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Mast Cell Disorders and Anaphylaxis

  • Sharzad Alagheband
  • Catherine Cranford
  • Patricia StewartEmail author
Living reference work entry

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.

Keywords

Mastocytosis Mast cell Diagnostic criteria Tryptase KIT D816V 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Sharzad Alagheband
    • 1
  • Catherine Cranford
    • 1
  • Patricia Stewart
    • 1
    Email author
  1. 1.Department of Medicine, Division of Clinical Immunology and AllergyUniversity of Mississippi Medical CenterJacksonUSA

Section editors and affiliations

  • Dennis K. Ledford
    • 1
  1. 1.Division of Allergy & Immunology, Dept. of Internal MedicineUniversity of South Florida Morsani College of Medicine, James A Haley Veterans' Hospital, Asthma and Immunology Associates of Tampa BayTampaUSA

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