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Anaphylaxis
Published in Pudupakkam K Vedanthan, Harold S Nelson, Shripad N Agashe, PA Mahesh, Rohit Katial, Textbook of Allergy for the Clinician, 2021
In some cases of anaphylaxis, a detailed history and extensive diagnostic evaluation, including testing for foods, medications, latex, exercise and insect stings are non-contributory. Idiopathic anaphylaxis is diagnosed when there is no identifiable cause or trigger for anaphylaxis (Greenberger and Lieberman 2014). To make this diagnosis, especially if there are recurrent episodes, underlying mast cell disorders (including systemic mastocytosis and monoclonal mast cell activation syndrome) should be ruled-out (Theoharides et al. 2015).
Mastocytosis
Published in Dongyou Liu, Tumors and Cancers, 2017
Due to somatic mutations in the KIT gene (frequently D816V; occasionally V1815, D816F, and D820G), mast cells may show uncontrolled clonal proliferation and/or defective apoptosis, leading to mast cell clonal disorders, which can be divided into mastocytosis and monoclonal mast cell activation syndrome (MCAS), depending on the level of clonality.
Recognition, treatment, and prevention of systemic allergic reactions and anaphylaxis *
Published in Richard F. Lockey, Dennis K. Ledford, Allergens and Allergen Immunotherapy, 2020
Emma Westermann-Clark, Stephen F. Kemp, Richard D. deShazo
Several systemic disorders share clinical features with anaphylaxis, with the vasodepressor (vasovagal) reaction probably the condition most commonly confused with it. In vasodepressor reactions, however, urticaria is absent, diaphoresis is often present, bradycardia is typically present, bronchospasm or dyspnea is usually absent, the blood pressure may be normal or depressed, and the skin is usually cool and pale. Tachycardia is the rule in anaphylaxis, but it may be absent in subjects with hypovolemia (after initial tachycardia), conduction defects, increased vagal tone due to a cardioinhibitory (Bezold-Jarisch) reflex, or in those who take sympatholytic (e.g. β-blockers) medications. Myocardial dysfunction may cause sudden hemodynamic collapse with or without an arrhythmia. A pulmonary embolism may produce tachycardia, dyspnea, tachypnea, and chest discomfort that can be pleuritic. Systemic mastocytosis, a disease characterized by mast cell proliferation in multiple organs, is usually associated with urticaria pigmentosa (brownish macules that transform into wheals upon stroking, called Darier's sign) and recurrent episodes of pruritus, flushing, tachycardia, abdominal pain, diarrhea, syncope, or headache. Mast cell activation syndrome (MCAS) is also associated with recurrent anaphylaxis; diagnostic algorithms for MCAS require three criteria including symptoms consistent with mast cell activation, biochemical markers such as tryptase, and response to medications that stabilize mast cells or inhibit mast cell mediators [52]. Neuroendocrine cell syndromes (e.g., carcinoid) may produce flushing and diarrhea. Other diagnostic considerations for children, in particular, include foreign-body aspiration, acute poisoning, and a seizure disorder. Diagnostic criteria for anaphylaxis in infants are published [90].
Systemic manifestations of Ehlers-Danlos syndrome
Published in Baylor University Medical Center Proceedings, 2021
Bo Song, Peter Yeh, John Harrell
Mast cell activation syndrome can present as flushing, pruritus, hypotension, asthma, diarrhea, bloating, and cramping.1 Patients may also exhibit food insensitivity and intolerance, and laboratory testing can reveal increased blood levels of mast cells and mast cell mediators such as histamine and tryptase.1 Cheung and Vadas reported a prevalence of 66% with mast cell syndrome as opposed to 24% in our study.26 This disparity could be due to several reasons. For one, mast cell activation syndrome can present similarly to common conditions such as seasonal allergies or the common cold.1 In addition, accurate diagnosis of mast cell activation syndrome often requires an allergist and utilization of advanced laboratory or tissue sampling measures.1 As no cure yet exists for mast cell activation syndrome, patients are treated symptomatically.27 Desensitization therapy can be used initially, and medications such as antihistamines, omalizumab, or leukotriene antagonists are alternatives.27 However, steroids should be avoided.2 Caution should also be exercised with using taping treatments for musculoskeletal pain as the skin can be hypersensitive to adhesives.28
Desensitization therapy using ‘Mariana Castells’ protocol in a patient with multiple autoimmune disorders- does it work?
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Syed Raza Shah, Terance Millan, Sardar Muhammad Alamzaib, Sue-Wei Luu
Our patient having a history of both multiple autoimmune diseases and mast-cell activation syndrome tolerated the protocol well with no complications. Appropriate treatment of the reactions including epinephrine use and management with personalized desensitization protocols can enhance the quality of life, life expectancy, and safety of an increasing at risk population of patients with infectious diseases allergic to their best medications. Most patients with reactions with phenotypes consistent with type I and type IV reactions are candidates for desensitization, which can provide advancement of personalized treatments.