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https://www.readbyqxmd.com/read/29451318/effective-management-of-severe-cutaneous-mastocytosis-in-young-children-with-omalizumab-xolair-%C3%A2
#1
J D M Hughes, T Olynyc, H Chapdelaine, L Segal, B Miedzybrodzki, M Ben-Shoshan
Omalizumab (Xolair ® ) is an anti-IgE monoclonal antibody, which may benefit adults with systemic mastocytosis. We report effective treatment with omalizumab in two toddlers with severe diffuse cutaneous mastocytosis. Our cases offer preliminary evidence to support the safe use of omalizumab in paediatric patients with cutaneous mastocytosis.
February 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29439183/the-kit-and-pdgfra-switch-control-inhibitor-dcc-2618-blocks-growth-and-survival-of-multiple-neoplastic-cell-types-in-advanced-mastocytosis
#2
Mathias Schneeweiss, Barbara Peter, Siham Bibi, Gregor Eisenwort, Dubravka Smiljkovic, Katharina Blatt, Mohamad Jawhar, Daniela Berger, Gabriele Stefanzl, Susanne Herndlhofer, Georg Greiner, Gregor Hoermann, Emir Hadzijusufovic, Karoline V Gleixner, Peter Bettelheim, Klaus Geissler, Wolfgang R Sperr, Andreas Reiter, Michel Arock, Peter Valent
Systemic mastocytosis is a complex disease defined by abnormal growth and accumulation of neoplastic mast cells in various organs. Most patients exhibit a D816V-mutated variant of KIT, which confers resistance against imatinib. Clinical problems in systemic mastocytosis arise from mediator-related symptoms and/or organ destruction caused by malignant expansion of neoplastic mast cells and/or other myeloid cells in various organ systems. DCC-2618 is a spectrum-selective pan KIT and PDGFRA inhibitor which blocks KIT D816V and multiple other kinase-targets relevant to systemic mastocytosis...
February 8, 2018: Haematologica
https://www.readbyqxmd.com/read/29437608/a-foamy-mastocytosis-case
#3
Rathana Kim, Ludovic Lhermitte
No abstract text is available yet for this article.
February 1, 2018: Blood
https://www.readbyqxmd.com/read/29435387/systemic-mastocytosis-the-difficult-patient-with-a-rare-disease-case-presentation-and-brief-review
#4
Daniel H Desmond, Mark G Carmichael
Mastocytosis is a rare process involving the activation and accumulation of clonal mast cells categorized by cutaneous or systemic involvement. Although the diagnosis of cutaneous disease can be straightforward and confirmed via skin biopsy, systemic disease mimics more common disease processes making diagnosis a challenge. The widespread physiologic distribution of mast cells causes a variety of symptoms with aberrant expression including fatigue, headache, depression, dyspnea, dyspepsia, nausea, and abdominal pain...
February 2018: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/29431216/mast-cell-neural-interactions-contribute-to-pain-and-itch
#5
REVIEW
Kalpna Gupta, Ilkka T Harvima
Mast cells are best recognized for their role in allergy and anaphylaxis, but increasing evidence supports their role in neurogenic inflammation leading to pain and itch. Mast cells act as a "power house" by releasing algogenic and pruritogenic mediators, which initiate a reciprocal communication with specific nociceptors on sensory nerve fibers. Consequently, nerve fibers release inflammatory and vasoactive neuropeptides, which in turn activate mast cells in a feedback mechanism, thus promoting a vicious cycle of mast cell and nociceptor activation leading to neurogenic inflammation and pain/pruritus...
March 2018: Immunological Reviews
https://www.readbyqxmd.com/read/29426921/the-2016-who-classification-and-diagnostic-criteria-for-myeloproliferative-neoplasms-document-summary-and-in-depth-discussion
#6
REVIEW
Tiziano Barbui, Jürgen Thiele, Heinz Gisslinger, Hans Michael Kvasnicka, Alessandro M Vannucchi, Paola Guglielmelli, Attilio Orazi, Ayalew Tefferi
The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category...
February 9, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29405310/development-and-validation-of-the-mastocytosis-activity-score-mas
#7
Frank Siebenhaar, Benno Sander, Huyen Tram, André Ellrich, Marcus Maurer, Karsten Weller
BACKGROUND: Mastocytosis is a heterogeneous disease characterized by a clonal expansion of mast cells in various organs. The vast majority of patients suffer from signs and symptoms caused by mediator release from mast cells. Although the disease burden is high, there is currently no specific and validated instrument to measure and monitor signs and symptoms in patients with mastocytosis. OBJECTIVE: To develop and validate a disease-specific tool to measure and monitor the activity of signs and symptoms in patients with mastocytosis, the Mastocytosis Activity Score (MAS)...
February 6, 2018: Allergy
https://www.readbyqxmd.com/read/29378725/variability-of-pd-l1-expression-in-mastocytosis
#8
Ellen W Hatch, Mary Beth Geeze, Cheyenne Martin, Mohamed E Salama, Karin Hartmann, Gregor Eisenwort, Katharina Blatt, Peter Valent, Jason Gotlib, Ji-Hyun Lee, Lu Chen, Heather H Ward, Diane S Lidke, Tracy I George
Mastocytosis is a rare disease with heterogeneous clinical manifestations and few effective therapies. Programmed death-1 (PD-1) and its ligands (PD-L1 and PD-L2) protect tissues from immune-mediated damage and permit tumors to evade immune destruction. Therapeutic antibodies against PD-1 and PD-L1 are effective in the treatment of a variety of neoplasms. In the present study, we sought to systematically analyze expression of PD-1 and PD-L1 in a large number of patients with mastocytosis using immunohistochemistry and multiplex fluorescence staining...
February 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29376426/-systemic-mastocytosis-with-progressive-disease-course
#9
Judit Várkonyi, Gergely Szombath, Anna Vályi-Nagy, Judit Csomor, Krisztina Egedi, Ilona Kovalszky, Katalin Tölgyesi, László Szerafin, László Tóth, Györgyi Soós, Tamás Masszi
Authors report on a case of a male patient of systemic mastocytosis that was associated with extensive cutaneous lesions. Chronic diarrhoea worsening his quality of life was well managed by the administration of antihistamines. The pleural fluid recurrence soon after drainage has been controlled by the administration of alpha interferon. 40 years after the onset of the first skin signs progression has been manifested in the development of "B" (bone marrow infiltration rate >30%, dysmyelopoiesis, serum tryptase >20 μg/L, hepato- and splenomegaly) and "C" symptoms (liver function test abnormalities, cytopenia, malabsorption, osteoporosis)...
February 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/29350409/comparison-of-lesional-skin-c-kit-mutations-with-clinical-phenotype-in-patients-with-mastocytosis
#10
I J Chan, M D Tharp
BACKGROUND: Activating c-KIT mutations cause abnormal mast cell growth and appear to play a role in mastocytosis. However, the correlation of c-KIT mutations with disease phenotypes is poorly characterized. AIM: To evaluate the correlation of c-KIT mutations with clinical presentations and laboratory findings. METHODS: Total cellular RNA was isolated from the skin lesions of 43 adults and 7 children with mastocytosis, and PCR amplicons of cDNA were sequenced for c-KIT mutations...
January 19, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29343975/spotlight-on-midostaurin-in-the-treatment-of-flt3-mutated-acute-myeloid-leukemia-and-systemic-mastocytosis-design-development-and-potential-place-in-therapy
#11
REVIEW
Ellen Weisberg, Martin Sattler, Paul W Manley, James D Griffin
The Fms-like tyrosine kinase-3 (FLT3; fetal liver kinase-2; human stem cell tyrosine kinase-1; CD135) is a class III receptor tyrosine kinase that is normally involved in regulating the proliferation, differentiation, and survival of both hematopoietic cells and dendritic cells. Mutations leading it to be constitutively activated make it an oncogenic driver in ~30% of acute myeloid leukemia (AML) patients where it is associated with poor prognosis. The prevalence of oncogenic FLT3 and the dependency on its constitutively activated kinase activity for leukemia growth make this protein an attractive target for therapeutic intervention...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29341334/incidence-and-prognostic-impact-of-cytogenetic-aberrations-in-patients-with-systemic-mastocytosis
#12
Nicole Naumann, Mohamad Jawhar, Juliana Schwaab, Sebastian Kluger, Johannes Lübke, Georgia Metzgeroth, Henning D Popp, Nada Khaled, Hans-Peter Horny, Karl Sotlar, Peter Valent, Claudia Haferlach, Gudrun Göhring, Brigitte Schlegelberger, Manja Meggendorfer, Wolf-Karsten Hofmann, Nicholas C P Cross, Andreas Reiter, Alice Fabarius
The clinical behavior of systemic mastocytosis (SM) is strongly associated with activating mutations in KIT (D816V in >80% of cases), with the severity of the phenotype influenced by additional somatic mutations, e.g. in SRSF2, ASXL1 or RUNX1. Complex molecular profiles are frequently associated with the presence of an associated hematologic neoplasm (AHN) and an unfavorable clinical outcome. However, little is known about the incidence and prognostic impact of cytogenetic aberrations. We analyzed cytogenetic and molecular characteristics of 109 patients (KIT D816V+, n=102, 94%) with indolent (ISM, n=26) and advanced SM (n=83) with (n=73, 88%) or without AHN...
January 17, 2018: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29331029/characterization-of-cd34-hematopoietic-cells-in-systemic-mastocytosis-potential-role-in-disease-dissemination
#13
A Mayado, C Teodosio, N Dasilva-Freire, M Jara-Acevedo, A C Garcia-Montero, I Álvarez-Twose, L Sánchez-Muñoz, A Matito, C Caldas, J I Muñoz-González, A Henriques, J I Sánchez-Gallego, L Escribano, A Orfao
BACKGROUND: Recent studies show that most systemic mastocytosis (SM) patients, including indolent SM (ISM) with (ISMs+) and without skin lesions (ISMs-), carry the KIT D816V mutation in PB leukocytes. We investigated the potential association between the degree of involvement of BM hematopoiesis by the KIT D816V mutation and the distribution of different maturation-associated compartments of bone marrow (BM) and peripheral blood (PB) CD34 + hematopoietic precursors (HPC) in ISM and identified the specific PB cell compartments that carry this mutation...
January 13, 2018: Allergy
https://www.readbyqxmd.com/read/29318637/factors-increasing-the-risk-for-a-severe-reaction-in-anaphylaxis-an-analysis-of-data-from-the-european-anaphylaxis-registry
#14
Margitta Worm, Wojciech Francuzik, Jean-Marie Renaudin, Maria Beatrice Bilo, Victòria Cardona, Kathrin Scherer Hofmeier, Alice Köhli, Andrea Bauer, George Christoff, Ewa Cichocka-Jarosz, Thomas Hawranek, Jonathan O'B Hourihane, Lars Lange, Vera Mahler, Antonella Muraro, Nikolaos G Papadopoulos, Claudia Pföhler, Iwona Poziomkowska-Gęsicka, Franziska Ruëff, Thomas Spindler, Regina Treudler, Montserrat Fernandez-Rivas, Sabine Dölle
BACKGROUND: Preventive measures to decrease the frequency and intensity of anaphylactic events are essential to provide optimal care for allergic patients. Aggravating factors may trigger or increase the severity of anaphylaxis and therefore need to be recognized and avoided. OBJECTIVE: To identify and prioritize factors associated with an increased risk of developing severe anaphylaxis. METHODS: Data from the Anaphylaxis Registry (122 centers in 11 European countries) was used in logistic regression models considering existing severity grading systems, elicitors, and symptoms to identify the relative risk of factors on the severity of anaphylaxis...
January 10, 2018: Allergy
https://www.readbyqxmd.com/read/29314691/mastocytosis-pathogenesis-clinical-manifestation-and-treatment
#15
Nicola Wagner, Petra Staubach
The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators. Dermatologists/allergists are frequently the first to establish the diagnosis. The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy...
January 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/29296647/clinical-and-molecular-categorization-of-progressive-adult-onset-cutaneous-mastocytosis
#16
Courtney Tate, Fiona Tang, Steven W Lane, Louise Seymour
No abstract text is available yet for this article.
January 2018: JAAD Case Reports
https://www.readbyqxmd.com/read/29296203/the-pathogenesis-of-diclofenac-induced-immunoallergic-hepatitis-in-a-canine-model-of-liver-injury
#17
Saravanakumar Selvaraj, Jung-Hwa Oh, Reinhard Spanel, Florian Länger, Hyoung-Yun Han, Eun-Hee Lee, Seokjoo Yoon, Jürgen Borlak
Hypersensitivity to non-steroidal anti-inflammatory drugs is a common adverse drug reaction and may result in serious inflammatory reactions of the liver. To investigate mechanism of immunoallergic hepatitis beagle dogs were given 1 or 3 mg/kg/day (HD) oral diclofenac for 28 days. HD diclofenac treatment caused liver function test abnormalities, reduced haematocrit and haemoglobin but induced reticulocyte, WBC, platelet, neutrophil and eosinophil counts. Histopathology evidenced hepatic steatosis and glycogen depletion, apoptosis, acute lobular hepatitis, granulomas and mastocytosis...
December 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/29288729/extracellular-vesicles-or-free-circulating-dna-where-to-search-for-braf-and-ckit-mutations
#18
Jennifer Klump, Ulrike Philipp, Marie Follo, Anna Eremin, Hannes Lehmann, Sigrun Nestel, Nikolas von Bubnoff, Irina Nazarenko
Clinical evidence in oncology argues for the advantages of performing molecular analysis of blood biomarkers to provide information about systemic changes and tumor heterogeneity. Whereas the diagnostic value of cell-free circulating DNA (fcDNA) has successfully been demonstrated in several studies, DNA enclosed in extracellular vesicles (EV) has only recently been described, and its potential diagnostic value is unclear. We established a protocol for separation of EV and fc fractions and tested for presence of mutant BRAFV600E mediating resistance to Vemurafenib and cKITD816V mediating resistance to Imatinib in blood of patients with melanoma and mastocytosis...
December 27, 2017: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/29277359/soho-state-of-the-art-update-and-next-questions-mpn
#19
REVIEW
Prithviraj Bose, Jason Gotlib, Claire N Harrison, Srdan Verstovsek
The discovery of the activating Janus kinase (JAK)2V617F mutation in 2005 in most patients with the classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) spurred intense interest in research into these disorders, culminating in the identification of activating mutations in MPL in 2006 and indels in the gene encoding calreticulin (CALR) in 2013, thus providing additional mechanistic explanations for the universal activation of JAK-signal transducer and activator of transcription (JAK-STAT) observed in these conditions, and the success of the JAK1/2 inhibitor ruxolitinib, which first received regulatory approval in 2011...
January 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29258788/anaphylactic-reactions-after-discontinuation-of-hymenoptera-venom-immunotherapy-a-clonal-mast-cell-disorder-should-be-suspected
#20
Patrizia Bonadonna, Roberta Zanotti, Mauro Pagani, Massimiliano Bonifacio, Luigi Scaffidi, Elisa Olivieri, Maurizio Franchini, Federico Reccardini, Maria Teresa Costantino, Chiara Roncallo, Marina Mauro, Elisa Boni, Fabio Lodi Rizzini, Maria Beatrice Bilò, Anna Rosaria Marcarelli, Giovanni Passalacqua
BACKGROUND: Up to 75% of patients with severe anaphylactic reactions after Hymenoptera sting are at risk of further severe reactions if re-stung. Venom immunotherapy (VIT) is highly effective in protecting individuals with ascertained Hymenoptera venom allergy (HVA) and previous severe reactions. After a 3- to 5-year VIT course, most patients remain protected after VIT discontinuation. Otherwise, a lifelong treatment should be considered in high-risk patients (eg, in mastocytosis). Several case reports evidenced that patients with mastocytosis and HVA, although protected during VIT, can re-experience severe and sometimes fatal reactions after VIT discontinuation...
December 16, 2017: Journal of Allergy and Clinical Immunology in Practice
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