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Systemic mastocytosis

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https://www.readbyqxmd.com/read/29025582/a-novel-trip11-flt3-fusion-in-a-patient-with-a-myeloid-lymphoid-neoplasm-with-eosinophilia
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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/28951704/the-role-of-serum-tryptase-in-the-diagnosis-and-monitoring-of-pediatric-mastocytosis-a-single-center-experience
#7
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
#8
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
#9
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
#10
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
#11
Theo Gülen, Cem Akin
No abstract text is available yet for this article.
September 13, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28881711/characterization-of-midostaurin-as-a-dual-inhibitor-of-flt3-and-syk-and-potentiation-of-flt3-inhibition-against-flt3-itd-driven-leukemia-harboring-activated-syk-kinase
#12
Ellen L Weisberg, Alexandre Puissant, Richard Stone, Martin Sattler, Sara J Buhrlage, Jing Yang, Paul W Manley, Chengcheng Meng, Michael Buonopane, John F Daley, Suzan Lazo, Renee Wright, David M Weinstock, Amanda L Christie, Kimberly Stegmaier, James D Griffin
Oncogenic FLT3 kinase is a clinically validated target in acute myeloid leukemia (AML), and both multi-targeted and selective FLT3 inhibitors have been developed. Spleen tyrosine kinase (SYK) has been shown to be activated and increased in FLT3-ITD-positive AML patients, and has further been shown to be critical for transformation and maintenance of the leukemic clone in these patients. Further, over-expression of constitutively activated SYK causes resistance to highly selective FLT3 tyrosine kinase inhibitors (TKI)...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28871925/mast-cell-disorders-melanoma-and-pancreatic-carcinoma-from-a-clinical-observation-to-a-brief-review-of-the-literature
#13
Giovanni Paolino, Maria Belmonte, Stefania Trasarti, Michelina Santopietro, Luisa Bizzoni, Mara Riminucci, Luisa Cardarelli, Emilia Iannella, Marcello Albanesi, Elisa Moliterni, Dario Didona, Stefano Calvieri, Robin Foà, Fiorina Giona
Mastocytosis can be associated with other clonal or non-clonal hematologic diseases as well as a variety of non-hematologic malignancies. A 75-year-old Caucasian male patient was referred to us with a 5-month history of neutrophilic leukocytosis and mild splenomegaly. He had developed a cutaneous melanoma sixteen years ago. According to the clinical and pathological features, a final diagnosis of systemic mastocytosis was established. The patient started treatment with interferon-α at a dose of 3 MIU/day, combined with low doses of prednisone...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28855975/case-report-unusual-manifestation-of-kit-negative-systemic-mastocytosis
#14
Beatriz Cáceres-Nazario, William Cáceres-Perkins, David Tasso, Elizabeth Calderón-Alicea, Daniel Conde-Sterling, Norma Arroyo-Portela
Aggressive systemic mastocytosis is a rare hematologic neoplastic disease that presents with a poor prognosis and low survival rate. It typically manifests with symptoms associated to mast cell release of bioactive substances, causing anaphylaxis, flushing, autonomic and hemodynamic instability, gastric distress and headache. Moreover, more than 95% of cases are related to a mutation in codon 816 of the KIT gene, located on human chromosome 4q12 which codes for a type III receptor tyrosine kinase. We present a 78 year-old Hispanic man diagnosed with the aggressive subtype of systemic mastocytosis, who had an atypical manifestation and a KIT negative variant...
December 2016: American Journal of Hematology/oncology
https://www.readbyqxmd.com/read/28796123/hymenoptera-induced-anaphylaxis-is-it-a-mast-cell-driven-hematological-disorder
#15
Knut Brockow, Cem Akin
PURPOSE OF REVIEW: Hymenoptera-induced allergy (HVA) is a common cause of anaphylaxis and may be fatal. It is associated with systemic mastocytosis in about 7% of adult patients. Systemic mastocytosis is a proliferative disorder of hematopoietic mast cell progenitors. There is recent data on the association between systemic mastocytosis and HVA. RECENT FINDINGS: Hymenoptera venoms are the most common immunoglobulin E-mediated elicitors of anaphylaxis in patients with mastocytosis...
August 8, 2017: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28770635/natural-history-and-treatment-of-cutaneous-and-systemic-mastocytosis
#16
Michelle Le, Barbara Miedzybrodzki, Tim Olynych, Hugo Chapdelaine, Moshe Ben-Shoshan
INTRODUCTION: Mastocytosis, a heterogeneous group of disorders, is characterized by an abnormal increase in the number of mast cells that is limited to the skin (cutaneous mastocytosis), involving extracutaneous tissues (systemic mastocytosis), or presenting as solid tumours (mastocytoma and mast cell sarcoma). Recent studies estimate that 1 in 10,000 people are diagnosed with mastocytosis. Although prompt diagnosis and appropriate management are crucial, little is known about the natural history and currently there are no established management guidelines...
August 21, 2017: Postgraduate Medicine
https://www.readbyqxmd.com/read/28757371/dystonia-as-an-unusual-presentation-of-systemic-mastocytosis-possible-link-between-histamine-release-and-movement-disorders
#17
Alexandra Phillips, Flavia C L Hoyte, Donald Y M Leung
No abstract text is available yet for this article.
July 27, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28744009/efficacy-and-safety-of-midostaurin-in-patients-with-advanced-systemic-mastocytosis-10-year-median-follow-up-of-a-phase-ii-trial
#18
D J DeAngelo, T I George, A Linder, C Langford, C Perkins, J Ma, P Westervelt, J D Merker, C Berube, S Coutre, M Liedtke, B Medeiros, D Sternberg, C Dutreix, P-A Ruffie, C Corless, T J Graubert, J Gotlib
Patients with advanced systemic mastocytosis (SM) (e.g. aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN) and mast cell leukemia (MCL)) have limited treatment options and exhibit reduced survival. Midostaurin is an oral multikinase inhibitor that inhibits D816V-mutated KIT, a primary driver of SM pathogenesis. We conducted a phase II trial of midostaurin 100 mg twice daily, administered as 28-day cycles, in 26 patients (ASM, n=3; SM-AHN, n= 17; MCL, n=6) with at least one sign of organ damage...
July 24, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28740494/insights-in-anaphylaxis-and-clonal-mast-cell-disorders
#19
REVIEW
David González-de-Olano, Iván Álvarez-Twose
The prevalence of anaphylaxis among patients with clonal mast cell disorders (MCD) is clearly higher comparing to the general population. Due to a lower frequency of symptoms outside of acute episodes, clonal MCD in the absence of skin lesions might sometimes be difficult to identify which may lead to underdiagnosis, and anaphylaxis is commonly the presenting symptom in these patients. Although the release of mast cell (MC) mediators upon MC activation might present with a wide variety of symptoms, particular clinical features typically characterize MC mediator release episodes in patients with clonal MCD without skin involvement...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28739366/management-of-mastocytosis-in-pregnancy-a%C3%A2-review
#20
Dawn Lei, Cem Akin, Anna Kovalszki
In this article, the authors present a case of pregnancy complicated by the need for management of indolent systemic mastocytosis. The diagnosis of mastocytosis is reviewed, as well as subtypes of mastocytosis and management options particularly in pregnancy. A table of pregnancy/lactaction categories for common medications used in mastocytosis is presented.
September 2017: Journal of Allergy and Clinical Immunology in Practice
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