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Systemic mastocytosis

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https://www.readbyqxmd.com/read/28185248/mastering-gut-permeability-new-roles-for-old-friends
#1
Michael Bramhall, Colby Zaph
Mast cells are innate immune cells that respond rapidly to infection in barrier tissues such as the skin and intestinal mucosa. Expulsion of parasitic worms in the gut involves a robust type 2 host response, and an acute mastocytosis is often generated at the site of infection. However, the role of mast cells in resistance to worm infections appears to be parasite specific. Mast cells are also involved in tissue repair, but the long-term contribution of mast cell activation after worm expulsion has not been definitively studied...
February 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28181948/kounis-syndrome-during-anesthesia-presentation-of-indolent-systemic-mastocytosis-a-case-report
#2
Elena de la Fuente Tornero, Arantza Vega Castro, Pedro Álvarez de Sierra Hernández, Javier Balaguer Recena, Sofía Carmen Zaragoza Casares, Francisco Miguel Serrano Baylin, Paloma Gallardo Culebradas, Beatriz Amorós Alfonso, Jose Ramón Rodríguez Fraile
Mastocytosis comprises a heterogeneous group of disorders characterized by mast cell accumulation and proliferation in distinct organs. Kounis syndrome is defined as the concurrence of acute coronary syndromes with mast cell activation in a setting of allergic or hypersensitivity reactions. This is the first reported case of an intraoperative Kounis syndrome as the onset of an indolent systemic mastocytosis probably triggered by succinylated gelatin infusion during general anesthesia. The presentation of this case is intended to contribute to the knowledge of mastocytosis and Kounis syndrome at the time of diagnostic workup during intraoperative anaphylaxis or myocardial ischemia...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28141734/anaesthesia-and-orphan-disease-rapid-sequence-induction-in-systemic-mastocytosis
#3
Christoph Unterbuchner, Marina Hierl, Timo Seyfried, Thomas Metterlein
No abstract text is available yet for this article.
March 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28135563/pathogenesis-and-pathology-of-mastocytosis
#4
Dean D Metcalfe, Yoseph A Mekori
Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in the molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations, including new and novel tyrosine kinase inhibitors...
January 24, 2017: Annual Review of Pathology
https://www.readbyqxmd.com/read/28133781/longitudinal-study-of-pediatric-urticaria-pigmentosa
#5
Adam Heinze, Travis J Kuemmet, Yvonne E Chiu, Sheila S Galbraith
BACKGROUND/OBJECTIVES: Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life. METHODS: Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI)...
January 30, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28112808/nerve-growth-factor-a-neuroimmune-crosstalk-mediator-for-all-seasons
#6
REVIEW
Stephen D Skaper
Neurotrophic factors comprise a broad family of biomolecules - most of which are peptides or small proteins - that support the growth, survival, and differentiation of both developing and mature neurons. The prototypical example and best-characterized neurotrophic factor is nerve growth factor (NGF) which is widely recognized as a target-derived factor responsible for the survival and maintenance of the phenotype of specific subsets of peripheral neurons and basal forebrain cholinergic nuclei during development and maturation...
January 23, 2017: Immunology
https://www.readbyqxmd.com/read/28095350/progressive-multifocal-leukoencephalopathy-in-a-patient-with-systemic-mastocytosis-treated-with-cladribine
#7
Karl B Alstadhaug, Randi Fykse Halstensen, Francis Odeh
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML. CASE DESCRIPTION: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis...
December 14, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28074480/clonal-reticulohistiocytosis-of-the-skin-and-bone-marrow-associated-with-systemic-mastocytosis-and-acute-myeloid-leukaemia
#8
Nicola Fusco, Arturo Bonometti, Claudia Augello, Sonia Fabris, Leonardo Boiocchi, Stefano Fiori, Denise Morotti, Nicola Fracchiolla, Emilio Berti, Umberto Gianelli
AIMS: The aims of this study were to define whether diffuse cutaneous reticulohistiocytosis could be underpinned by somatic genetic alterations and represent precursor to more aggressive forms of disease. METHODS AND RESULTS: A 59-year-old man with diffuse cutaneous reticulohistiocytosis, experienced bone marrow localisation of the disease, with associated systemic mastocytosis and acute myeloid leukaemia. Cytogenetic analyses of the bone marrow aspirate revealed the presence of a derivative chromosome giving rise to a partial trisomy of chromosome 1q and a partial monosomy of chromosome 9q...
January 11, 2017: Histopathology
https://www.readbyqxmd.com/read/28069280/a-new-therapeutic-advance-for-symptomatic-systemic-mastocytosis
#9
Michel Arock
No abstract text is available yet for this article.
January 6, 2017: Lancet
https://www.readbyqxmd.com/read/28069279/masitinib-for-treatment-of-severely-symptomatic-indolent-systemic-mastocytosis-a-randomised-placebo-controlled-phase-3-study
#10
Olivier Lortholary, Marie Olivia Chandesris, Cristina Bulai Livideanu, Carle Paul, Gérard Guillet, Ewa Jassem, Marek Niedoszytko, Stéphane Barete, Srdan Verstovsek, Clive Grattan, Gandhi Damaj, Danielle Canioni, Sylvie Fraitag, Ludovic Lhermitte, Sophie Georgin Lavialle, Laurent Frenzel, Lawrence B Afrin, Katia Hanssens, Julie Agopian, Raphael Gaillard, Jean-Pierre Kinet, Christian Auclair, Colin Mansfield, Alain Moussy, Patrice Dubreuil, Olivier Hermine
BACKGROUND: Indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with reduced quality of life. Masitinib inhibits KIT and LYN kinases that are involved in indolent systemic mastocytosis pathogenesis. We aimed to assess safety and efficacy of masitinib versus placebo in severely symptomatic patients who were unresponsive to optimal symptomatic treatments. METHODS: In this randomised, double-blind, placebo-controlled, phase 3 study, we enrolled adults (aged 18-75 years) with indolent or smouldering systemic mastocytosis, according to WHO classification or documented mastocytosis based on histological criteria, at 50 centres in 15 countries...
6, 2017: Lancet
https://www.readbyqxmd.com/read/28038453/histone-deacetylase-inhibitor-saha-mediates-mast-cell-death-and-epigenetic-silencing-of-constitutively-active-d816v-kit-in-systemic-mastocytosis
#11
Katarina Lyberg, Hani Abdulkadir Ali, Jennine Grootens, Matilda Kjellander, Malin Tirfing, Michel Arock, Hans Hägglund, Gunnar Nilsson, Johanna Ungerstedt
Systemic mastocytosis (SM) is a clonal bone marrow disorder, where therapeutical options are limited. Over 90% of the patients carry the D816V point mutation in the KIT receptor that renders this receptor constitutively active. We assessed the sensitivity of primary mast cells (MC) and mast cell lines HMC1.2 (D816V mutated), ROSA (KIT WT) and ROSA (KIT D816V) cells to histone deacetylase inhibitor (HDACi) treatment. We found that of four HDACi, suberoyl anilide hydroxamic acid (SAHA) was the most effective in killing mutated MC...
December 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27981619/familial-progressive-hyperpigmentation-cutaneous-mastocytosis-and-gastrointestinal-stromal-tumor-as-clinical-manifestations-of-mutations-in-the-c-kit-receptor-gene
#12
Tatiana Piqueres-Zubiaurre, Zuriñe Martínez de Lagrán, Ricardo González-Pérez, Amaia Urtaran-Ibarzabal, Guiomar Perez de Nanclares
BACKGROUND: Familial progressive hyperpigmentation (FPH) is an autosomal dominant disorder characterized by the appearance of hyperpigmented patches on the skin from early infancy that increase in size and number with age. METHODS: We report the clinical and molecular studies of an 11-year-old boy who had areas of hyperpigmentation since birth that had spread across his body as irregular hyperpigmented macules and papules, and include relevant history in family members...
December 16, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27970630/development-and-content-validity-of-the-advanced-systemic-mastocytosis-symptom-assessment-form-advsm-saf
#13
Mazar, E Evans, F Taylor, A Patki, O Ojo, R E Lamoureux, H Horny, A Reiter, J Gotlib, A Shields
No abstract text is available yet for this article.
November 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/27968936/mast-cell-disorders-kids-are-not-just-little-people
#14
Marti J Rothe, Jane M Grant-Kels, Hanspaul S Makkar
Cutaneous mastocytosis is characterized by a pathologic increase in mast cells in the skin and may also involve extracutaneous organs. Symptoms, which are triggered by mast cell degranulation, vary depending on the burden of skin disease and the presence of extracutaneous disease. The clinical presentation, risk of systemic disease, pathogenesis, prognosis, and treatment options differ, largely depending on age at presentation. In the pediatric population, spontaneous remission is typical, generally by puberty, whereas in adults, progression is observed...
November 2016: Clinics in Dermatology
https://www.readbyqxmd.com/read/27942432/mast-cell-clonal-disorders-classification-diagnosis-and-management
#15
REVIEW
Merel C Onnes, Luciana K Tanno, Joanne N G Oude Elberink
Mast cell clonal disorders are characterized by the clonal proliferation of pathological mast cells as a result of somatic mutations in the KIT gene, most commonly the D816V mutation. Accumulation and degranulation of these cells causes a wide variety of symptoms. Mast cell clonal disorders can be divided into mastocytosis and monoclonal mast cell activation syndrome, depending of the level of clonality. The severity of mastocytosis varies from an indolent variant with a good prognosis, to an aggressive condition with short life expectancy...
2016: Current Treatment Options in Allergy
https://www.readbyqxmd.com/read/27921051/systemic-mastocytosis-causing-refractory-pruritus-in-a-liver-disease-patient
#16
Naga S Addepally, Jagpal S Klair, Mohit Girotra, Johnny Jones, Farshad Aduli
Systemic mastocytosis (SM) results from clonal, neoplastic proliferation of abnormal mast cells. Patients become susceptible to itching, urticaria, and anaphylactic shock, which occurs due to histamine release from mast cells. SM may coexist alongside other systemic diseases, thus confounding the overall clinical presentation. We discuss a 23-year-old woman with refractory pruritus, which was initially attributed to primary sclerosing cholangitis but had a nonresponse to antihistaminics, ursodiol, and cholestyramine...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27909880/il-9-producing-cells-in-the-development-of-ige-mediated-food-allergy
#17
REVIEW
Dana Shik, Sunil Tomar, Jee-Boong Lee, Chun-Yu Chen, Andrew Smith, Yui-Hsi Wang
Food allergy is a harmful immune reaction driven by uncontrolled type 2 immune responses. Considerable evidence demonstrates the key roles of mast cells, IgE, and TH2 cytokines in mediating food allergy. However, this evidence provides limited insight into why only some, rather than all, food allergic individuals are prone to develop life-threatening anaphylaxis. Clinical observations suggest that patients sensitized to food through the skin early in life may later develop severe food allergies. Aberrant epidermal thymic stromal lymphopoietin and interleukin (IL) 33 production and genetic predisposition can initiate an allergic immune response mediated by dendritic cells and CD4(+)TH2 cells in inflamed skin...
January 2017: Seminars in Immunopathology
https://www.readbyqxmd.com/read/27909577/advances-in-the-understanding-and-clinical-management-of-mastocytosis-and-clonal-mast-cell-activation-syndromes
#18
REVIEW
David González-de-Olano, Almudena Matito, Alberto Orfao, Luis Escribano
Clonal mast cell activation syndromes and indolent systemic mastocytosis without skin involvement are two emerging entities that sometimes might be clinically difficult to distinguish, and they involve a great challenge for the physician from both a diagnostic and a therapeutic point of view. Furthermore, final diagnosis of both entities requires a bone marrow study; it is recommended that this be done in reference centers. In this article, we address the current consensus and guidelines for the suspicion, diagnosis, classification, treatment, and management of these two entities...
2016: F1000Research
https://www.readbyqxmd.com/read/27856463/ccl-2-is-a-kit-d816v-dependent-modulator-of-the-bone-marrow-microenvironment-in-systemic-mastocytosis
#19
Georg Greiner, Nadine Witzeneder, Angelika Berger, Klaus Schmetterer, Gregor Eisenwort, Ana-Iris Schiefer, Simone Roos, Theresia Popow-Kraupp, Leonhard Müllauer, Johannes Zuber, Veronika Sexl, Lukas Kenner, Wolfgang R Sperr, Peter Valent, Matthias Mayerhofer, Gregor Hoermann
Systemic mastocytosis (SM) is characterized by abnormal accumulation of neoplastic mast cells harboring the activating KIT mutation D816V in the bone marrow and other internal organs. Similar to other myeloproliferative neoplasms, increased production of pro-fibrogenic and angiogenic cytokines and related alterations of the bone marrow microenvironment are commonly found in SM. However, only little is known about mechanisms and effector molecules triggering fibrosis and angiogenesis in SM. Here we show that KIT D816V promotes expression of the pro-angiogenic cytokine CCL-2 in neoplastic mast cells...
November 16, 2016: Blood
https://www.readbyqxmd.com/read/27833446/transient-hypogammaglobulinaemia-of-infants-in-children-with-mastocytosis-strengthened-indications-for-vaccinations
#20
Joanna Renke, Magdalena Lange, Joanna Dawicka, Elżbieta Adamkiewicz-Drożyńska
Mastocytosis is a disease caused by the accumulation of mast cells (MC) in the skin and/or in other tissues. Both the cutaneous form of the disease (CM) predominating in children and the systemic form (SM) typical for adults are associated with the occurrence of MC mediator-related symptoms. The release of mediators can be induced by physical stimuli and/or specific triggering factors. The routine vaccination program performed in the majority of children in infancy can be considered as an additional factor provoking exacerbation of CM...
2016: Central-European Journal of Immunology
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