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Mast Cell Disorders and Anaphylaxis

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Abstract

Mast cells arise from pluripotent stem cells. From the original identification of mast cells in the late 1800s, our understanding of these cells’ normal function and role in pathologic disease has expanded greatly; and the understanding of mastocytosis has led to advances in classification and treatment of these diseases. The term mastocytosis describes a group of disorders characterized by abnormal proliferation of mast cells. Mast cell numbers are increased and pathologically infiltrate various organ systems, resulting in a spectrum of disorders from cutaneous mastocytosis (more common in children) to multiple subvariants of systemic mastocytosis, mast cell leukemia, and mastocytomas. Diagnostic criteria have been modified recently to aid in classifying the type of disease, which allows for better determination of both the prognosis and treatment. While treatment is largely symptomatic, with important focus on the management of anaphylaxis, several promising therapeutic targets and agents have recently been identified which may lead to improved survival in more advanced subtypes of disease. These therapies include several tyrosine kinase inhibitors which combat the activating KIT mutations present in most patients with aggressive mastocytosis.

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Alagheband, S., Cranford, C., Stewart, P. (2019). Mast Cell Disorders and Anaphylaxis. In: Craig, T., Ledford, D. (eds) Allergy and Asthma. Springer, Cham. https://doi.org/10.1007/978-3-319-58726-4_30-1

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