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Systemic mastocytosis

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https://www.readbyqxmd.com/read/29223146/investigation-of-mast-cell-toll-like-receptor-3-in-chronic-fatigue-syndrome-myalgic-encephalomyelitis-and-systemic-mastocytosis-participants-using-the-novel-application-of-automacs-magnetic-separation-and-flow-cytometry
#1
Cassandra Balinas, Thao Nguyen, Samantha Johnston, Peter Smith, Donald Staines, Sonya Marshall-Gradisnik
BACKGROUND: Viral infections and hypersensitivities are commonly reported by Chronic Fatigue Syndrome/Myalgic Encephalomyelitis (CFS/ME) patients. Mast Cells (MC) uniquely mediate type 1 hypersensitivities and resolve viral infections via toll-like receptor 3 (TLR3). OBJECTIVE: To characterise and compare mast cell progenitors (MCPs) in CFS/ME participants with a known MC disorder, Systemic mastocytosis (SM), and secondly, to investigate the role of MC TLR3 in CFS/ME participants following Polyinosinic:polycytidylic acid (Poly I:C) stimulation...
December 10, 2017: Asian Pacific Journal of Allergy and Immunology
https://www.readbyqxmd.com/read/29212100/lower-prevalence-of-vitamin-d-insufficiency-in-german-patients-with-systemic-mast-cell-activation-syndrome-compared-with-the-general-population
#2
Stefan Wirz, Hans-Jörg Hertfelder, Holger Seidel, Jürgen Homann, Gerhard J Molderings
Introduction The common mastocytosis variant systemic mast cell activation syndrome (MCAS) may underlie at least a subset of patients with irritable bowel syndrome (IBS). A critical role of vitamin D (VD) in the stabilization of mast cells (MCs) with deficiency of VD resulting in MC activation has been demonstrated. If so, supplementation of VD would be a potential therapeutic approach in the treatment of those IBS patients. Methods We investigated in the present study for the first time systematically whether the VD level in 100 MCAS patients differed from that in the German general population (Ggp) and made a first attempt to elucidate potential reasons for possible differences by simultaneously determining the blood levels of heparin and cholesterol...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29202591/venom-immunotherapy-in-patients-with-allergic-reactions-to-insect-stings
#3
Cristoforo Incorvaia, Marina Mauro, Bruna L Gritti, Eleni Makri, Erminia Ridolo
Allergy to Hymenoptera (Apis mellifera, Vespula species, Polistes species, Vespa crabro) venom can be safely and effectively treated by venom immunotherapy (VIT), which in the 40 years since its introduction has been able to prevent reactions to stings, and to treatment as well, though systemic reactions, occasionally severe, are possible. Areas covered: We reviewed the recent literature on VIT by searching in PubMed for the terms "venom immunotherapy" and "Hymenoptera venom immunotherapy" to highlight the current status of VIT and the likely development in the coming years...
December 5, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29196283/ringed-telangiectasias-an-unusual-presentation-of-telangiectasia-macularis-eruptiva-perstans
#4
Laila Siddique, Hadjh Ahrns, Elizabeth Seiverling
Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis...
November 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29192973/neonatal-aggressive-systemic-mastocytosis
#5
J E Mellerio
No abstract text is available yet for this article.
November 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29162274/maxillar-osteonecrosis-associated-to-denosumab-in-a-patient-with-systemic-mastocytosis
#6
José Darío Sánchez López, Paolo Cariati, Miguel Pérez de Perceval Tara
No abstract text is available yet for this article.
November 18, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29161766/identification-of-alpha-gal-sensitivity-in-patients-with-a-diagnosis-of-idiopathic-anaphylaxis
#7
Melody C Carter, Karina N Ruiz-Esteves, Lisa Workman, Philip Lieberman, Thomas A E Platts-Mills, Dean D Metcalfe
IgE antibodies (Ab) specific to galactose-α-1,3-galactose (alpha-gal) are responsible for a delayed form of anaphylaxis that occurs 3 to 6 hours after red meat ingestion. In a unique prospective study of seventy participants referred with a diagnosis of idiopathic anaphylaxis (IA), six (9%) were found to have IgE to alpha-gal. Upon institution of a diet free of red meat, all patients had no further episodes of anaphylaxis. Two of these individuals had indolent systemic mastocytosis (ISM). Those with ISM had more severe clinical reactions but lower specific IgE to alpha-gal and higher serum tryptase levels, reflective of the mast cell burden...
November 21, 2017: Allergy
https://www.readbyqxmd.com/read/29137311/crenolanib-is-a-type-i-tyrosine-kinase-inhibitor-that-inhibits-mutant-kit-d816-isoforms-prevalent-in-systemic-mastocytosis-and-core-binding-factor-leukemia
#8
Kerstin Maria Kampa-Schittenhelm, Julia Frey, Lara A Haeusser, Barbara Illing, Ashly A Pavlovsky, Gunnar Blumenstock, Marcus Matthias Schittenhelm
Activating D816 mutations of the class III receptor tyrosine kinase KIT are associated with the majority of patients with systemic mastocytosis (SM), but also core binding factor (CBF) AML, making KIT mutations attractive therapeutic targets for the treatment of these cancers. Crenolanib is a potent and selective inhibitor of wild-type as well as mutant isoforms of the class III receptor tyrosine kinases FLT3 and PDGFRα/β. Notably, crenolanib inhibits constitutively active mutant-FLT3 isoforms resulting from amino acid substitutions of aspartic acid at codon 835, which is homologous to codon 816 in the KIT gene - suggesting sensitivity against mutant-KIT D816 isoforms as well...
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29117990/simultaneous-physiologically-based-pharmacokinetic-pbpk-modeling-of-parent-and-active-metabolites-to-investigate-complex-cyp3a4-drug-drug-interaction-potential-a-case-example-of-midostaurin
#9
Helen Gu, Catherine Dutreix, Sam Rebello, Taoufik Ouatas, Lai Wang, Dung Yu Chun, Heidi J Einolf, Handan He
Midostaurin (PKC412) is being investigated for the treatment of acute myeloid leukemia (AML) and advanced systemic mastocytosis (advSM). It is extensively metabolized by cytochrome P450 (CYP) 3A4 to form 2 major active metabolites, CGP52421 and CGP62221. In vitro and clinical drug-drug interaction (DDI) studies indicated that midostaurin and its metabolites are substrates, reversible and time-dependent inhibitors, and inducers of CYP3A4. A simultaneous pharmacokinetic model of parent and active metabolites was initially developed by incorporating data from in vitro, preclinical, and clinical pharmacokinetic studies in healthy volunteers and in patients with AML or advSM...
November 8, 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/29107667/fgfr1-translocation-with-concurrent-myeloproliferative-neoplasm-systemic-mastocytosis-and-lymphoblastic-lymphoma-a-case-report
#10
Koping Chang, Jia-Hau Liu, Shan-Chi Yu, Chung-Wu Lin
FGFR1 translocation may cause myeloid or lymphoid neoplasm but rarely systemic mastocytosis (SM). Conversely, SM is associated with myeloproliferative neoplasm (MPN), but rarely lymphoblastic lymphoma (LBL) or FGFR1 translocation. We report the first case of FGFR1 translocation in a patient with concurrent LBL, MPN, and SM. A 21-year-old male patient presented with diffuse lymphadenopathies and leukocytosis. TdT(+)/cytoCD3(+)/CD79a(weakly+) LBL was identified in the lymph node. Bone marrow had MPN, SM, and TdT(+)/CD79a(+)/cytoCD3(weakly+) LBL...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29093181/a-precision-therapy-against-cancers-driven-by-kit-pdgfra-mutations
#11
Erica K Evans, Alexandra K Gardino, Joseph L Kim, Brian L Hodous, Adam Shutes, Alison Davis, Xing Julia Zhu, Oleg Schmidt-Kittler, Doug Wilson, Kevin Wilson, Lucian DiPietro, Yulian Zhang, Natasja Brooijmans, Timothy P LaBranche, Agnieszka Wozniak, Yemarshet K Gebreyohannes, Patrick Schöffski, Michael C Heinrich, Daniel J DeAngelo, Stephen Miller, Beni Wolf, Nancy Kohl, Timothy Guzi, Nicholas Lydon, Andy Boral, Christoph Lengauer
Targeting oncogenic kinase drivers with small-molecule inhibitors can have marked therapeutic benefit, especially when administered to an appropriate genomically defined patient population. Cancer genomics and mechanistic studies have revealed that heterogeneous mutations within a single kinase can result in various mechanisms of kinase activation. Therapeutic benefit to patients can best be optimized through an in-depth understanding of the disease-driving mutations combined with the ability to match these insights to tailored highly selective drugs...
November 1, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29055165/development-of-tripe-palms-and-soles-in-a-patient-with-long-pre-existing-systemic-mastocytosis-and-newly-developed-non-small-cell-lung-cancer
#12
Hanna Bonnekoh, Tatevik Ohanyan, Dido Lenze, Karoline Krause, Marcus Maurer, Torsten Zuberbier, Frank Siebenhaar
A 71-year old woman with systemic mastocytosis presented to our department with an apoplectic insult one year ago and a 100 pack year smoking history. Skin manifestation of mastocytosis had started at the age of 50 years accompanied by a substantial increase of serum tryptase since the age of 60. Bone marrow involvement was confirmed 16 years later. Therapy of mastocytosis included antihistamines, montelukast, sodium cromoglycate, and PUVA therapy, all with limited success. This article is protected by copyright...
October 21, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29051803/midostaurin-a-novel-therapeutic-agent-for-patients-with-flt3-mutated-acute-myeloid-leukemia-and-systemic-mastocytosis
#13
REVIEW
Molly M Gallogly, Hillard M Lazarus, Brenda W Cooper
The development of FLT3-targeted inhibitors represents an important paradigm shift in the management of patients with highly aggressive fms-like tyrosine kinase 3-mutated (FLT3-mut) acute myeloid leukemia (AML). Midostaurin is an orally administered type III tyrosine kinase inhibitor which in addition to FLT3 inhibits c-kit, platelet-derived growth factor receptors, src, and vascular endothelial growth factor receptor. Midostaurin is the first FLT3 inhibitor that has been shown to significantly improve survival in younger patients with FLT3-mut AML when given in combination with standard cytotoxic chemotherapy based on the recently completed RATIFY study...
September 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29048112/how-we-diagnose-and-treat-systemic-mastocytosis-in-adults
#14
REVIEW
Robyn M Scherber, Uma Borate
Rapid advances in the understanding of the molecular biology, data from translational and clinical trials, and retrospective analyses has influenced the diagnosis and treatment of systemic mastocytosis (SM). Many options have existed for the symptomatic management of SM patients, but recent evolution in regards to the molecular underpinnings of this disease and our ability to distinguish clonal mastocytosis from mast cell activation syndrome has changed our treatment paradigm and opened new opportunities for understanding genetic risk, transformation to mast cell leukaemia, and treatment choices...
October 19, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29043985/from-the-observation-of-atypical-cells-on-blood-smear-to-the-diagnosis-of-mast-cell-leukemia-a-case-report-in-a-79-year-old-woman-consulting-for-anemia
#15
Julien Decker, Sabine Meyer, Véronique Latger-Cannard, Sorin Visanica, Elena Loppinet, Jean-François Lesesve, Blandine Bénet
Mast cell leukemia is an extremely rare disease, which belongs to the systemic mastocytosis group (WHO 2016). We are reporting the case of a 79-year-old woman, without any hematological particular history consulting for hyperthermia, repeated malaise and subacute anemia. Her clinical examination was normal. Unusual cells were seen on blood and bone marrow smears. They represent more than 10% of blood nucleated cells end more than 20% of the bone marrow nucleated cells. Bone marrow immunophenotyping was performed to characterize these cells...
October 18, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/29025582/a-novel-trip11-flt3-fusion-in-a-patient-with-a-myeloid-lymphoid-neoplasm-with-eosinophilia
#16
Alfred Chung, Yanli Hou, Robert S Ohgami, Ann Von Gehr, Dianna G Fisk, Krishna M Roskin, Xu Li, Linda Gojenola, Charles D Bangs, Daniel A Arber, Andrew Z Fire, Athena M Cherry, James L Zehnder, Jason Gotlib, Jason D Merker
FLT3 fusions are associated with myeloid and lymphoid neoplasms with eosinophilia. We describe a patient presenting with clinicopathologic features of both chronic eosinophilic leukemia, not otherwise specified (CEL, NOS) and systemic mastocytosis (SM). The bone marrow demonstrated a myeloproliferative neoplasm with eosinophilia and aggregates of atypical mast cells. Cytogenetic analysis revealed a t(13;14)(q12;q32), which was subsequently molecularly characterized as a novel TRIP11-FLT3 rearrangement. A KIT D816V mutation was also identified...
October 2017: Cancer Genetics
https://www.readbyqxmd.com/read/28988349/mastocytosis-in-children
#17
REVIEW
Nicholas Klaiber, Santhosh Kumar, Anne-Marie Irani
PURPOSE OF REVIEW: In this review, we examine the current understanding of the pathogenesis, clinical presentations, diagnostic tools, and treatment options of pediatric mastocytosis as well as the natural history of the disease. RECENT FINDINGS: We discuss the emerging concept of mast cell activation syndrome. Mastocytosis in children presents most commonly as isolated cutaneous lesions and is a relatively rare occurrence with excellent prognosis and spontaneous regression often occurring by adolescence...
October 7, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28978170/imatinib-in-systemic-mastocytosis-a-phase-iv-clinical-trial-in-patients-lacking-exon-17-kit-mutations-and-review-of-the-literature
#18
Iván Alvarez-Twose, Almudena Matito, José Mário Morgado, Laura Sánchez-Muñoz, María Jara-Acevedo, Andrés García-Montero, Andrea Mayado, Carolina Caldas, Cristina Teodósio, Javier Ignacio Muñoz-González, Manuela Mollejo, Luis Escribano, Alberto Orfao
Resistance to imatinib has been recurrently reported in systemic mastocytosis (SM) carrying exon 17 KIT mutations. We evaluated the efficacy and safety of imatinib therapy in 10 adult SM patients lacking exon 17 KIT mutations, 9 of which fulfilled criteria for well-differentiated SM (WDSM). The World Health Organization 2008 disease categories among WDSM patients were mast cell (MC) leukemia (n = 3), indolent SM (n = 3) and cutaneous mastocytosis (n = 3); the remainder case had SM associated with a clonal haematological non-MC disease...
September 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28976442/general-anesthesia-for-electroconvulsive-therapy-in-a-patient-with-systemic-mastocytosis
#19
Ethan O Bryson, Amy S Aloysi, Kate G Farber, Charles H Kellner
No abstract text is available yet for this article.
October 3, 2017: Journal of ECT
https://www.readbyqxmd.com/read/28970467/systemic-mastocytosis-in-association-with-small-lymphocytic-lymphoma
#20
Muhammad F Iqbal, Paolo Marco K Soriano, Sanjai Nagendra, Sherjeel Sana
BACKGROUND Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare entity, and the majority of systemic mastocytosis cases are associated with myeloid neoplasm. Lymphoproliferative disorders are less commonly associated with systemic mastocytosis and a few cases of systemic mastocytosis associated with chronic lymphocytic leukemia have been described in the literature. CASE REPORT We present a case of indolent systemic mastocytosis associated with small lymphocytic lymphoma...
October 3, 2017: American Journal of Case Reports
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