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Current Allergy and Asthma Reports

, Volume 13, Issue 1, pp 27–32 | Cite as

Mast Cell Activation Syndrome: A Review

  • Marianne FrieriEmail author
  • Reenal Patel
  • Jocelyn Celestin
BASIC AND APPLIED SCIENCE (M FRIERI, SECTION EDITOR)

Abstract

Mast cell activation syndrome (MCAS) is a condition with signs and symptoms involving the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems. It can be classified into primary, secondary, and idiopathic. Earlier proposed criteria for the diagnosis of MCAS included episodic symptoms consistent with mast cell mediator release affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope or near syncope, tachycardia, wheezing, conjunctival injection, pruritus, and nasal stuffiness. Other criteria included a decrease in the frequency, severity, or resolution of symptoms with anti-mediator therapy including H1 and H2histamine receptor antagonists, anti-leukotrienes, or mast cell stabilizers. Laboratory data that support the diagnosis include an increase of a validated urinary or serum marker of mast cell activation (MCA), namely the documentation of an increase of the marker above the patient’s baseline value during symptomatic periods on more than two occasions, or baseline serum tryptase levels that are persistently above 15 ng/ml, or documentation of an increase of the tryptase level above baseline value on one occasion. Less specific assays are 24-h urine histamine metabolites, PGD2 (Prostaglandin D2) or its metabolite, 11-β-prostaglandin F2 alpha. A recent global definition, criteria, and classification include typical clinical symptoms, a substantial transient increase in serum total tryptase level or an increase in other mast cell derived mediators, such as histamine or PGD2 or their urinary metabolites, and a response of clinical symptoms to agents that attenuate the production or activities of mast cell mediators.

Keywords

Mast cell activation Mast cell activation syndrome Mastocytosis Idiopathic anaphylaxis Clonal MCAD Nonclonal MCAD Classification Treatment 

Notes

Disclosure

No potential conflicts of interest relevant to this article were reported.

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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Marianne Frieri
    • 1
    Email author
  • Reenal Patel
    • 2
  • Jocelyn Celestin
    • 3
  1. 1.Department of Medicine and PediatricsNassau University Medical CenterEast MeadowUSA
  2. 2.Division of Allergy Immunology, Department of MedicineNassau University Medical CenterEast MeadowUSA
  3. 3.Division of Allergy and ImmunologyAlbany Medical CollegeAlbanyUSA

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