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https://www.readbyqxmd.com/read/28807108/etiologies-and-management-of-cutaneous-flushing-malignant-causes
#1
REVIEW
Azeen Sadeghian, Hailey Rouhana, Brittany Oswald-Stumpf, Erin Boh
The second article in this 2-part continuing medical education series reviews the following malignant causes of flushing: mastocytosis, medullary thyroid carcinoma, pheochromocytoma, carcinoid tumors, gastroenteropancreatic neuroendocrine tumors, bronchogenic carcinoma, vasointestinal polypeptide secreting tumors, and renal cell carcinoma. The information provided will allow physicians to better distinguish patients who have worrisome presentations that require a more thorough investigation. Appropriate diagnostic workup and treatment options for these malignancies are reviewed...
September 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28796123/hymenoptera-induced-anaphylaxis-is-it-a-mast-cell-driven-hematological-disorder
#2
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
#3
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
#4
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
#5
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) (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
#6
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
#7
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.
July 21, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28731672/-urticaria-pigmentosa-clinical-and-therapeutic-aspects-for-the-paediatrician
#8
Catherina Moll-Manzur, Esteban Araos-Baeriswyl, Camila Downey, María T Dossi
Urticaria pigmentosa, also known as maculopapular mastocytosis, is the most common type of paediatric mastocytosis. It presents with yellow to brown macules or papules, usually located on trunk and extremities. Regarding its diagnostic and therapeutic implications, the objective of this article is to serve as an update for the paediatrician on the most relevant aspects of this pathology.
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28725969/mastocytosis-from-a-molecular-point-of-view
#9
REVIEW
Daniel Elieh Ali Komi, Todd Rambasek, Stefan Wöhrl
Mast cells (MCs) are physiologically activated by binding of stem cell factor (SCF) to the extracellular domains of the Kit receptor. This binding increases the proliferation and prolongs the survival of normal mature MCs, as well as intensifies the release of mediators. In mastocytosis, somatic mutations of the coding Kit gene cause autocrine dysregulation and lead to constitutive KIT activation even in the absence of its ligand SCF. Clinical symptoms are caused by MC-mediator release and/or infiltration of MCs into tissues...
July 19, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28706155/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
#10
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)...
July 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28705853/a-molecular-roadmap-for-midostaurin-in-mastocytosis
#11
Jason Gotlib
No abstract text is available yet for this article.
July 13, 2017: Blood
https://www.readbyqxmd.com/read/28700253/house-dust-mite-induced-allergic-airway-disease-is-independent-of-ige-and-fc%C3%A9-ri%C3%AE
#12
Christopher G McKnight, Joseph A Jude, Zhenqi Zhu, Reynold A Panettieri, Fred D Finkelman
IgE contributes to disease exacerbations but not to baseline airway hyperresponsiveness (AHR) in human asthma. In rodent allergic airway disease (AAD), mast cell and IgE dependence for the induction of AHR has only been observed when mice are immunized with a relatively weak allergen without adjuvant. To evaluate the role of IgE in murine AAD that is induced by a potent allergen, we inoculated BALB/c and FVB/N background wild-type, and IgE- or FcεRIα-deficient mice intratracheally with large or limiting doses of house dust mite extract (HDM) and evaluated AHR, pulmonary eosinophilia, goblet cell metaplasia, serum IgE, and lung mastocytosis...
July 12, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28677275/ultraviolet-radiation-and-skin-mast-cells-effects-mechanisms-and-relevance-for-skin-diseases
#13
REVIEW
Hanna Siiskonen, Anna Smorodchenko, Karoline Krause, Marcus Maurer
Mast cells (MCs) are well-known as versatile effector cells in allergic reactions and several other immune responses. Skin MCs and cutaneous MC responses are subject to the effects of environmental factors including ultraviolet radiation (UVR). Numerous studies have assessed the effects of UVR on MCs, in vitro and in vivo. Interestingly, UVR seems to have variable effects on nonactivated and activated mast cells. In general, UV therapy is beneficial in the treatment of urticaria and mastocytosis, but the effects are variable depending on treatment regimen and type of UVR...
July 4, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28673393/tyrosine-kinase-inhibitors-in-the-treatment-of-eosinophilic-neoplasms-and-systemic-mastocytosis
#14
REVIEW
Jason Gotlib
The World Health Organization's semimolecular classification of eosinophilias emphasizes neoplasms driven by fusion tyrosine kinases. More than 80% of patients with systemic mastocytosis carry the KIT D816V mutation, the primary driver of disease pathogenesis. Genetic annotation of these diseases is critical and affords opportunities for targeted therapy. This article discusses our understanding of the mutated tyrosine kinome of eosinophilic neoplasms and systemic mast cell disease, and the successes and limitations of available therapies...
August 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28663576/setd2-and-histone-h3-lysine-36-methylation-deficiency-in-advanced-systemic-mastocytosis
#15
G Martinelli, M Mancini, C De Benedittis, M Rondoni, C Papayannidis, M Manfrini, M Meggendorfer, R Calogero, V Guadagnuolo, M C Fontana, L Bavaro, A Padella, E Zago, L Pagano, R Zanotti, L Scaffidi, G Specchia, F Albano, S Merante, C Elena, P Savini, D Gangemi, P Tosi, F Ciceri, G Poletti, L Riccioni, F Morigi, M Delledonne, T Haferlach, M Cavo, P Valent, S Soverini
The molecular basis of advanced systemic mastocytosis (SM) is not fully understood and despite novel therapies the prognosis remains dismal. Exome sequencing of an index-patient with mast cell leukemia (MCL) uncovered biallelic loss-of-function mutations in the SETD2 histone methyltransferase gene. Copy-neutral loss-of-heterozygosity at 3p21.3 (where SETD2 maps) was subsequently found in SM patients and prompted us to undertake an in-depth analysis of SETD2 copy number, mutation status, transcript expression and methylation levels, as well as functional studies in the HMC-1 cell line and in a validation cohort of 57 additional cases with SM, including MCL, aggressive SM and indolent SM...
June 16, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28662309/omalizumab-prevents-anaphylaxis-and-improves-symptoms-in-systemic-mastocytosis-efficacy-and-safety-observations
#16
S Broesby-Olsen, H Vestergaard, C G Mortz, B Jensen, T Havelund, A P Hermann, F Siebenhaar, M B Møller, T K Kristensen, C Bindslev-Jensen
BACKGROUND: Patients with systemic mastocytosis (SM) may suffer from mast cell (MC) mediator-related symptoms insufficiently controlled by conventional therapy. Omalizumab is an established treatment in other MC-driven diseases, but experiences in SM are limited. OBJECTIVE: To assess the efficacy and safety of omalizumab in SM. METHODS: In our patient cohort, we evaluated all SM patients treated with omalizumab. A physician global assessment of type and severity of symptoms was performed at baseline, at 3 and 6 months and at latest follow-up...
June 29, 2017: Allergy
https://www.readbyqxmd.com/read/28646532/acquired-bilateral-telangiectasia-macularis-eruptiva-perstans-a-unique-clinical-feature-of-photodamaging-rather-than-a-subtype-of-cutaneous-mastocytosis
#17
Hye-Rim Moon, Young Jae Kim, Joon Min Jung, Chong Hyun Won, Mi Woo Lee, Jee Ho Choi, Sung Eun Chang
Telangiectasia macularis eruptiva perstans (TMEP) is a rare subtype of cutaneous mastocytosis, characterized by telangiectatic tan to brown macules on the trunk and extremities. Although TMEP has been descried as an uncommon disease in the literature, we often encounter patients with TMEP lesions in the outpatient clinic. We aimed to assess the clinical and histopathological characteristics of acquired bilateral TMEP, and the pathophysiological mechanism of acquired bilateral TMEP among these patients. We retrospectively reviewed 30 patients (28 men and 2 women) with acquired bilateral TMEP; multiple telangiectatic dark red to brown macules that were symmetrically distributed...
June 23, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28634542/angioedema-perioperative-management
#18
Andrew A Maynard, Christina F Burger, Joseph J Schlesinger
OBJECTIVE: To describe the perioperative management of a patient with acquired angioedema (AAE). METHODS: A 66-year-old Caucasian male presented from an outside hospital with a history of acquired angioedema and gastrointestinal stromal tumor-related intractable urticaria and mastocytosis. He was admitted for urgent laparoscopic partial gastrectomy, secondary to gastric outlet obstruction symptomatology. Previous combined attacks were characterized by a widespread rash, abdominal pain and respiratory distress resulting in hospitalization...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28632811/management-of-poorly-controlled-indolent-systemic-mastocytosis-using-narrowband-uvb-phototherapy
#19
Zain Husain, Dylan Waterman, Kathleen Ellison, Jennifer A DeSimone
The mastocytoses comprise a group of proliferative stem cell disorders defined by the abnormal accumulation of mast cells (MCs) in the skin or other body tissues including the bone marrow, gastrointestinal tract, and liver. Systemic mastocytosis is defined by the presence of one major and one minor criterion or 3 minor criteria delineated by the World Health Organization (WHO). We present the case of a 57-year-old woman with a 10-year history of red-brown pruritic maculopapular lesions on the upper and lower extremities and trunk who was originally diagnosed with cutaneous mastocytosis...
May 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28629749/a-distinct-biomolecular-profile-identifies-monoclonal-mast-cell-disorders-in-patients-with-idiopathic-anaphylaxis
#20
Melody C Carter, Avanti Desai, Hirsh D Komarow, Yun Bai, Sarah T Clayton, Alicia S Clark, Karina N Ruiz-Esteves, Lauren M Long, Daly Cantave, Todd M Wilson, Linda M Scott, Olga Simakova, Mi-Yeon Jung, Jamie Hahn, Irina Maric, Dean D Metcalfe
BACKGROUND: Clonal mast cell disorders are known to occur in a subset of patients with systemic reactions to Hymenoptera stings. This observation has prompted the question as to whether clonal mast cell disorders also occur in patients with idiopathic anaphylaxis (IA). OBJECTIVE: We sought to determine the prevalence of clonal mast cell disorders among patients with IA, criteria to identify those patients who require a bone marrow biopsy and whether the pathogenesis of IA involves a hyper-responsive mast cell compartment...
June 16, 2017: Journal of Allergy and Clinical Immunology
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