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Mast cells (MCs) are important component of the immune system. Their physiological function is related to multiple areas of human physiology, thus symptoms of their increased activation vary greatly from severe allergic reactions such as anaphylaxis, to chronic symptoms like depression or osteoporosis. Studies on mastocytosis revealed a subgroup of patients presenting symptoms of increased degranulation of MCs, defined as Mast Cell Activation Syndrome (MCAS). Among them patients with primary MCAS presenting clonal abnormal MCs, who do not