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https://www.readbyqxmd.com/read/27909880/il-9-producing-cells-in-the-development-of-ige-mediated-food-allergy
#1
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...
December 1, 2016: Seminars in Immunopathology
https://www.readbyqxmd.com/read/27909577/advances-in-the-understanding-and-clinical-management-of-mastocytosis-and-clonal-mast-cell-activation-syndromes
#2
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/27886904/mastocytosis-and-anaphylaxis
#3
REVIEW
Anna Schuch, Knut Brockow
This article updates current knowledge on epidemiology, risk factors, triggers, and management of anaphylaxis in patients with mastocytosis. Hyperactive mast cells and higher number of effector mast cells are speculated to facilitate anaphylaxis in this condition. In children, increased risk is limited to those with extensive skin involvement and high tryptase. In adults, manifestations of anaphylaxis are severe with high frequency of cardiovascular symptoms. Hymenoptera stings are the most common triggers for these reactions; however, idiopathic anaphylaxis and reactions to food or drugs occur...
February 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/27856463/ccl-2-is-a-kit-d816v-dependent-modulator-of-the-bone-marrow-microenvironment-in-systemic-mastocytosis
#4
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/27853507/developing-food-allergy-a-potential-immunologic-pathway-linking-skin-barrier-to-gut
#5
REVIEW
Yui-Hsi Wang
Immunoglobulin E (IgE)-mediated food allergy is an adverse reaction to foods and is driven by uncontrolled type-2 immune responses. Current knowledge cannot explain why only some individuals among those with food allergy are prone to develop life-threatening anaphylaxis. It is increasingly evident that the immunologic mechanisms involved in developing IgE-mediated food allergy are far more complex than allergic sensitization. Clinical observations suggest that patients who develop severe allergic reactions to food are often sensitized through the skin in early infancy...
2016: F1000Research
https://www.readbyqxmd.com/read/27846948/exploring-the-prevalence-of-learning-disabilities-in-children-with-cutaneous-mastocytosis-a-pilot-cohort-study
#6
Alexandra Seamens, Brian Taussig, Katherine Penziner, Aimee Smidt, Leslie P Lawley
No abstract text is available yet for this article.
December 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27843665/a-dog-is-a-doctor-s-best-friend-the-use-of-a-service-dog-as-a-perioperative-assistant
#7
Shannon Tew, Brad M Taicher
Service dogs are beneficial in providing assistance to people with multiple types of disabilities and medical disorders including visual impairment, physical disabilities, seizure disorders, diabetes, and mental illness. Some service animals have been trained as a screening tool for cancer. We review a case involving a 6-year-old female with a history of mast cell mediator release and immediate hypersensitivity due to the urticaria pigmentosa variant of cutaneous mastocytosis who underwent a cystourethroscopy...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27833446/transient-hypogammaglobulinaemia-of-infants-in-children-with-mastocytosis-strengthened-indications-for-vaccinations
#8
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
https://www.readbyqxmd.com/read/27822310/anaphylactic-shock-during-cement-implantation-of-a-total-hip-arthroplasty-in-a-patient-with-underlying-mastocytosis-case-report-of-a-rare-intraoperative-complication
#9
Anita Ten Hagen, Pieter Doldersum, Tom van Raaij
BACKGROUND: Cemented total hip arthroplasty (THA) is a safe and common procedure. In rare cases life threatening bone cement implantation syndrome (BCIS) may occur, which is commonly caused by pulmonary embolism (PE). CASE PRESENTATION: We describe the rare case of a 70-year old patient who underwent an elective total hip replacement. Before surgery he was diagnosed with underlying systemic indolent mastocytosis, a rare pathological disorder that may result in anaphylaxis after massive systemic mast cell activation...
2016: Patient Safety in Surgery
https://www.readbyqxmd.com/read/27808598/anaplastic-mast-cell-sarcoma-a-unique-pathologic-entity-in-mastocytosis
#10
Fiona He, Hans-Peter Horny, Jamie Boone, Ahmad Raza, Megan Griffith, Peter Hurley, Michelle Dolan, Zuzan Cayci, Michael A Linden, Robert McKenna, Celalettin Ustun
No abstract text is available yet for this article.
November 3, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27807556/ileal-pouch-biopsy-triggers-investigation-and-diagnosis-of-systemic-mastocytosis
#11
Abul Ala Syed Rifat Mannan, Bo Shen, Fred Hsieh, Deepa T Patil
We report a unique case of systemic mastocytosis (SM) diagnosed in an ileal pouch biopsy obtained from a 44-year-old woman with ulcerative colitis. She presented with intermittent abdominal pain and watery diarrhea that did not respond to antibiotic therapy. The pouch biopsy showed expansion of the lamina propria by aggregates of CD117 and CD25-positive abnormal mast cells. A subsequent bone marrow analysis showed an increase in abnormal mast cells. Based on World Health Organization criteria, she was diagnosed with SM and responded to cromolyn sodium therapy...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27787922/bone-health-in-patients-with-hematopoietic-disorders-of-bone-marrow-origin-systematic-review-and-meta-analysis
#12
Kieran Steer, Mariya Stavnichuk, Martin Morris, Svetlana V Komarova
Blood cell production and bone homeostasis are physically interlinked systems that exhibit active cross-talk. We examined how bone health is affected in patients with hematopoietic disorders due to abnormal proliferation of bone marrow cells. The electronic databases Medline, Embase, PubMed, BIOSIS Previews, Web of Science, and Cochrane were searched for studies presenting numerical values for trabecular bone volume or bone mineral density in control and patients with hematopoietic disorders. We identified 5 studies for beta-thalassemia, 6 for sickle cell anemia, 2 for polycythemia vera and essential thrombocythemia, 3 for chronic myelogenous leukemia, 6 for myelofibrosis, 5 for multiple myeloma, and 4 studies each for systemic mastocytosis, lymphocytic leukemia, and hemochromatosis...
October 27, 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27783996/a-new-humanized-in-vivo-model-of-kit-d816v-advanced-systemic-mastocytosis-monitored-using-a-secreted-luciferase
#13
Siham Bibi, Yanyan Zhang, Caroline Hugonin, Mallorie Depond Mangean, Liang He, Ghaith Wedeh, Jean-Marie Launay, Sjoerd Van Rijn, Thomas Würdinger, Fawzia Louache, Michel Arock
Systemic mastocytosis are rare neoplasms characterized by accumulation of mast cells in at least one internal organ. The majority of systemic mastocytosis patients carry KIT D816V mutation, which activates constitutively the KIT receptor. Patient with advanced forms of systemic mastocytosis, such as aggressive systemic mastocytosis or mast cell leukemia, are poorly treated to date. Unfortunately, the lack of in vivo models reflecting KIT D816V+ advanced disease hampers pathophysiological studies and preclinical development of new therapies for such patients...
October 22, 2016: Oncotarget
https://www.readbyqxmd.com/read/27781377/a-variant-c-kit-mutation-d816h-fundamental-to-the-sequential-development-of-an-ovarian-mixed-germ-cell-tumor-and-systemic-mastocytosis-with-cmml
#14
Sarah G Mitchell, Silvia T Bunting, Debra Saxe, Thomas Olson, Frank G Keller
An activating point mutation of the c-KIT tyrosine kinase receptor gene, D816H, has been described in germ cell tumors (GCTs). We report an adolescent diagnosed with an ovarian mixed GCT and systemic mastocytosis with chronic myelomonocytic leukemia (SM-CMML). The teratoma and dysgerminoma differed by copy number aberrations via single nucleotide polymorphism (SNP) microarray, but were inclusive of the same c-KIT D816H point mutation (c.2446G>C) also identified in blood and bone marrow mast cells. These findings indicate not only a clonal origin of the GCT and hematologic malignancy, but also suggest a rare KIT mutation may be playing a fundamental role in malignancy development...
October 26, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27779084/mast-cell-disorders-in-drug-hypersensitivity
#15
Patrizia Bonadonna, Massimiliano Bonifacio, Roberta Zanotti
Mastocytosis is a clonal disease characterized by proliferation and accumulation of mast cells (MC) in different tissues, preferentially skin and bone marrow, leading to a wide variety of clinical manifestations, mainly caused by the inappropriate release of MC mediators. As a consequence, patients with mastocytosis may experience symptoms due to massive MC activation and release of mediators. Anaphylaxis is the most frequent manifestation of this phenomenon.Drugs are possible triggers of anaphylaxis in patients with mastocytosis, even though the association between mastocytosis and drug anaphylaxis does not appear to be as strong as anaphylaxis after hymenoptera sting; nevertheless, MC disorders might be ruled out in cases of severe systemic reactions to drugs...
September 28, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27777718/germline-mutations-of-kit-in-gastrointestinal-stromal-tumor-gist-and-mastocytosis
#16
Hengning Ke, Julhash U Kazi, Hui Zhao, Jianmin Sun
Somatic mutations of KIT are frequently found in mastocytosis and gastrointestinal stromal tumor (GIST), while germline mutations of KIT are rare, and only found in few cases of familial GIST and mastocytosis. Although ligand-independent activation is the common feature of KIT mutations, the phenotypes mediated by various germline KIT mutations are different. Germline KIT mutations affect different tissues such as interstitial cells of Cajal (ICC), mast cells or melanocytes, and thereby lead to GIST, mastocytosis, or abnormal pigmentation...
2016: Cell & Bioscience
https://www.readbyqxmd.com/read/27776809/-metameric-macular-and-papular-skin-mastocytosis
#17
M Maciejewski, J-M Debarre, S Georgin-Lavialle, S Kettani, S Olschwang, M Guérin-Moreau, Y Le Corre, L Martin
BACKGROUND: Mastocytosis is characterised by the presence of abnormal quantities of mastocytes in one or more organs. Although it occurs in systemic forms of mastocytosis, isolated skin involvement is the predominant presentation, particularly in children, in the form of more or less extensive though non-systematic lesions. Herein, we report a case of maculopapular cutaneous mastocytosis that is unusual in terms of its metameric topography. PATIENTS AND METHODS: A 16-year-old youth presented with an erythematous maculopapular rash of 18 months' duration and involving pruritic inflammatory episodes strictly localised in segment T8 to the left...
October 21, 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/27772953/a-case-of-systemic-mastocytosis-diagnosed-on-colonic-biopsy
#18
Rushmi Sooriyabandara, Peter Crowley, Afaf Haddad
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27762455/systemic-mastocytosis-in-adults-2017-update-on-diagnosis-risk-stratification-and-management
#19
Animesh Pardanani
: Disease overview:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs. DIAGNOSIS: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V. Risk stratification: The 2008 World Health Organization (WHO) classification of SM has been shown to be prognostically relevant...
November 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27759482/dermatologic-uses-of-omalizumab
#20
Justin C Chia, P Régine Mydlarski
PURPOSE: Omalizumab is a recombinant humanized monoclonal antibody that inhibits the binding of immunoglobulin E (IgE) to the high-affinity IgE receptor (FceRI) on the surface of mast cells and basophils. Omalizumab has been approved for use in asthma, and new reports show promise in a variety of dermatologic diseases. Herein, we review the literature on omalizumab in dermatology and discuss the safety, efficacy and mechanisms of action for this emerging therapy. MATERIALS AND METHODS: PubMED, MEDLINE and Embase databases were searched for the period 1 January 1990 to 1 September 2016...
November 7, 2016: Journal of Dermatological Treatment
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