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Immunology and Allergy Clinics of North America

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https://www.readbyqxmd.com/read/30007470/mastocytosis-moving-the-field-to-precision-and-personalized-medicine
#1
EDITORIAL
Mariana C Castells
No abstract text is available yet for this article.
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007469/the-many-faces-of-mast-cell-disorders
#2
EDITORIAL
Stephen A Tilles
No abstract text is available yet for this article.
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007468/tyrosine-kinase-inhibition-in-mastocytosis-kit-and-beyond-kit
#3
REVIEW
Siham Bibi, Michel Arock
Mastocytosis is a group of rare disorders characterized by abnormal accumulation of mast cells in one or several organs. Mastocytosis can be seen at any age; but, in adults, the disease is usually systemic and chronic. Patients with indolent systemic mastocytosis (SM) are usually treated symptomatically, but cytoreductive treatments are needed in more advanced SM. In most patients with SM, an activating KIT D816V mutation is found. Thus, patients with advanced SM benefit from treatment with KIT-targeting tyrosine kinase inhibitors...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007467/patient-perceptions-in-mast-cell-disorders
#4
REVIEW
Susan V Jennings, Valerie M Slee, Rachel M Zack, Srdan Verstovsek, Tracy I George, Hongliang Shi, Philina Lee, Mariana C Castells
Understanding experiences, perceptions, and perspectives of patients with a mast cell disorder (MCD), including cutaneous mastocytosis, systemic mastocytosis, mast cell activation syndromes, and hereditary α-tryptasemia, is an important aspect of successful care, treatment, and informed development of novel therapies. This article reviews existing studies and presents new data on MCD patient perceptions regarding medical care, symptoms, allergies/sensitivities, triggers, future health/disease progression, treatment, impact on daily living, quality of life, support needs, and concerns regarding possible familial disease...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007466/association-of-postural-tachycardia-syndrome-and-ehlers-danlos-syndrome-with-mast-cell-activation-disorders
#5
REVIEW
Rafael Bonamichi-Santos, Kelly Yoshimi-Kanamori, Pedro Giavina-Bianchi, Marcelo Vivolo Aun
Mast cell activation disorders (MCADs) consist of episodic systemic symptoms due to mast cell mediator release. Diagnosis is based on clinical presentation and determination of high levels of tryptase or histamine. Ehlers-Danlos syndrome (EDS) and postural tachycardia syndrome (POTS) frequently coexist. It has been described that individuals with these syndromes can even present symptoms compatible to MCADs, which could represent a new specific phenotype. Preliminary genetic data suggest a role for tryptase in the pathogenesis of MCADs, EDS, and POTS association...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007465/hereditary-alpha-tryptasemia-genotyping-and-associated-clinical-features
#6
REVIEW
Jonathan J Lyons
Hereditary alpha tryptasemia is an autosomal dominant genetic trait caused by increased germline copies of TPSAB1 encoding alpha-tryptase. Individuals with this trait have elevated basal serum tryptase, and may present with associated multisystem complaints. Both basal serum tryptase levels and severity of clinical symptoms display a gene-dose relationship with TPSAB1, whereby higher tryptase levels and greater symptom severity are correlated with increasing numbers of alpha-encoding TPSAB1. As the functional effects of increased basal serum tryptase and/or altered tryptase gene expression are elucidated, greater insights will be gained into the symptoms associated with hereditary alpha tryptasemia and their potential therapy...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007464/nonclonal-mast-cell-activation-syndrome-a-growing-body-of-evidence
#7
REVIEW
Matthew J Hamilton
Patients who present with typical features of mast cell activation with laboratory confirmation and without evidence of a clonal mast cell disorder or other medical condition should be initiated on medical treatment to block mast cells and their mediators. If a major response is achieved, a diagnosis of nonclonal mast cell activation syndrome (NC-MCAS) is likely and treatment should be optimized, including management of any associated conditions. In this review, the latest evidence with regard to the diagnosis and treatment of NC-MCAS is presented...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007463/hymenoptera-anaphylaxis-as-a-clonal-mast-cell-disorder
#8
REVIEW
Patrizia Bonadonna, Luigi Scaffidi
Up to 7% of adult patients with Hymenoptera venom allergy may suffer from a clonal mast cell disease. Patients with clonal mast cell disease and Hymenoptera venom anaphylaxis are commonly males, without skin lesions, and anaphylaxis is characterized by hypotension and syncope in the absence of urticaria and angioedema. A normal value of tryptase does not exclude a mastocytosis. The diagnosis of a mast cell disease leads to several therapeutic consequences concerning the treatment of Hymenoptera venom allergy as matter of fact these patients have to undergo long-life venom immunotherapy, to prevent further, potentially fatal severe reactions...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007462/bone-disease-in-mastocytosis
#9
REVIEW
Giovanni Orsolini, Ombretta Viapiana, Maurizio Rossini, Massimiliano Bonifacio, Roberta Zanotti
Systemic mastocytosis can give very different bone pictures: from osteosclerosis to osteoporosis. Osteoporosis is one of the most frequent manifestations particularly in adults and the most clinical relevant. It is often complicated by a high recurrence of mainly vertebral fragility fractures. The main factor of bone loss is the osteoclast with a relative or absolute predominance of bone resorption. The RANK-RANKL pathway seems of key importance, but histamine and other cytokines also play a significant role in the process...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007461/gastrointestinal-involvement-in-mast-cell-activation-disorders
#10
REVIEW
Fred H Hsieh
Gastrointestinal (GI) symptoms are commonly reported in patients with mast cell disease. GI involvement in systemic mastocytosis is heterogeneous and symptoms may be caused by infiltration of abnormal mast cells in the GI tract and/or by the downstream effect of mast cell mediators on GI tissues. GI symptoms described the monoclonal mast cell activation syndrome are best characterized in the context of acute anaphylaxis. 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...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007460/kit-mutations-new-insights-and-diagnostic-value
#11
REVIEW
Lorenzo Falchi, Srdan Verstovsek
Mastocytosis is a World Health Organization-defined clonal mast cell disorder characterized by significant clinicopathologic heterogeneity. Despite this diversity, a mutation of the KIT gene, most commonly D816V, is found in almost all cases and believed a driver lesion. Peripheral blood allele-specific oligonucleotide polymerase chain reaction can reliably detect KIT D816V and is used for the initial screening of adults with suspected systemic mastocytosis. The discovery of KIT mutations as central to the pathobiology of mastocytosis has prompted development of KIT-targeted agents, including imatinib and midostaurin (approved medications for patients with advanced systemic mastocytosis), and drugs in development, like KIT D816V-specific inhibitor avapritinib...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007459/mast-cell-mediators-of-significance-in-clinical-practice-in-mastocytosis
#12
REVIEW
Joseph H Butterfield, Anupama Ravi, Thanai Pongdee
Mast cells leave evidence, a "fingerprint," of their participation in acute and chronic clinical events. That fingerprint is an elevation, either chronic or acute, in levels of their secreted mediators or their metabolites. Of these, only serum tryptase is currently one of the diagnostic criteria for systemic mastocytosis or mast cell activation. Combinations of easily obtained and quantified urinary mast cell mediator metabolite levels correlate well with bone marrow findings of systemic mastocytosis...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007458/bone-marrow-expression-of-mast-cell-disorders
#13
REVIEW
Laura Sánchez-Muñoz, Ana Filipa Henriques, Iván Alvarez-Twose, Almudena Matito
Mast cell disorders comprise a heterogeneous group of rare diseases, the diagnosis of which still remains a challenge. Bone marrow analysis constitutes the most appropriate site for screening systemic involvement in mastocytosis. Morphologic, immunohistochemical, flow cytometric immunophenotyping, and molecular studies should be routinely performed for diagnostic/prognostic purposes in experienced reference centers during the diagnostic workup in suspected systemic mastocytosis. The authors review the most relevant characteristics of bone marrow expression of mast cell disorders as well as the different methodological approaches to be applied to perform an objective and reproducible diagnosis and classification of mastocytosis and other mast cell disorders...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007457/pediatric-expression-of-mast-cell-activation-disorders
#14
REVIEW
Sigurd Broesby-Olsen, Melody Carter, Henrik Fomsgaard Kjaer, Charlotte Gotthard Mortz, Michael Boe Møller, Thomas Kielsgaard Kristensen, Carsten Bindslev-Jensen, Lone Agertoft
Mast cell activation disorders is a term proposed to cover diseases and conditions related to activation of mast cells and effects of mast cell mediators. In its broadest sense, the term encompasses a wide range of diseases from allergic asthma to rhinoconjunctivitis, urticaria, food allergy, anaphylaxis, mastocytosis, and other conditions where MC activation is contributing to the pathogenesis. This article focuses on clinical presentations, challenges, and controversies in pediatric mastocytosis and gives an overview of current knowledge and areas in need of further research...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007456/cutaneous-mastocytosis-in-adults-and-children-new-classification-and-prognostic-factors
#15
REVIEW
Almudena Matito, José Manuel Azaña, Antonio Torrelo, Iván Alvarez-Twose
The skin is one of the most frequent tissues affected in patients with mastocytosis, but cutaneous lesions are highly heterogeneous in shape, size, color, number, localization, and distribution. The World Health Organization recognizes 3 subtypes of cutaneous mastocytosis (CM): maculopapular CM (MPCM), diffuse CM, and mastocytoma of skin. An international task force of experts in mastocytosis has recently proposed subdividing MPCM into monomorphic and polymorphic, which could predict the duration of the disease in children...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/30007455/new-insights-into-clonal-mast-cell-disorders-including-mastocytosis
#16
REVIEW
Sofija Volertas, Charles F Schuler, Cem Akin
Mastocytosis is a heterogeneous group of neoplasms that involve the clonal expansion of mast cells into one or more organ systems, which typically involves the skin and hematopoietic systems. Systemic mastocytosis consists of a multifocal infiltration of mast cells into various noncutaneous tissue sites, especially the bone marrow. Diagnosis requires tissue confirmation, and algorithms have been developed to assist clinicians in this process. The current classification system focuses on delineating prognostic categories...
August 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/29631744/exercise-and-the-total-airway-a-call-to-action
#17
EDITORIAL
J Tod Olin, James H Hull
No abstract text is available yet for this article.
May 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/29631743/exercise-induced-airway-dysfunction-in-athletes
#18
EDITORIAL
Stephen A Tilles
No abstract text is available yet for this article.
May 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/29631742/food-allergy-point-of-care-pearls
#19
REVIEW
J Andrew Bird
Food allergy should be suspected in individuals with a history of immediate reactivity following ingestion (ie, typically within 20 minutes and almost always within 2 hours) with typical symptoms of immunoglobulin E-mediated reactivity (eg, urticaria, angioedema, coughing, wheezing, vomiting). Testing for food allergy should focus on the most likely allergen to provoke the reaction based on the patient's history. Safe introduction of peanut-containing foods into the diet of an infant at high risk of developing peanut allergy at 4 to 6 months is likely to reduce the risk of peanut allergy...
May 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/29631741/the-future-of-exertional-respiratory-problems-what-do-we-know-about-the-total-airway-approach-and-what-do-we-need-to-know
#20
REVIEW
J Tod Olin, James H Hull
Exercise is increasingly viewed as a preventative and therapeutic modality for medical and behavioral health disorders. Therefore, it is imperative that the medical and scientific communities minimize barriers that discourage exercise. This issue of Immunology and Allergy Clinics of North America details a "total airway approach" to the evaluation of exertional respiratory problems. Reviews guide clinicians through evaluation and therapy. Moving forward, there is much room for growth with respect to research in each of these areas as well as for common inflammatory pathways and neurophysiologic coupling across all airway segments...
May 2018: Immunology and Allergy Clinics of North America
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