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Mastocytosis is a heterogeneous clonal disorder with varying clinical features and prognosis, characterized by the proliferation and accumulation of mast cells (mast cells) in different tissues, mainly in the skin and bone marrow. Cutaneous mastocytosis (CM) is the most common form in children, while adults are almost always affected by a systemic disease (SM) characterized by involvement of at least one or more extracutaneous (EC) organs, with or without skin lesions. Most SM patients have a somatic "autoactivating" point mutation at codon 816 of the KIT receptor gene.