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https://www.readbyqxmd.com/read/29643855/targeting-sphingosine-kinase-isoforms-effectively-reduces-growth-and-survival-of-neoplastic-mast-cells-with-d816v-kit
#1
Geethani Bandara, Rosa Muñoz-Cano, Araceli Tobío, Yuzhi Yin, Hirsh D Komarow, Avanti Desai, Dean D Metcalfe, Ana Olivera
Mastocytosis is a disorder resulting from an abnormal mast cell (MC) accumulation in tissues that is often associated with the D816V mutation in KIT, the tyrosine kinase receptor for stem cell factor. Therapies available to treat aggressive presentations of mastocytosis are limited, thus exploration of novel pharmacological targets that reduce MC burden is desirable. Since increased generation of the lipid mediator sphingosine-1-phosphate (S1P) by sphingosine kinase (SPHK) has been linked to oncogenesis, we studied the involvement of the two SPHK isoforms (SPHK1 and SPHK2) in the regulation of neoplastic human MC growth...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29619541/systemic-mastocytosis-identified-in-two-women-developing-fragility-fractures-during-lactation
#2
J J Zhu, D Mahendran, M H Lee, J Seah, S Fourlanos, S Varadarajan, A Ghasem-Zadeh, R J MacIsaac, E Seeman
Two women presenting with fragility fractures during lactation had bone mineral density (BMD) reduced more greatly than usually associated with lactation. The first woman was 29 years old with a BMD T-score of - 3.2 SD at the spine and- 2.0 SD at the femoral neck. The second woman was 35 years old with a BMD T-score of - 4.5 SD at the spine and - 2.8 SD at the femoral neck. Both women had increased cortical porosity and reduced trabecular density. Investigation identified an elevated serum tryptase, and marrow biopsy confirmed the diagnosis of mastocytosis...
April 4, 2018: Osteoporosis International
https://www.readbyqxmd.com/read/29618463/stone-rm-manley-pw-larson-ra-capdeville-r-midostaurin-its-odyssey-from-discovery-to-approval-for-treating-acute-myeloid-leukemia-and-advanced-systemic-mastocytosis-blood-adv-2017-2-4-444-453
#3
https://www.readbyqxmd.com/read/29602231/eosinophil-phagocytosis-in-advanced-systemic-mastocytosis-with-eosinophilia
#4
Rosario M Morales-Camacho, Sandra Villanueva-Herraiz, Concepción Prats-Martín, Juan J Borrero, Ricardo Bernal, M Teresa Vargas
No abstract text is available yet for this article.
March 30, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29599147/systemic-mastocytosis-with-concurrent-multiple-myeloma
#5
Ashvind A Prabahran, Surender K Juneja
No abstract text is available yet for this article.
March 29, 2018: Blood
https://www.readbyqxmd.com/read/29575311/bubble-trouble-cutaneous-mastocytosis-mistaken-as-non-accidental-injury
#6
Wei Chern Gavin Fong, Wedad Abdelrahman, Susannah E Hoey
No abstract text is available yet for this article.
March 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29571984/a-case-of-mast-cell-leukemia-a-review-of-the-pathophysiology-of-systemic-mastocytosis-and-associated-psychiatric-symptoms
#7
Nathan K Jamison, Emily G Holmes
No abstract text is available yet for this article.
February 12, 2018: Psychosomatics
https://www.readbyqxmd.com/read/29569284/low-frequency-of-acetyl-salicylic-acid-hypersensitivity-in-mastocytosis-the-results-of-a-double-blind-placebo-controlled-challenge-study
#8
M A W Hermans, S Q A van der Vet, P M van Hagen, R Gerth van Wijk, P L A van Daele
BACKGROUND: Patients with mastocytosis are at increased risk of anaphylaxis. The use of nonsteroidal anti-inflammatory drugs (NSAIDs) is often discouraged because of this reason. However, the actual prevalence and severity of NSAID-related hypersensitivity among patients with mastocytosis is unknown. METHODS: A double-blind, placebo-controlled acetylsalicylic acid (ASA) challenge up to a cumulative dose of 520 mg was performed among adult patients with mastocytosis...
March 22, 2018: Allergy
https://www.readbyqxmd.com/read/29567760/blatt-k-cerny-reiterer-s-schwaab-j-et-al-identification-of-the-ki-1-antigen-cd30-as-a-novel-therapeutic-target-in-systemic-mastocytosis-blood-2015-126-26-2832-2841
#9
https://www.readbyqxmd.com/read/29566880/mastocytosis-with-associated-essential-thrombocythemia
#10
J Marcoval
No abstract text is available yet for this article.
March 19, 2018: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29517164/anaphylactic-reaction-to-an-ultrasound-contrast-agent-lumason-in-a-patient-with-systemic-mastocytosis
#11
Michael C Olson, Thomas D Atwell, John M Knudsen
Severe adverse reactions to ultrasound (US) contrast agents are rare, and only a few cases of anaphylaxis following the administration of US contrast agents have been reported, often without a defined etiology. We present a case of anaphylactic reaction to the injection of an US contrast agent in a patient with systemic mastocytosis, which highlights the disorder as a possible risk factor warranting additional consideration prior to performing a contrast-enhanced US examination.
March 8, 2018: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/29507554/the-role-of-regulatory-t-cells-and-genes-involved-in-their-differentiation-in-pathogenesis-of-selected-inflammatory-and-neoplastic-skin-diseases-part-ii-the-treg-role-in-skin-diseases-pathogenesis
#12
REVIEW
Bogusław Nedoszytko, Magdalena Lange, Małgorzata Sokołowska-Wojdyło, Joanna Renke, Piotr Trzonkowski, Michał Sobjanek, Aneta Szczerkowska-Dobosz, Marek Niedoszytko, Aleksandra Górska, Jan Romantowski, Justyna Czarny, Jarosław Skokowski, Leszek Kalinowski, Roman Nowicki
Regulatory FOXP3+ T cells (Tregs) constitute 5% to 10% of T cells in the normal human skin. They play an important role in the induction and maintenance of immunological tolerance. The suppressive effects of these cells are exerted by various mechanisms including the direct cytotoxic effect, anti-inflammatory cytokines, metabolic disruption, and modulation of the dendritic cells function. The deficiency of Treg cells number or function are one of the basic elements of the pathogenesis of many skin diseases, such as psoriasis, atopic dermatitis, bacterial and viral infections...
October 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29498772/slam-family-member-8-is-involved-in-oncogenic-kit-mediated-signaling-in-human-mastocytosis
#13
Akihiko Sugimoto, Tatsuki R Kataoka, Chiyuki Ueshima, Yusuke Takei, Kyohei Kitamura, Masahiro Hirata, Takashi Nomura, Hironori Haga
The signaling lymphocytic activation molecule family member 8 (SLAMF8)/CD353 is a member of the CD2 family of proteins. Its ligand has not been identified. SLAMF8 is expressed by macrophages and suppresses cellular functions. No study has yet explored SLAMF8 expression or function in human mastocytosis, which features oncogenic KIT-mediated proliferation of human mast cells. SLAMF8 protein was expressed in human mastocytosis cells, immunohistochemically. SLAMF8 expression was also evident in the human mast cell lines, HMC1...
March 2, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29490771/interleukin-33-and-interleukin-4-regulate-interleukin-31-gene-expression-and-secretion-from-human-laboratory-of-allergic-diseases-2-mast-cells-stimulated-by-substance-p-and-or-immunoglobulin-e
#14
Anastasia I Petra, Irene Tsilioni, Alexandra Taracanova, Alexandra Katsarou-Katsari, Theoharis C Theoharides
BACKGROUND: Cytokine interleukin (IL) 31 has emerged as an important component of allergic and inflammatory diseases associated with pruritus, such as atopic dermatitis (AD) and mastocytosis. Mast cells (MC) are stimulated by allergic and nonallergic triggers, and play a critical role in such diseases by secreting histamine and tryptase as well as cytokines and chemokines. IL-33 has been reported to augment MC responses, but its effect on secretion of IL-31 is not known. OBJECTIVES: To investigate whether IL-33 can stimulate the secretion of IL-31 from cultured human MCs and whether this response is augmented by either the neuropeptide substance P (SP) or immunoglobulin E (IgE) and anti-IgE in the absence or presence of IL-4...
March 1, 2018: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/29487059/midostaurin-its-odyssey-from-discovery-to-approval-for-treating-acute-myeloid-leukemia-and-advanced-systemic-mastocytosis
#15
REVIEW
Richard M Stone, Paul W Manley, Richard A Larson, Renaud Capdeville
Midostaurin was a prototype kinase inhibitor, originally developed as a protein kinase C inhibitor and subsequently as an angiogenesis inhibitor, based on its inhibition of vascular endothelial growth factor receptor. Despite promising preclinical data, early clinical trials in multiple diseases showed only modest efficacy. In 1996, the relatively frequent occurrence of fms-like tyrosine kinase 3 ( FLT3 ) activating mutations in acute myeloid leukemia (AML) was first recognized. Several years later, midostaurin was discovered to be a potent inhibitor of the FLT3 tyrosine kinase and to have activity against mutant forms of KIT proto-oncogene receptor tyrosine kinase, which drive advanced systemic mastocytosis (SM)...
February 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29481892/concurrent-ige-mediated-nmba-allergy-in-systemic-mastocytosis
#16
Pascale Dewachter, Claudie Mouton-Faivre
No abstract text is available yet for this article.
February 23, 2018: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/29469787/telangiectasia-macularis-eruptiva-perstans-associated-with-a-sicca-complex
#17
Ramone Faith Williams, Rosalynn M Nazarian, Gideon P Smith
We present the case of woman in her 50s who developed numerous red-brown telangiectatic macules on her trunk and extremities, as well as persistent dry eyes and dry mouth. Skin biopsy was consistent with telangiectasia macularis eruptiva perstans (TMEP). Serum tryptase was elevated suggesting systemic involvement. Anti-Ro and La were negative. ANA was positive. Salivary gland biopsy revealed a focus score of 3 and immunostains revealed infiltrates of aberrant CD117 positive mast cells. This case suggests a mechanistic role of mastocytosis in salivary compromise...
October 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29469737/telangiectasia-macularis-eruptiva-perstans-an-old-terminology-still-frequently-used
#18
Kelly Wilmas, Iris Wohlmuth-Wieser, Madeleine Duvic
The term telangiectasia macularis eruptiva perstans (TMEP) was originally used to describe a rare form of cutaneous mastocytosis (CM) that was limited to the skin with lesions consisting of irregular, telangiectatic macules ranging in color from red to brown. Recent guidelines, however, recommended that the sole presence of telangiectasias should not form the basis of a distinct variant of CM. We conducted a review of the literature from 1930 to 2017 and found 76 cases that were reported as TMEP. Owing to a general misconception about diagnosis of CM and SM, there is a need for further discussion and awareness of the newly proposed World Health Organization (WHO) guidelines...
August 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29466766/recent-advances-in-the-genomics-and-therapy-of-bcr-abl1-positive-and-negative-chronic-myeloproliferative-neoplasms
#19
REVIEW
Tariq I Mughal, Jason Gotlib, Ruben Mesa, Steffen Koschmieder, H Jean Khoury, Jorge E Cortes, Tiziano Barbui, Rüdiger Hehlmann, Michael Mauro, Susanne Saussele, Jerald P Radich, Richard A Van Etten, Giuseppe Saglio, Srdnan Verstovek, Robert Peter Gale, Omar Abdel-Wahab
This review is based on the presentations and deliberations at the 7th John Goldman Chronic Myeloid Leukemia (CML) and Myeloproliferative Neoplasms (MPN) Colloquium which took place in Estoril, Portugal on the 15th October 2017, and the 11th post-ASH International Workshop on CML and MPN which took place on the 6th-7th December 2016, immediately after the 58th American Society of Hematology Annual Meeting. Rather than present a resume of the proceedings, we have elected to address some of the topical translational research and clinically relevant topics in greater detail...
February 14, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29460435/topical-pimecrolimus-for-paediatric-cutaneous-mastocytosis
#20
J Mashiah, A Harel, C Bodemer, S Hadj-Rabia, I Goldberg, E Sprecher, A Kutz
BACKGROUND: Most cases of paediatric cutaneous mastocytosis (CM) occur before the age of 2 years, and regression occurs in only 67% of children. Given the absence of any specific therapy, CM is usually treated symptomatically. A few publications have reported the beneficial effect of calcineurin inhibitors for CM. AIM: We sought to evaluate the clinical effectiveness and safety profile of topical pimecrolimus cream for the treatment of CM. METHODS: We performed a retrospective study of all diagnosed cases of CM treated with topical pimecrolimus 1% cream between 2013 and 2015...
February 20, 2018: Clinical and Experimental Dermatology
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