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Mastocytosis

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https://www.readbyqxmd.com/read/29057738/chemotherapy-in-mastocytosis-administration-issues-hypersensitivity-and-rapid-drug-desensitization
#1
E Solano-Solares, R Madrigal-Burgaleta, L V Carpio-Escalona, L Bernal-Rubio, M P Berges-Gimeno, E Alvarez-Cuesta
No abstract text is available yet for this article.
2017: Journal of Investigational Allergology & Clinical Immunology
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
#2
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
#3
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/29050542/hepatic-mastocytosis-in-japanese-black-cattle
#4
Susumu Ohfuji
In 5 Japanese Black steers (2-2.4 years old) that originated from 5 different feedlots, the livers were found at slaughter to have multiple nodular or cordlike lesions (5 steers) and an extensive fibrotic area (1 steer). Microscopic changes included extensive fibroplasia in the portal tracts and chronic proliferative endophlebitis-like lesions confined to the portal vein branches. Fibroplasia was much more prominent in the macroscopic fibrotic lesion of 1 steer. Portal vein branches presented irregular variciform dilation of the vascular lumen and fibroplastic changes in the subendothelial areas that showed occasional hemorrhage and were simultaneously infiltrated with large numbers of mast cells and moderate to large numbers of eosinophils...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/29048112/how-we-diagnose-and-treat-systemic-mastocytosis-in-adults
#5
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
#6
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/29033257/chromogranin-a-is-not-a-biomarker-of-mastocytosis
#7
Pahul Hanjra, Chyi-Chia R Lee, Irina Maric, Melody Carter, Ana Olivera, Dean D Metcalfe, Hirsh D Komarow
No abstract text is available yet for this article.
October 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29025582/a-novel-trip11-flt3-fusion-in-a-patient-with-a-myeloid-lymphoid-neoplasm-with-eosinophilia
#8
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
#9
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/28982960/management-of-endocrine-disease-flushing-current-concepts
#10
REVIEW
Isabel Huguet, Ashley Grossman
OBJECTIVE: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions. DESIGN AND METHODS: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'...
November 2017: European Journal of Endocrinology
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28960095/midostaurin-treatment-in-flt3-mutated-acute-myeloid-leukemia-and-systemic-mastocytosis
#14
Sabine Kayser, Mark J Levis, Richard F Schlenk
A number of tyrosine kinase inhibitors (TKIs) have been developed that inhibit the constitutively activated kinase activity caused by activating tyrosine kinase mutations, such as FLT3 or KIT, thus interrupting signaling pathways. Currently, midostaurin is the only approved TKI as monotherapy for aggressive systemic mastocytosis (SM), SM with associated hematological neoplasm, or mast cell leukemia displaying a KIT mutation as well as in combination with standard intensive chemotherapy for adult patients with newly diagnosed FLT3-mutated acute myeloid leukemia (AML)...
September 29, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28952818/incidence-of-second-solid-cancers-in-mastocytosis-patients-a-seer-database-analysis
#15
Velizar Shivarov, Ralitza Gueorguieva, Milena Ivanova, Angel Stoimenov
No abstract text is available yet for this article.
September 27, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28951704/the-role-of-serum-tryptase-in-the-diagnosis-and-monitoring-of-pediatric-mastocytosis-a-single-center-experience
#16
Magdalena Lange, Agata Zawadzka, Stephanie Schrörs, Justyna Słomka, Hanna Ługowska-Umer, Bogusław Nedoszytko, Roman Nowicki
INTRODUCTION: In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up. AIM: To compare clinical presentation and the course of mastocytosis in children with normal and clearly elevated bsT levels as well as to assess its usefulness in the diagnosis and monitoring of the course of the disease...
August 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28945834/midostaurin-a-magic-bullet-that-blocks-mast-cell-expansion-and-activation
#17
P Valent, C Akin, K Hartmann, T I George, K Sotlar, B Peter, K V Gleixner, K Blatt, W R Sperr, P W Manley, O Hermine, H C Kluin-Nelemans, M Arock, H-P Horny, A Reiter, J Gotlib
Clinically relevant features in patients with systemic mastocytosis (SM) include the cosmetic burden of lesional skin, mediator-related symptoms, and organ damage resulting from mast cell (MC) infiltration in advanced forms of SM. Regardless of the SM variant, expansion of neoplastic MC in the skin and other organs is triggered by mutant forms of KIT, the most prevalent being D816V. Activation of MC with subsequent release of chemical mediators is often caused by IgE-dependent mechanisms in these patients. Midostaurin, also known as PKC412, blocks the kinase activity of wild-type KIT and KIT D816V, counteracts KIT-dependent growth of neoplastic MC, and inhibits IgE-dependent mediator secretion...
October 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28934791/a-practical-guide-for-treatment-of-pain-in-patients-with-systemic-mast-cell-activation-disease
#18
Stefan Wirz, Gerhard J Molderings
Systemic mast cell activation disease (MCAD, a subclass of mastocytosis), which has a prevalence of around 17% (at least in the German population), is characterized by accumulation of genetically altered dysfunctional mast cells with abnormal release of these cells' mediators. Since mast cells affect functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing, this disease has to be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity of a generally inflammatory and allergic theme...
September 2017: Pain Physician
https://www.readbyqxmd.com/read/28919366/prevalence-and-risk-factors-for-fragility-fracture-in-systemic-mastocytosis
#19
Yannick Degboé, Marine Eischen, Delphine Nigon, Pol-André Apoil, Claire Mailhol, Emilie Tournier, Camille Laurent, Katia Hanssens, Olivier Hermine, Carle Paul, Michel Laroche, Cristina Bulai-Livideanu
OBJECTIVES: Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Secondary objectives were to evaluate the ability of bone marrow tryptase (BMT) level to identify patients with FF, and to describe bone involvement in SM. METHODS: We analyzed retrospectively all consecutive patients seen in our expert center, with a diagnosis of SM according to the 2001 WHO criteria, and with complete bone assessment...
September 14, 2017: Bone
https://www.readbyqxmd.com/read/28902950/pre-emptive-evaluation-of-venom-allergy-in-a-patient-with-systemic-mastocytosis
#20
Theo Gülen, Cem Akin
No abstract text is available yet for this article.
September 13, 2017: Acta Dermato-venereologica
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