Current Treatment Options in Allergy

, Volume 3, Issue 4, pp 384–400 | Cite as

Mast Cell Activation Syndromes

  • Rafael Bonamichi-Santos
  • Mariana Castells
Urticaria and Atopic Dermatitis (M Ferrer-Puga, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Urticaria and Atopic Dermatitis

Opinion statement

Mast cell activation syndromes(MCAS) are disorders associated with mast cell activation (MCA), and include Primary MCAS, Secondary MCAS and Idiopathic MCAS. MCAS are characterized by clinical symptoms of MCA in cutaneous, gastrointestinal, respiratory, cardiovascular, musculoskeletal and neurological organs. Mast cell (MC) mediators such as tryptase in serum, and/or histamine or prostaglandin urinary metabolites are typically elevated at base line or transiently during episodes of MCA, and there is a total or partial response to mast cell mediators controller medications. In primary MCAS an activating point mutation at codon 816 of KIT on MC is present in most cases of Systemic Mastocytosis (SM) and Monoclonal Mast Cell Activation Syndrome (MMCAS) and is absent in secondary and Idiopathic MCAS. MCAS might be the underlying cause of unexplained symptoms when several organ systems are involved, such as the gastrointestinal tract and the skin. It is especially important to be able to recognize the constellation of clinical features because response to anti-MC mediator medications is often excellent. This update on mast cell disorders (MCD) provides an insight into the classification, clinical presentations, diagnosis, treatment and management. We describe associated conditions, such as Hymenoptera Reactions, Familial Tryptasemia, Postural Orthostatic Tachycardia Syndrome and Ehlers-Danlos Syndrome.


Mast cell Mast cell activation Tryptase Histamine Allergy Anaphylaxis Antihistamines 



Cutaneous mastocytosis


Ehlers-Danlos syndrome


Hymenoptera venom


Idiopathic mast cell activation syndrome


Mast cell


Mast cell activation


Mast cell activation syndromes


Mast Cell T


Mast cell TC


Monoclonal mast cell activation syndrome


Postural tachycardia syndrome


Systemic mastocytosis


Venom immunotherapy


Compliance with Ethical Standards

Conflict of Interest

Dr. Rafael Bonamichi-Santos declares that he has no conflict of interest.

Dr. Mariana Castells declares that she has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer International Publishing AG 2016

Authors and Affiliations

  1. 1.Division of Rheumatology, Immunology and Allergy; Department of MedicineBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Clinical Immunology and Allergy DivisionUniversity of São PauloSão PauloBrazil

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