Abstract
Mast cells may be activated secondary to different physiological and pathological conditions. Mast cell activation disorders are classified as primary – associated with a monoclonal mast cell population – or secondary (with a normal number of so-called hyperreactive mast cells), or even idiopathic. Mast Cell Activation Syndrome (MCAS) is characterized by an inappropriate and excessive release of mast cells mediators, leading to clinical symptoms that may affect several body systems, or even cause repeated anaphylaxis. We wanted to provide clear answers to the following questions to help the overall management of patients presenting with MACS: what is the current definition and classification of MCAS? Which diagnostic, clinical and laboratory criteria should be used? What are the most comment therapeutic strategies? The aim of the present work is to present current knowledge on MCAS, to aid clinicians in the diagnostic and therapeutic process of these conditions.