Tu1845 – Identification of a Unique Population of Gi Mast Cells in a Subset of Patients with Mast Cell Activation Syndrome

Background: It has been shown repeatedly that mast cells can promote or prevent cancer development and growth. If development and/or progression of a solid cancer is substantially influenced by mast cell activity, the frequencies of occurrence of solid cancers in patients with primary mast cells disorders would be expected to differ from the corresponding prevalence data in the general population. In fact, a recent study demonstrated that patients with systemic mastocytosis (i.e., a rare neoplastic variant of the primary mast cell activation disease) have increased risk for solid cancers, in particular melanoma and non-melanoma skin cancers. The aim of the present study is to examine whether the risk of solid cancer is increased in systemic mast cell activation syndrome (MCAS), the common systemic variant of mast cell activation disease. Methods: In the present descriptive study, we have analysed a large (n=828) patient group with MCAS, consisting of cohorts from Germany and the USA, for occurrence of solid forms of cancer and compared the frequencies of the different cancers with corresponding prevalence data for German and U.S. general populations. Results: Sixty-eight of the 828 MCAS patients (46 female, 22 male) had developed a solid tumor before the diagnosis of MCAS was made. Comparison of the frequencies of the malignancies in the MCAS patients with their prevalence in the general population revealed a significantly increased prevalence for melanoma and cancers of the breast, cervix uteri, ovary, lung, and thyroid in MCAS patients. Conclusions: Our data support the view that mast cells may promote development of certain malignant tumors. These findings indicate a need for increased surveillance of certain types of cancer in MCAS patients irrespective of its individual clinical presentation.

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Comparative analysis of mutation of tyrosine kinase kit in mast cells from patients with systemic mast cell activation syndrome and healthy subjects

Immunogenetics ◽

10.1007/s00251-010-0474-8 ◽

2010 ◽

Vol 62 (11-12) ◽

pp. 721-727 ◽

Cited By ~ 36

Author(s):

Gerhard J. Molderings ◽

Kirsten Meis ◽

Ulrich W. Kolck ◽

Jürgen Homann ◽

Thomas Frieling

Keyword(s):

Mast Cell ◽

Comparative Analysis ◽

Mast Cells ◽

Tyrosine Kinase ◽

Healthy Subjects ◽

Cell Activation ◽

Mast Cell Activation ◽

Mast Cell Activation Syndrome ◽

Activation Syndrome

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Diagnosis of mast cell activation syndrome: a global “consensus-2”

Diagnosis ◽

10.1515/dx-2020-0005 ◽

2020 ◽

Vol 0 (0) ◽

Cited By ~ 1

Author(s):

Lawrence B. Afrin ◽

Mary B. Ackerley ◽

Linda S. Bluestein ◽

Joseph H. Brewer ◽

Jill B. Brook ◽

...

Keyword(s):

Mast Cell ◽

Mast Cells ◽

General Population ◽

Diagnostic Criteria ◽

Cell Activation ◽

Mast Cell Activation ◽

Sustained Improvement ◽

Mast Cell Activation Syndrome ◽

Global Consensus ◽

Activation Syndrome

AbstractThe concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of “mast cell activation syndrome” (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a “consensus” (re-termed here as “consensus-1”). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as “consensus-2”), resembling “consensus-1” in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by “consensus-2” criteria has potential to be problematic, but underdiagnosis by “consensus-1” criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.

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Diagnostic and Subdiagnostic Accumulation of Mast Cells in the Bone Marrow of Patients with Anaphylaxis: Monoclonal Mast Cell Activation Syndrome

International Archives of Allergy and Immunology ◽

10.1159/000096442 ◽

2006 ◽

Vol 142 (2) ◽

pp. 158-164 ◽

Cited By ~ 84

Author(s):

Karoline Sonneck ◽

Stefan Florian ◽

Leonhard Müllauer ◽

Friedrich Wimazal ◽

Manuela Födinger ◽

...

Keyword(s):

Bone Marrow ◽

Mast Cell ◽

Mast Cells ◽

Cell Activation ◽

Mast Cell Activation ◽

Mast Cell Activation Syndrome ◽

Activation Syndrome

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Mast Cell Activation Disorders

Medicina ◽

10.3390/medicina57020124 ◽

2021 ◽

Vol 57 (2) ◽

pp. 124

Author(s):

Arianna Giannetti ◽

Emanuele Filice ◽

Carlo Caffarelli ◽

Giampaolo Ricci ◽

Andrea Pession

Keyword(s):

Mast Cell ◽

Mast Cells ◽

Cell Activation ◽

Clinical Manifestations ◽

The Body ◽

Mast Cell Activation ◽

Detailed Knowledge ◽

Pediatric Age ◽

Mast Cell Activation Syndrome ◽

Activation Syndrome

Background and Objectives: Mast cell disorders comprise a wide spectrum of syndromes caused by mast cells’ degranulation with acute or chronic clinical manifestations. Materials and Methods: In this review article we reviewed the latest findings in scientific papers about mast cell disorders with a particular focus on mast cell activation syndrome and mastocytosis in pediatric age. Results: Patients with mast cell activation syndrome have a normal number of mast cells that are hyperreactive upon stimulation of various triggers. We tried to emphasize the diagnostic criteria, differential diagnosis, and therapeutic strategies. Another primary mast cell disorder is mastocytosis, a condition with a long-known disease, in which patients have an increased number of mast cells that accumulate in different regions of the body with different clinical evolution in pediatric age. Conclusions: Mast cell activation syndrome overlaps with different clinical entities. No consensus was found on biomarkers and no clearly resolutive treatment is available. Therefore, a more detailed knowledge of this syndrome is of fundamental importance for a correct diagnosis and effective therapy.

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Idiopathic mast cell activation syndrome and radiation therapy: a case study, literature review, and discussion of mast cell disorders and radiotherapy

Radiation Oncology ◽

10.1186/s13014-019-1434-6 ◽

2019 ◽

Vol 14 (1) ◽

Author(s):

Robin E. Landy ◽

William C. Stross ◽

Jackson M. May ◽

Tasneem A. Kaleem ◽

Timothy D. Malouff ◽

...

Keyword(s):

Mast Cell ◽

Radiation Therapy ◽

Mast Cells ◽

Cell Activation ◽

Breast Conservation ◽

Mast Cell Activation ◽

Radiation Dermatitis ◽

Mast Cell Activation Syndrome ◽

Tissue Reactions ◽

Activation Syndrome

Abstract Background Mast Cell Activation Syndrome (MCAS) is classified as an idiopathic mast cell disorder where inconsistent or unknown triggers release inflammatory mediators and cause a constellation of symptoms. Studies demonstrate mast cells increase histamine, tryptase, and inflammatory cytokine expression following ionizing radiation. Additionally, there are cases of cutaneous mastocytosis developing within the initial radiation field suggesting mast cells play a role in local tissue reactions. Literature is sparse on radiation induced toxicity in patients with mast cell disorders. Case presentation A 62 year old female patient with a history of MCAS received breast conservation therapy for invasive lobular carcinoma of the left breast initially AJCC 7th Stage IIB, pT3 pN0 M0. The patient underwent external beam radiotherapy (EBRT) and received 4500 cGy to the left whole breast, followed by a 1000 cGy boost to the lumpectomy cavity. She developed grade 1 radiation dermatitis. Two years later she progressed distantly and received stereotactic body radiation therapy to a lumbar vertebrae lesion to a dose of 2400 cGy in a single fraction. She developed no in-field dermatologic or systemic flare in her MCAS symptoms during radiation therapy. Conclusions To our knowledge there are no reported cases in the literature of patients diagnosed with MCAS or other idiopathic mast cell disorders undergoing radiation therapy. Idiopathic mast cell disorders such as MCAS and primary mast cell disorders alike should not be considered a contraindication to treatment with EBRT. This patient population appears to tolerate treatment without systemic flares in symptoms.

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Successful treatment of refractory anaphylaxis using omalizumab in monoclonal mast cell activation syndrome.

10.26226/morressier.5acc8ad1d462b8028d89a458 ◽

2018 ◽

Author(s):

Andrew O'Keefe

Keyword(s):

Mast Cell ◽

Successful Treatment ◽

Cell Activation ◽

Mast Cell Activation ◽

Mast Cell Activation Syndrome ◽

Activation Syndrome

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Association of Stiff person Syndrome with Anti GAD Antibodies with Immune Dysregulation and Symptoms of Mast Cell Activation Syndrome

10.26226/morressier.5acc8ad6d462b8028d89a67e ◽

2018 ◽

Author(s):

Ilona DuBuske

Keyword(s):

Mast Cell ◽

Cell Activation ◽

Immune Dysregulation ◽

Mast Cell Activation ◽

Stiff Person Syndrome ◽

Gad Antibodies ◽

Mast Cell Activation Syndrome ◽

Activation Syndrome

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Depression, psychosocial correlates, and psychosocial resources in individuals with mast cell activation syndrome

Journal of Health Psychology ◽

10.1177/13591053211014583 ◽

2021 ◽

pp. 135910532110145

Author(s):

Jennifer Nicoloro SantaBarbara ◽

Marci Lobel

Keyword(s):

Mast Cell ◽

Depressive Symptoms ◽

Cell Activation ◽

Physical Symptoms ◽

Mast Cell Activation ◽

Emotional States ◽

Mast Cell Activation Syndrome ◽

Disease Experience ◽

Clinically Significant ◽

Activation Syndrome

Individuals with Mast Cell Activation Syndrome (MCAS), a rare chronic disease, experience unpredictable physical symptoms and diagnostic challenges resulting in poor emotional states. The prevalence and correlates of depressive symptoms were examined among 125 participants who completed the CES-D and relevant instruments. The majority reported a clinically-significant level of depression which was especially common among younger participants and those who reported greater loneliness or more disease-specific stressors. Greater magnitude of depressive symptoms was associated with greater illness intrusiveness, less social support, and lower optimism. Results highlight the value of interventions targeting loneliness and stressors unique to this population.

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