportion. Because of the relative prevalence of the disease, the routine differential diagnosis must be carried out in patients with chronic multisystem polymorbidity. This article briefly explains the diagnostic approach for mast cell activation syndrome.

The term mast cell activation syndrome is a collection of disorders characterized by the accumulation of pathological mast cells in potentially any or all organs and tissues and/or aberrant release of variable subsets of mast cell mediators. Mast cell leukemia is a rare variant of this disease. Proposed criteria for mast cell activation syndrome: Episodic multisystem symptoms consistent with mast cell activation Appropriate response to medications targeting mast cell activation Documented increase in validated…