scholarly journals Mast Cell Activation Disorders

Medicina ◽  
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.

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

2019 ◽  
Vol 156 (6) ◽  
pp. S-1146
Author(s):  
Matthew Hamilton ◽  
Liza Konnikova ◽  
Olga Pozdnyakova ◽  
Jonathan J. Lyons ◽  
Joshua D. Milner ◽  
...  
Keyword(s):  
Mast Cell ◽  
Mast Cells ◽  
Cell Activation ◽  
Mast Cell Activation ◽  
Mast Cell Activation Syndrome ◽  
Activation Syndrome

scholarly journals Risk of solid cancer in patients with mast cell activation syndrome: Results from Germany and USA

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 1889 ◽  
Author(s):  
Gerhard J. Molderings ◽  
Thomas Zienkiewicz ◽  
Jürgen Homann ◽  
Markus Menzen ◽  
Lawrence B. Afrin
Keyword(s):  
Mast Cell ◽  
Mast Cells ◽  
General Population ◽  
Cell Activation ◽  
Mast Cell Activation ◽  
Solid Cancer ◽  
Prevalence Data ◽  
Mast Cell Activation Syndrome ◽  
Mast Cell Activation Disease ◽  
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.

Mast cell activation syndrome: A newly recognized disorder with systemic clinical manifestations

2011 ◽  
Vol 128 (1) ◽  
pp. 147-152.e2 ◽  
Author(s):  
Matthew J. Hamilton ◽  
Jason L. Hornick ◽  
Cem Akin ◽  
Mariana C. Castells ◽  
Norton J. Greenberger
Keyword(s):  
Mast Cell ◽  
Cell Activation ◽  
Clinical Manifestations ◽  
Mast Cell Activation ◽  
Mast Cell Activation Syndrome ◽  
Activation Syndrome

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

Diagnosis ◽  
2020 ◽  
Vol 0 (0) ◽  
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.

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

2006 ◽  
Vol 142 (2) ◽  
pp. 158-164 ◽  
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

scholarly journals Manejo anestésico para craniectomía descompresiva por malformación de Chiari tipo I en paciente con sospecha de síndrome de activación mastocitaria

2020 ◽  
Vol 12 (1) ◽  
pp. 2
Author(s):  
Fátima Ortiz Parra ◽  
M González De La Aleja ◽  
Claudia Olea Vielba
Keyword(s):  
Mast Cell ◽  
Cell Activation ◽  
Clinical Manifestations ◽  
Anaesthetic Management ◽  
Mast Cell Activation ◽  
Type I ◽  
Mast Cell Activation Syndrome ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Activation Syndrome

La mastocitosis es una patología infrecuente a la que, sin embargo, todo anestesiólogo debe saber hacer frente por las repercusiones clínicas que pueden derivarse de un manejo perioperatorio inadecuado.  Sus manifestaciones clínicas se deben a la liberación de gránulos de histamina, prostaglandinas y otras sustancias vasoactivas contenidas en los mastocitos, desencadenada por multitud de factores y fármacos, muchos de los cuales son habitualmente empleados en el acto anestésico.  Presentamos el caso de una paciente con sospecha de síndrome de activación mastocitaria y múltiples alergias documentadas, sometida a craniectomía descompresiva por una malformación de Chiari tipo 1 con siringomielia.   ABSTRACT Anaesthetic management for descompressive craniectomy in Chiari type I malformation in a patient with suspicion of mast cell activation syndrome Mastocytosis is a rare disease whose management must be performed by any anaesthetist because of the clinical consequences that an innapropiate perioperative management may bring.   Its clinical manifestations are due to the release of histamine and other pro-inflammatory substances holded by mast cells, triggered by multiple elements and many drugs: more specifically, many of the drugs we use in the anaesthetic procedure. We present the case of a patient with suspicion of mast cell activation syndrome and many certified allergies, who was proposed to be operated on because of a Chiari type 1 malformation with syringomyelia.

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