Pharmacological treatment options for mast cell activation disease

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