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 2015
  • 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… Expand
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Drug desensitization in 17-year-old male with Mast Cell Activation Syndrome, pneumonia, and antibiotic hypersensitivities.
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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. Expand
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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. Expand
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A primer on mast cell diseases, including triggers, signs and symptoms, diagnosis, treatment, and resources for patients and providers is provided. Expand
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  • Medicine
  • Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
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The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators, based on clonal mast cell proliferation. Expand
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The suppressive effect of dabrafenib, a therapeutic agent for metastatic melanoma, in IgE-mediated allergic inflammation.
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Dabrafenib is a therapeutic drug candidate that controls IgE-mediated allergic inflammatory diseases through suppression of mast cell activity and is suggested to be a specific target for treating immunoglobulin (Ig)E- mediated allergic reactions. Expand
Das Ehlers-Danlos-Syndrom mit Schwerpunkt auf dem hypermobilen Typ und dessen Begleiterkrankungen - eine Literaturübersicht
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References

SHOWING 1-10 OF 79 REFERENCES
Mast Cell Activation Syndrome: A Review
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. Expand
Spectrum of mast cell activation disorders
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. Expand
Prevention of anaphylaxis related to mast cell activation syndrome with omalizumab.
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  • Medicine
  • Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology
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TLDR
A case of the successful use of omalizumab (monoclonal antibody to IgE) in the treatment of a pediatric patient who met the proposed diagnostic criteria for MCAS is presented. Expand
Utility of hydroxyurea in mast cell activation syndrome
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  • Medicine
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TLDR
Hydroxyurea may be useful in treating mediator symptoms in MCAS, but further study is needed to define optimal dosing strategies and patient subpopulations most likely to benefit. Expand
Hyperadrenergic Postural Tachycardia Syndrome in Mast Cell Activation Disorders
TLDR
Patients with MCA should be considered in patients with POTS presenting with flushing, but &bgr;-blockers should be used with great caution, if at all, and treatment directed against mast cell mediators may be required. Expand
Mast cell activation syndrome: a newly recognized disorder with systemic clinical manifestations.
TLDR
MC activation syndrome might be the underlying cause of unexplained symptoms when several organ systems are involved, such as the gastrointestinal tract and the skin, and response to anti-MC mediator medications is often excellent. Expand
Mast cell activation syndrome masquerading as agranulocytosis.
TLDR
Mast cell activation syndrome should be considered in otherwise idiopathic agranulocytosis presenting with comorbidities best explained by MC mediator release, and different presentations of different MCAS patients reflect elaboration of different mediators likely consequent to different Kit mutations. Expand
Expanding spectrum of mast cell activation disorders: monoclonal and idiopathic mast cell activation syndromes.
TLDR
MMAS and MCAS are 2 newly described, rare syndromes of mast cell activation that affect predominantly women in whom no mast cell abnormality or external triggers account for their episodes of mastcell activation. Expand
Evaluation of Mast Cell Activation Syndromes: Impact of Pathology and Immunohistology
TLDR
When the activating point mutant KIT D816V is detectable but criteria for diagnosis of mastocytosis are not completely met, a so-called (mono)clonal MCAS as a subvariant of primary MCAS should be diagnosed. Expand
Mast cell activation syndrome: Proposed diagnostic criteria.
The term mast cell activation syndrome (MCAS) is finding increasing use as a diagnosis for subjects who present with signs and symptoms involving the dermis, gastrointestinal track, and… Expand
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