Mast Cell Activation: When the Whole Is Greater than the Sum of Its Parts

Dilawar Khokhar; Cem Akin

doi:10.1016/j.mcna.2019.09.002

Mast Cell Activation: When the Whole Is Greater than the Sum of Its Parts

Med Clin North Am. 2020 Jan;104(1):177-187. doi: 10.1016/j.mcna.2019.09.002.

Authors

Dilawar Khokhar¹, Cem Akin²

Affiliations

¹ Department of Internal Medicine, Division of Allergy and Clinical Immunology, University of Michigan, 24 Frank Lloyd Wright Drive, Lobby H Suite H-2100, Ann Arbor, MI 48106, USA.
² Department of Internal Medicine, Division of Allergy and Clinical Immunology, University of Michigan, 24 Frank Lloyd Wright Drive, Lobby H Suite H-2100, Ann Arbor, MI 48106, USA. Electronic address: cemakin@med.umich.edu.

PMID: 31757235
DOI: 10.1016/j.mcna.2019.09.002

Abstract

Mast cell activation syndrome (MCAS) is a heterogeneous and rare disorder with episodic and severe activation of mast cells. Because symptoms of mast cell activation are nonspecific, it is important to base the diagnosis on best available clinical and scientific evidence, and not make it one of exclusion. MCAS, much like the mast cell itself, as a whole is greater than the sum of its proposed diagnostic criteria. When each component is considered in isolation, criteria can seem nonspecific, and thus, a broad constellation of symptoms can be attributed to MCAS when they may be due to other disease processes.

Keywords: Idiopathic anaphylaxis; MCAS; Mast cell activation; Mast cell disorder; Mast cell mediators; Mastocytosis; Tryptase.

Publication types

Review

MeSH terms

Diagnosis, Differential
Humans
Mastocytosis / diagnosis*
Symptom Assessment*