Mast Cell Activation Syndrome

A clinically and genetically heterogeneous group of mast cell disorders in which there is aberrant release of mast cell mediators with little to no accompanying proliferation of MAST CELLS.

Also Known As:

Mast Cell Activation Syndromes; Non Clonal Mast Cell Activation Disorder; Non Clonal Mast Cell Activation Syndrome; nc MCAS; Idiopathic Mast Cell Activation Disorder; Non-Clonal Mast Cell Activation Disorder; Non-Clonal Mast Cell Activation Syndrome; Nonclonal Mast Cell Activation Syndrome; nc-MCAS

Networked: 73 relevant articles (5 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Immune System Diseases: 4321
- Mast Cell Activation Disorders: 110
  - Mast Cell Activation Syndrome: 73
    - Hereditary alpha-tryptasemia syndrome: 28

Related Diseases

1.	Cutaneous Mastocytosis
2.	Pruritus (Itching)
3.	Asthma (Bronchial Asthma)
4.	Anaphylaxis (Anaphylactic Shock)
5.	Mastocytosis

Experts

1.	Afrin, Lawrence B: 8 articles (05/2022 - 07/2011)
2.	Molderings, Gerhard J: 6 articles (05/2022 - 12/2010)
3.	Bonadonna, Patrizia: 5 articles (01/2022 - 01/2019)
4.	Arock, Michel: 4 articles (01/2022 - 01/2019)
5.	Brockow, Knut: 4 articles (01/2022 - 01/2013)
6.	Valent, Peter: 4 articles (01/2022 - 01/2019)
7.	Akin, Cem: 4 articles (01/2021 - 05/2015)
8.	Hertfelder, Hans-Jörg: 3 articles (05/2022 - 01/2017)
9.	Homann, Jürgen: 3 articles (05/2022 - 12/2010)
10.	Weinstock, Leonard B: 3 articles (05/2022 - 01/2018)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Mast Cell Activation Syndrome:

1.	Tryptases (Tryptase)IBA 01/01/2019 - "Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome." 11/01/2014 - "Mast cell activation syndrome: improved identification by combined determinations of serum tryptase and 24-hour urine 11β-prostaglandin2α." 03/01/2023 - "Evaluation of a tryptase depletion index for better pathologic identification of mast cell activation syndrome." 12/09/2022 - "Mast cell activation syndrome (MCAS) derives from pathologic reaction of mast cells with increased tryptase serum level as a marker. " 01/01/2022 - "Idiopathic mast cell activation syndrome (MCAS) is characterized by three diagnostic criteria: (1) episodic mast cell (MC)-driven signs/symptoms of at least two organ systems in the absence of clonal MC expansion and definite triggers, (2) episodic increase in tryptase, and (3) response to MC-targeted treatment. "
2.	Omalizumab (Xolair)FDA Link 01/01/2020 - "Timing of response and long term efficacy of Omalizumab in non-clonal Mast Cell Activation Syndrome: A case series." 01/01/2019 - "Successful treatment of idiopathic mast cell activation syndrome with low-dose Omalizumab." 05/01/2012 - "Prevention of anaphylaxis related to mast cell activation syndrome with omalizumab."
3.	Hydroxychloroquine (Plaquenil)FDA LinkGeneric 01/01/2018 - "We report the efficacy of hydroxychloroquine in four patients with cutaneous mastocytosis (n = 2) and mast cell activation syndrome (n = 2). "
4.	Non-Steroidal Anti-Inflammatory Agents (NSAIDs)IBA 11/01/2018 - "Generalized pruritus relieved by NSAIDs in the setting of mast cell activation syndrome."
5.	Prostaglandins EIBA 10/01/1995 - "Our previous studies have examined the effects of intravenously administered PGE(2) in mast-cell-mediated diseases, including aspirin-sensitive asthma and systemic mast-cell-activation syndrome. "
6.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 10/01/1995 - "Our previous studies have examined the effects of intravenously administered PGE(2) in mast-cell-mediated diseases, including aspirin-sensitive asthma and systemic mast-cell-activation syndrome. "
7.	Histamine (Histamine Dihydrochloride)FDA Link 01/01/2023 - "Patients with mast cell activation syndrome presumably experience symptoms related to the aberrant elaboration of histamine and other mediators from normal-appearing mast cells present in normal numbers. " 01/01/2020 - "Comparing histamine intolerance and non-clonal mast cell activation syndrome." 01/01/2017 - "More controversially, other triggers of anaphylaxis, such as increased sensitivity to or reduced catabolism of histamine ("histamine intolerance") or mast cell activation syndrome also named mast cell activation disorder have been suggested, but still with very limited epidemiological evidence that a significant proportion of the observed reactions are caused by these alleged conditions. " 01/01/2022 - "Elevated plasma and tissues histamine concentrations can cause severe symptoms in mast cell activation syndrome, mastocytosis or anaphylaxis. " 01/01/2021 - "Excessive plasma histamine concentrations cause symptoms in mast cell activation syndrome, mastocytosis, or anaphylaxis. "
8.	Immunoglobulin E (IgE)IBA 01/01/2016 - "We present a case of a new onset, IgE mediated food allergy in combination with a mast cell activation syndrome in an elderly patient not known to have a history of atopy. " 01/01/2016 - "A novel combination of an IgE mediated adult onset food allergy and a suspected mast cell activation syndrome presenting as anaphylaxis." 02/01/2015 - "Mast cell activation syndromes (MCAS), mastocytosis, IgE to galactose-alpha-1,3-galactose (α-gal), and certain medications have recently been recognized as causes of anaphylaxis that were previously labeled idiopathic. " 10/01/2021 - "PubMed was searched between 1980 and 2021 using the following terms: mast cell activation syndromes, mast cell activation, anaphylaxis, KIT mutations, KIT D816V, indolent systemic mastocytosis, bone marrow mastocytosis, cutaneous mastocytosis, IgE anaphylaxis, and idiopathic anaphylaxis. " 01/01/2015 - "Age, sex, concomitant diseases, therapies, total IgE, serum tryptase levels and signs and symptoms suggesting mast cell activation syndrome (MCAS) were analyzed in relation to the severity of the reaction in accordance with the Mueller classification. "
9.	Prostaglandin D2IBA 01/01/2021 - "Increased urinary excretion of the metabolites of prostaglandin D2, 11β-prostaglandin(PG)F2α and (2,3-dinor)-11β-PGF2α, have been documented in systemic mastocytosis (SM) and in mast cell activation syndrome (MCAS). " 01/01/2022 - "Quantitation of urinary metabolites of histamine, prostaglandin D2, and leukotriene E4 can fill the gap in our current efforts to improve diagnosis and management of symptomatic patients with systemic mastocytosis, and/or mast cell activation syndrome, In addition, patients symptomatic due to mast cell activation but who do not meet all the criteria for mast cell activation syndrome can have elevated baseline mediator metabolites. " 01/01/2023 - "One requirement for diagnosing mast cell activation syndrome (MCAS) is an increase, above an established baseline level, in serum tryptase by 20% plus 2 ng/mL. However, there is no consensus of what constitutes excretion of a substantial increase in metabolites from prostaglandin D2, histamine, or leukotriene E4 in MCAS. "
10.	Histamine Antagonists (Antihistamines)IBA 01/01/2018 - "This effect, following extended and/or high antihistamine dosing, may have implications for other individuals with a disorder of mast cell activation, such as mastocytosis or mast cell activation syndrome. " 04/24/2017 - "Continuous diphenhydramine infusion can provide promising outcomes following the failure of intermittent antihistamine dosing in patients with severe mast cell activation syndrome. " 01/01/2022 - "Standards of care for mast cell activation syndrome (MCAS), as well as clinical reviews, experimental research, and case reports, suggest that effective and cost-efficient remedies are available, including antihistamines, vitamin C, and quercetin, among others. "

Therapies and Procedures

1.	Therapeutics 12/18/2021 - "Mast cell activation syndrome is a heterogeneous disorder, defined by a combination of (1) recurrent symptoms typical of mast cell activation, (2) an increase of validated mast cell derived mediators, and (3) response to treatment with mast cell stabilizing or mast cell mediator-targeted therapies. " 01/01/2019 - "This work provides a guide for dermatologists to strengthen diagnostic acuity for idiopathic mast cell activation syndrome, therefore contributing toward a goal of helping patients to receive timely, effective, and targeted therapy. " 01/01/2018 - "The presence of GI symptoms and a subjective response of symptoms to anti-mast cell mediator therapy are considered qualifying criteria in the diagnosis of the idiopathic mast cell activation syndrome. " 05/01/2015 - "Mast cell activation syndrome (MCAS) is a multisystem disorder characterized by objective documentation of elevated mast cell mediators during attacks and a favorable response to antimediator therapy. " 07/01/2014 - "Although many SM patients are suffering from mild or even no mediator-related symptoms, others have recurrent episodes of severe anaphylaxis, with clear signs of a mast cell activation syndrome (MCAS) despite prophylactic therapy with anti-mediator-type drugs. "
2.	Drug Therapy (Chemotherapy) 06/01/2021 - "Safe administration of chemotherapy in mast cell activation syndrome." 03/01/2021 - "Long-COVID syndrome symptoms, especially brain fog, are similar to those experienced by patients undertaking or following chemotherapy for cancer (chemofog or chemobrain), as well in patients with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) or mast cell activation syndrome (MCAS). "
3.	Skin Cream 06/01/2017 - "This flavonoid was formulated in a skin lotion along with olive fruit extract and was first tested for tolerability in 25 patients with mastocytosis or mast cell activation syndrome and very sensitive skin who reported back through a questionnaire. "
4.	Precision Medicine 11/27/2020 - "Diagnosis, Classification and Management of Mast Cell Activation Syndromes (MCAS) in the Era of Personalized Medicine."
5.	Embolectomy 05/01/2017 - "Successful Management of a Patient With Possible Mast Cell Activation Syndrome Undergoing Pulmonary Embolectomy: A Case Report."