Pediatric Expression of Mast Cell Activation Disorders

Immunol Allergy Clin North Am. 2018 Aug;38(3):365-377. doi: 10.1016/j.iac.2018.04.009. Epub 2018 May 21.

Abstract

Mast cell activation disorders is a term proposed to cover diseases and conditions related to activation of mast cells and effects of mast cell mediators. In its broadest sense, the term encompasses a wide range of diseases from allergic asthma to rhinoconjunctivitis, urticaria, food allergy, anaphylaxis, mastocytosis, and other conditions where MC activation is contributing to the pathogenesis. This article focuses on clinical presentations, challenges, and controversies in pediatric mastocytosis and gives an overview of current knowledge and areas in need of further research.

Keywords: Anaphylaxis; KIT mutations; Mast cell activation; Mast cell activation disorders; Mastocytosis; Tryptase; Urticaria pigmentosa.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Adult
  • Anaphylaxis / genetics
  • Anaphylaxis / immunology*
  • Cell Degranulation*
  • Child
  • Humans
  • Mast Cells / physiology*
  • Mastocytosis / genetics
  • Mastocytosis / immunology*
  • Proto-Oncogene Proteins c-kit / genetics*
  • Tryptases / metabolism
  • Urticaria / genetics
  • Urticaria / immunology*

Substances

  • KIT protein, human
  • Proto-Oncogene Proteins c-kit
  • Tryptases