Mast Cell Activation Syndrome

@article{Frieri2015MastCA,
  title={Mast Cell Activation Syndrome},
  author={Marianne Frieri},
  journal={Clinical Reviews in Allergy \& Immunology},
  year={2015},
  volume={54},
  pages={353-365}
}
  • M. Frieri
  • Published 1 June 2018
  • Medicine
  • Clinical Reviews in Allergy & Immunology
Mast cell activation syndrome (MCAS) involves the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems, classified as primary, secondary, and idiopathic. Earlier criteria for MCAS diagnosis included episodic symptoms with mast cell mediators affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope, tachycardia, wheezing, conjunctival injection, pruritus, nasal stuffiness, decrease in… 
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45 Citations
Highly Influential Citations
2
Background Citations
17

45 Citations

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist
TLDR
Gastroenterologists who become aware of mast cell activation syndrome can dramatically improve the quality of life for their patients who previously have been labeled with a functional gastrointestinal disorder.
Mast Cell Activation Disorder and Postural Orthostatic Tachycardia Syndrome: A Clinical Association
TLDR
Laboratory findings suggesting MCA disorder were relatively common in patients diagnosed with POTS and who present with additional nonorthostatic gastrointestinal, cutaneous, and allergic symptoms, which favor MCA Disorder being considered in such cases.
Mast cell activation may explain many cases of chemical intolerance
TLDR
Close correspondence between QEESI scores and symptom patterns for MCAS and TILT patients supports xenobiotic-driven mast cell activation and mediator release (i.e., MCAS) as a plausible unifying biological mechanism for CI/TILT, with profound implications for medicine, public health, and regulatory toxicology.
The Mast Cell Activation Syndrome: A Mini Review
TLDR
This review will present a brief history of the mast cell and its subsequent association with multiple clinical disease states, as well as the criteria needed to delineate and classify these disease states into categories.
Mastocytosis and Mast Cell Activation Disorders: Clearing the Air
TLDR
The role of this review is to clarify the classification ofmast cell disorders, point to molecular aspects of mast cell signaling, elucidate underlying genetic defects, and provide approaches to targeted therapies that may benefit patients.
Drug desensitization in 17-year-old male with Mast Cell Activation Syndrome, pneumonia, and antibiotic hypersensitivities.
TLDR
This case demonstrates that desensitization protocols can be used in MCAS patients with noted hypersensitivities, and presented with pneumonia requiring treatment with ceftriaxone and azithromycin, both provided via desensItization protocols.
Mastocytosis: from a Molecular Point of View
TLDR
Mast cells (MCs) are physiologically activated by binding of stem cell factor (SCF) to the extracellular domains of the Kit receptor and lead to constitutive KIT activation even in the absence of its ligand SCF.
A Primer on Mast Cell Activation Disease for the Nurse Practitioner
The pathophysiology of anaphylaxis.
The role of mast cells in pediatric gastrointestinal disease
TLDR
The current understanding of the role that mast cells play in the development of pediatric gastrointestinal disorders, including eosinophilic esophagitis, functional dyspepsia, irritable bowel syndrome, celiac disease, inflammatory bowel disease, histologically negative appendicitis, Hirschsprung’s disease, intestinal neuronal dysplasia, and food protein-induced enterocolitis syndrome is summarized.
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References

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TLDR
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 are included.
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TLDR
The use of the term ‘spectrum’ is proposed to highlight the main symptoms, useful diagnostic tests and treatment approaches associated with mast cell activation disorders and propose the use of c-kit mutation and clonal MC activation disorder.
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TLDR
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