Penissi Alicia B
Mast cell activation disease (MCAD) may be a term pertaining to a heterogeneous group of disorders characterized by aberrant release of variable subsets of mastocyte (MC) mediators along side accumulation of either morphologically altered and immunohistochemically identifiable mutated MCs thanks to MC proliferation (systemic masto cytosis [SM] and MC leukemia [MCL]) or morphologically ordinary MCs thanks to decreased apoptosis (MC activation syndrome [MCAS] and well-differentiated SM). Clinical signs and symptoms in MCAD vary counting on disease subtype and result from excessive mediator release by MCs and, in aggressive forms, from organ failure associated with MC infiltration. In most cases, treatment of MCAD is directed primarily at controlling the symptoms related to MC mediator release. In advanced forms, like aggressive SM and MCL, agents targeting MC proliferation like kinase inhibitors could also be provided. Targeted therapies aimed toward blocking mutant protein variants and/or downstream signaling pathways are currently being developed. Other targets, like specific surface antigens expressed on neoplastic MCs, could be considered for the event of future therapies. Since clinicians are often underprepared to guage, diagnose, and effectively treat this clinically heterogeneous disease, we seek to familiarize clinicians with MCAD and review current and future treatment approaches.
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