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https://www.readbyqxmd.com/read/28609557/validation-of-international-consensus-equation-for-acute-serum-total-tryptase-in-mast-cell-activation-a-perioperative-perspective
#1
Richard L Baretto, Sarah Beck, Jane Heslegrave, Cathryn Melchior, Omar Mohamed, Anjali Ekbote, Aarnoud P Huissoon, Mamidipudi T Krishna
INTRODUCTION: There is no standardised method for assessing serum total mast cell tryptase (MCT) in anaphylaxis. The consensus equation (peak MCT should be>1.2x baseline tryptase+2mg/L) has been proposed to interpret acute MCT in mast cell activation syndrome (MCAS). AIM: To validate consensus equation in a setting of perioperative anaphylaxis. METHODS: Analyses of suspected perioperative anaphylaxis during general anaesthesia (GA). Anaphylaxis was defined as per World Allergy Organisation (WAO) criteria...
June 13, 2017: Allergy
https://www.readbyqxmd.com/read/28386644/mutational-profiling-in-the-peripheral-blood-leukocytes-of-patients-with-systemic-mast-cell-activation-syndrome-using-next-generation-sequencing
#2
Janine Altmüller, Britta Haenisch, Amit Kawalia, Markus Menzen, Markus M Nöthen, Heide Fier, Gerhard J Molderings
Mast cell activation syndrome (MCAS) and systemic mastocytosis (SM) are two clinical systemic mast cell activation disease variants. Few studies to date have investigated the genetic basis of MCAS. The present study had two aims. First, to investigate whether peripheral blood leukocytes from MCAS patients also harbor somatic mutations in genes implicated in SM using next-generation sequencing (NGS) technology and a relatively large MCAS cohort. We also addressed the question, whether some of the previously as somatic reported mutations are indeed germline mutations...
June 2017: Immunogenetics
https://www.readbyqxmd.com/read/28382662/successful-targeted-treatment-of-mast-cell-activation-syndrome-with-tofacitinib
#3
Lawrence B Afrin, Roger W Fox, Susan L Zito, Leo Choe, Sarah C Glover
Mast cell (MC) activation syndrome (MCAS) is a collection of illnesses of inappropriate MC activation with little to no neoplastic MC proliferation, distinguishing it from mastocytosis. MCAS presents as chronic, generally inflammatory multisystem polymorbidity likely driven in most by heterogeneous patterns of constitutively activating mutations in MC regulatory elements, posing challenges for identifying optimal mutation-targeted treatment in individual patients. Targeting commonly affected downstream effectors may yield clinical benefit independent of upstream mutational profile...
April 6, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28262205/characterization-of-mast-cell-activation-syndrome
#4
Lawrence B Afrin, Sally Self, Jeremiah Menk, John Lazarchick
BACKGROUND: Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. METHOD: Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS...
March 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28262030/highly-sensitive-assays-are-mandatory-for-the-differential-diagnosis-of-patients-presenting-with-symptoms-of-mast-cell-activation-diagnostic-work-up-of-38-patients
#5
Bea Van den Poel, Anne-Marie Kochuyt, Elke Del Biondo, Barbara Dewaele, Els Lierman, Thomas Tousseyn, Gert de Hertogh, Peter Vandenberghe, Nancy Boeckx
Mastocytosis is a heterogeneous disease caused by excessive mast cell (MC) proliferation. Diagnosis of systemic mastocytosis (SM) is based on the presence of major and minor criteria defined by the World Health Organization. Symptoms of MC activation can also occur in patients without SM or without allergic or inflammatory disease. These MC activation syndromes (MCAS) can be divided into primary (monoclonal) MCAS (MMAS) vs. secondary and idiopathic MCAS. In this single center study, the diagnostic work-up of 38 patients with a clinical suspicion of SM and/or with elevated basic tryptase levels is presented...
April 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/27132234/pharmacological-treatment-options-for-mast-cell-activation-disease
#6
REVIEW
Gerhard J Molderings, Britta Haenisch, Stefan Brettner, Jürgen Homann, Markus Menzen, Franz Ludwig Dumoulin, Jens Panse, Joseph Butterfield, Lawrence B Afrin
Mast cell activation disease (MCAD) is a term referring to a heterogeneous group of disorders characterized by aberrant release of variable subsets of mast cell (MC) mediators together with accumulation of either morphologically altered and immunohistochemically identifiable mutated MCs due to MC proliferation (systemic mastocytosis [SM] and MC leukemia [MCL]) or morphologically ordinary MCs due to decreased apoptosis (MC activation syndrome [MCAS] and well-differentiated SM). Clinical signs and symptoms in MCAD vary depending on disease subtype and result from excessive mediator release by MCs and, in aggressive forms, from organ failure related to MC infiltration...
July 2016: Naunyn-Schmiedeberg's Archives of Pharmacology
https://www.readbyqxmd.com/read/27012973/often-seen-rarely-recognized-mast-cell-activation-disease-a-guide-to-diagnosis-and-therapeutic-options
#7
REVIEW
Lawrence B Afrin, Joseph H Butterfield, Martin Raithel, Gerhard J Molderings
Mast cell (MC) disease has long been thought to be just the rare disease of mastocytosis (in various forms, principally cutaneous and systemic), with aberrant MC mediator release at symptomatic levels due to neoplastic MC proliferation. Recent discoveries now show a new view is in order, with mastocytosis capping a metaphorical iceberg now called "MC activation disease" (MCAD, i.e. disease principally manifesting inappropriate MC activation), with the bulk of the iceberg being the recently recognized "MC activation syndrome" (MCAS), featuring inappropriate MC activation to symptomatic levels with little to no inappropriate MC proliferation...
2016: Annals of Medicine
https://www.readbyqxmd.com/read/26714690/hymenoptera-allergy-and-mast-cell-activation-syndromes
#8
REVIEW
Patrizia Bonadonna, Massimiliano Bonifacio, Carla Lombardo, Roberta Zanotti
Mast cell activation syndrome (MCAS) can be diagnosed in patients with recurrent, severe symptoms from mast cell (MC)-derived mediators, which are transiently increased in serum and are attenuated by mediator-targeting drugs. When KIT-mutated, clonal MC are detected in these patients, a diagnosis of primary MCAS can be made. Severe systemic reactions to hymenoptera venom (HV) represent the most common form of anaphylaxis in patients with mastocytosis. Patients with primary MCAS and HV anaphylaxis are predominantly males and do not have skin lesions in the majority of cases, and anaphylaxis is characterized by hypotension and syncope in the absence of urticaria and angioedema...
January 2016: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/26480615/-non-clonal-mast-cell-activation-syndrome-description-of-series-of-patients-diagnostic-criteria-and-treatment
#9
Ronit Confino-Cohen, Yoseph A Mekori
A non-clonal mast cell activation syndrome is a newly emerged diagnosis. It shares the clinical features of anaphylaxis and mastocytosis despite having distinct mast cell biology. In this,paper we describe a series of patients representing the spectrum of non-clonal mast cell activation syndrome (nc-MCAS). We highlight the clinical manifestations of nc-MCAS as well as the diagnostic criteria and treatment options.
August 2015: Harefuah
https://www.readbyqxmd.com/read/26095756/h1-antihistamines-for-primary-mast-cell-activation-syndromes-a-systematic-review
#10
REVIEW
U B Nurmatov, E Rhatigan, F E R Simons, A Sheikh
BACKGROUND: Primary mast cell activation syndromes (MCAS) are a group of disorders presenting with symptoms of mast cell mediator release. OBJECTIVES: To assess the effectiveness and safety of orally administered H1 -antihistamines in the treatment of primary MCAS compared with placebo and other pharmacologic treatments. METHODS: We systematically searched five databases and three trial repositories and contacted an international panel of experts to identify published and unpublished trials...
September 2015: Allergy
https://www.readbyqxmd.com/read/26072665/successful-treatment-of-mast-cell-activation-syndrome-with-sunitinib
#11
Lawrence B Afrin, Frank M Cichocki, Kamal Patel, Gerhard J Molderings
Mast cell (MC) activation syndrome (MCAS) is a recently recognized, likely prevalent collection of heterogeneous illnesses of inappropriate MC activation with little to no MC neoplasia likely driven by heterogeneous patterns of constitutively activating mutations in MC regulatory elements including various tyrosine kinases (TKs, dominantly KIT). MCAS typically presents as chronic multisystem polymorbidity of generally inflammatory ± allergic theme. As with indolent systemic mastocytosis (SM), treatment of MCAS focuses more against MC mediators than MC neoplasia, but some cases prove refractory even to the TK inhibitor (TKI) imatinib reported useful both in uncommon SM cases not bearing SM's usual imatinib-resistant KIT-D816V mutation and in some cases of MCAS (which rarely bears KIT-D816V)...
December 2015: European Journal of Haematology
https://www.readbyqxmd.com/read/25968421/anti-amoxicillin-immunoglobulin-e-histamine-2-receptor-antagonist-therapy-and-mast-cell-activation-syndrome-are-risk-factors-for-amoxicillin-anaphylaxis
#12
Elide Anna Pastorello, Chrysi Stafylaraki, Corrado Mirone, Donatella Preziosi, Maria Gloria Aversano, Ambra Mascheri, Laura Michelina Losappio, Valeria Ortolani, Michele Nichelatti, Laura Farioli
BACKGROUND: β-Lactam antibiotics (mainly amoxicillin, AX) are the drugs that most frequently induce systemic drug allergy reactions. OBJECTIVE: We attempted to identify the risk factors associated with systemic reactions to AX. METHODS: All patients who were referred to our department for suspected hypersensitivity reactions to AX over a 6-month period were evaluated for anti-AX immunoglobulin E (IgE) levels and skin-test positivity for β-lactams...
2015: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/25944644/mast-cell-activation-syndrome
#13
Marianne Frieri
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 frequency, severity, or resolution of symptoms with anti-mediator therapy including H1/H2 receptor antagonists, anti-leukotrienes, or mast cell stabilizers...
May 6, 2015: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/25909362/determination-of-plasma-heparin-level-improves-identification-of-systemic-mast-cell-activation-disease
#14
Milda Vysniauskaite, Hans-Jörg Hertfelder, Johannes Oldenburg, Peter Dreßen, Stefan Brettner, Jürgen Homann, Gerhard J Molderings
Diagnosis of mast cell activation disease (MCAD), i.e. systemic mastocytosis (SM) and idiopathic systemic mast cell activation syndrome (MCAS), usually requires demonstration of increased mast cell (MC) mediator release. Since only a few MC mediators are currently established as biomarkers of MCAD, the sensitivity of plasma heparin level (pHL) as an indicator of increased MC activation was compared with that of serum tryptase, chromogranin A and urinary N-methylhistamine levels in 257 MCAD patients. Basal pHL had a sensitivity of 41% in MCAS patients and 27% in SM patients...
2015: PloS One
https://www.readbyqxmd.com/read/25841551/mast-cell-activation-syndromes-presenting-as-anaphylaxis
#15
REVIEW
Cem Akin
Anaphylaxis results from severe systemic mast cell activation. In addition to IgE-mediated and physical triggers, it may occur with a clonal mast cell disease and in an idiopathic fashion without clear provoking factors. Disorders of mast cell activation are classified into primary (clonal), secondary, and idiopathic. 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...
May 2015: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/25725228/pathogenesis-newly-recognized-etiologies-and-management-of-idiopathic-anaphylaxis
#16
REVIEW
James L Kuhlen, Yamini V Virkud
Idiopathic anaphylaxis (IA) is a life-threatening allergic disease and the most common diagnosis given to patients following an anaphylactic event. The inability of the healthcare provider and the patient to identify the trigger for anaphylaxis makes standard allergen avoidance measures ineffectual. IA is diagnosed after other causes of anaphylaxis have been excluded. 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...
February 2015: Discovery Medicine
https://www.readbyqxmd.com/read/25439370/mast-cell-activation-syndrome-improved-identification-by-combined-determinations-of-serum-tryptase-and-24-hour-urine-11%C3%AE-prostaglandin2%C3%AE
#17
Anupama Ravi, Joseph Butterfield, Catherine R Weiler
BACKGROUND: Mast cell activation syndrome (MCAS) describes patients with episodes of mast cell mediator release, with negative bone marrow biopsy results, and the failure to meet the criteria for systemic mastocytosis. OBJECTIVE: Identify elevation of mast cell mediators of patients with MCAS. METHODS: We performed a retrospective study of 25 patients with MCAS who were evaluated at Mayo Clinic from 2006 to 2012. Patients were reviewed for MCAS symptoms and mast cell mediators, including serum tryptase and 24-hour urine N-methyl histamine (N-MH) and 11β-prostaglandin-F₂α (11β-PGF₂α)...
November 2014: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/25171546/mast-cell-activation-syndrome-as-a-significant-comorbidity-in-sickle-cell-disease
#18
Lawrence B Afrin
Some sickle cell anemia (SCA) patients suffer significantly worse phenotypes than others. Causes of such disparities are incompletely understood. Comorbid chronic inflammation likely is a factor. Recently, mast cell (MC) activation (creating an inflammatory state) was found to be a significant factor in sickle pathobiology and pain in a murine SCA model. Also, a new realm of relatively noncytoproliferative MC disease termed MC activation syndrome (MCAS) has been identified recently. MCAS has not previously been described in SCA...
December 2014: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/24784142/spectrum-of-mast-cell-activation-disorders
#19
REVIEW
Anastasia I Petra, Smaro Panagiotidou, Julia M Stewart, Pio Conti, Theoharis C Theoharides
Mast cell (MC) activation disorders present with multiple symptoms including flushing, pruritus, hypotension, gastrointestinal complaints, irritability, headaches, concentration/memory loss and neuropsychiatric issues. These disorders are classified as: cutaneous and systemic mastocytosis with a c-kit mutation and clonal MC activation disorder, allergies, urticarias and inflammatory disorders and mast cell activation syndrome (MCAS), idiopathic urticaria and angioedema. MCs are activated by IgE, but also by cytokines, environmental, food, infectious, drug and stress triggers, leading to secretion of multiple mediators...
June 2014: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/24702655/risk-factors-and-management-of-severe-life-threatening-anaphylaxis-in-patients-with-clonal-mast-cell-disorders
#20
REVIEW
P Valent
Several different risk factors and conditions may predispose to severe life-threatening anaphylaxis. Systemic mastocytosis (SM) is one such condition. 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. In several of these patients, an IgE-dependent allergy is diagnosed. The severity and frequency of MCAS reactions neither correlate with the burden of neoplastic mast cells nor with the levels of specific IgE or the basal tryptase level...
July 2014: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
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