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Mastocytosis

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https://www.readbyqxmd.com/read/28543193/patterns-of-anaphylaxis-after-diagnostic-work-up-a-follow-up-study-of-226-patients-with-suspected-anaphylaxis
#1
Athamaica Ruiz Oropeza, Carsten Bindslev-Jensen, Sigurd Broesby-Olsen, Thomas Kristensen, Michael Boe Møller, Hanne Vestergaard, Henrik Fomsgaard Kjaer, Susanne Halken, Annmarie Lassen, Charlotte G Mortz
BACKGROUND: Most published studies on anaphylaxis are retrospective or register based. Data on subsequent diagnostic work-up are sparse. We aimed to characterize patients seen with suspected anaphylaxis at the emergency care setting (ECS), after subsequent diagnostic work-up at our Allergy Center (AC). METHODS: Prospective study including patients from the ECS, Odense University Hospital, during May 2013-April 2014. Possible anaphylaxis cases were daily identified based on a broad search profile including history and symptoms in patient records, diagnostic codes and pharmacological treatments...
May 19, 2017: Allergy
https://www.readbyqxmd.com/read/28520972/r634w-kit-mutation-in-an-adult-with-systemic-mastocytosis
#2
John M Astle, Michal G Rose, Frederick K Racke, Christopher A Tormey, Alexa J Siddon
Mastocytosis is a clonal neoplasm with the potential to affect various organs within the body. It can range in clinical severity from benign to extremely aggressive. Mastocytosis can be separated into cutaneous, systemic, and leukemic forms, as well as mast-cell sarcoma and extracutaneous mastocytoma. It is most often an acquired condition but can be inherited; the most commonly identified genetic aberrations leading to mastocytosis are activating mutations involving codon 816 of the KIT gene. Herein, we present the case of a 30-year-old Caucasian man with systemic mastocytosis discovered to have a p...
May 18, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/28512562/morphologic-confounders-and-cd19-negativity-in-a-case-of-hairy-cell-leukemia
#3
Pulkit Rastogi, Sreejesh Sreedharanunni, Uday Yanamandra, Man Updesh Singh Sachdeva, Neelam Varma
OBJECTIVES: We report a case of hairy cell leukemia (HCL) initially misdiagnosed as plasma cell dyscrasia due to various clinical, morphological and immunophenotypic confounders. METHODS AND RESULTS: In a patient diagnosed of marrow plasmacytosis and serum monoclonal protein elsewhere and referred to our hospital, morphological evaluation of bone marrow aspirate smears and trephine biopsy, immunophenotyping, and molecular testing (BRAFV600E mutation) were done. Clinically, the patient was asymptomatic; bone marrow revealed plasmacytosis, mastocytosis, and lymphocytosis with a few "hairy" cells...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28501742/differences-in-the-imaging-features-and-distribution-of-non-indolent-and-indolent-mastocytosis-a-single-institution-experience-of-29-patients
#4
Yan Epelboym, Abhishek R Keraliya, Sree Harsha Tirumani, Jason L Hornick, Nikhil H Ramaiya, Atul B Shinagare
PURPOSE: Compare imaging features of indolent and non-indolent mastocytosis. METHODS: For 29 patients, imaging features, imaging indications, and distribution of indolent and non-indolent mastocytosis subtypes were analyzed. RESULTS: 16/29 (55%) patients had three distinct patterns of osseous abnormality, not significantly differing between cohorts. Non-indolent disease was more likely to present with hepatomegaly (p=0.0004), splenomegaly (p=0...
May 6, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28499778/fatal-anaphylaxis-to-yellow-jacket-stings-in-mastocytosis-options-for-identification-and-treatment-of-at-risk-patients
#5
Byrthe J P R Vos, Bjorn van Anrooij, Jasper J van Doormaal, Anthony E J Dubois, Joanne N G Oude Elberink
BACKGROUND: Patients with indolent systemic mastocytosis (ISM) are at risk for severe anaphylactic reactions to yellow jacket (YJ) stings while demonstration of sensitization can be challenging because specific IgE (sIgE) levels are regularly below 0.35 kUA/L. The implication of missing YJ allergy is illustrated by a case of fatal anaphylaxis. OBJECTIVE: To explore the natural course of YJ venom allergy and the diagnostic accuracy and therapeutic consequence of YJ venom sIgE in patients with ISM...
May 10, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28486845/routine-abdominal-ultrasonography-has-limited-value-in-the-care-for-patients-with-indolent-systemic-mastocytosis
#6
C L de Mol, M A W Hermans, R Gerth van Wijk, P M van Hagen, P L A van Daele
OBJECTIVES: Systemic mastocytosis (SM) is a myeloproliferative disease characterized by the accumulation of aberrant mast cells. Since advanced subtypes of SM can lead to organ dysfunction and shortened survival, timely recognition of progressive disease is important for the adequate treatment of SM patients. METHODS: Here, we report the results of our cohort study on the value of routine abdominal ultrasonography for the detection of progression of indolent systemic mastocytosis (ISM)...
May 10, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28483325/tryptase-increase-without-mastocytosis-or-anaphylaxis
#7
EDITORIAL
Dennis K Ledford, Cem Akin
No abstract text is available yet for this article.
May 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28480804/tolerability-and-benefit-of-a-tetramethoxyluteolin-containing-skin-lotion
#8
Theoharis C Theoharides, Julia M Stewart, Irene Tsilioni
As many as 40% of people have sensitive skin and at least half of them suffer from pruritus associated with allergies, atopic dermatitis (AD), chronic urticaria (CU), cutaneous mastocytosis (CM), and psoriasis. Unfortunately, the available topical formulations contain antihistamines that are often not as effective as those containing corticosteroids. Certain natural flavonoids have anti-inflammatory actions. We recently reported that the natural flavonoid tetramethoxyluteolin has potent antiallergic and anti-inflammatory actions in vitro and in vivo...
May 1, 2017: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/28461029/-uncommon-dermatologic-disorders-triggered-by-radiation-therapy-of-breast-cancer-a-case-series
#9
C Dalmasso, É Tournier, B de Lafontan, A Modesto, F Dalenc, É Chantalat, L Gladieff, E Jouve, C Livideanu, F Izar, V Sibaud
Radiotherapy's main skin toxicities are now well-separated, acute (acute radiation dermatitis) or chronic complications (chronic radiation dermatitis, induced cutaneous carcinoma, aesthetic sequelae). Exceptionally, radiotherapy may induce, by isomorphic reaction or Koebner's phenomenon, some specific dermatosis. In this article, we report five new observations of these unusual complications of radiation therapy, occurring in very variable time after breast irradiation and remaining strictly localized in the irradiated field (cutaneous mastocytosis, Sweet syndrome, lichen planus, vitiligo)...
April 28, 2017: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
https://www.readbyqxmd.com/read/28445138/killer-cell-immunoglobulin-like-receptor-2dl4-is-expressed-in-and-suppresses-the-cell-growth-of-langerhans-cell-histiocytosis
#10
Yusuke Takei, Chiyuki Ueshima, Tatsuki R Kataoka, Masahiro Hirata, Akihiko Sugimoto, Mariyo Rokutan-Kurata, Koki Moriyoshi, Kazuo Ono, Ichiro Murakami, Sanju Iwamoto, Hironori Haga
Killer cell immunoglobulin-like receptor (KIR) 2DL4 (CD158d) is a receptor for human leukocyte antigen-G. The function of KIR2DL4 has been reported in human natural killer cell lymphoma and mastocytosis, but not in Langerhans cell histiocytosis (LCH). Herein, we examined the expression and function of KIR2DL4 in LCHs. In pathological specimens, 27 of 36 LCH cases (75.0%) were immunohistochemically positive for KIR2DL4. Its expression was independent of age, gender, location, multi- or single-system, and the status of BRAFV600E immunostaining...
April 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28439288/systemic-mastocytosis-with-kit-v560g-mutation-presenting-as-recurrent-episodes-of-vascular-collapse-response-to-disodium-cromoglycate-and-disease-outcome
#11
Iolanda Conde-Fernandes, Rita Sampaio, Filipa Moreno, José Palla-Garcia, Maria Dos Anjos Teixeira, Inês Freitas, Esmeralda Neves, Maria Jara-Acevedo, Luis Escribano, Margarida Lima
BACKGROUND: Mastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(-), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28432683/prospective-evaluation-of-the-diagnostic-value-of-sensitive-kit-d816v-mutation-analysis-of-blood-in-adults-with-suspected-systemic-mastocytosis
#12
Thomas Kristensen, Hanne Vestergaard, Carsten Bindslev-Jensen, Charlotte Gotthard Mortz, Henrik Fomsgaard Kjaer, Markus Ollert, Michael Boe Møller, Sigurd Broesby-Olsen
BACKGROUND: Sensitive KIT D816V mutation analysis of blood has been proposed to guide bone marrow (BM) investigation in suspected systemic mastocytosis (SM). The aim of this prospective study was for the first time to compare the D816V-status of the "screening blood sample" used to guide BM biopsy in suspected SM to the outcome of the subsequent BM investigation. METHODS: 58 adult patients with suspected SM were included. The outcome of sensitive KIT D816V-analysis of blood was compared to the result of the BM investigation...
April 22, 2017: Allergy
https://www.readbyqxmd.com/read/28424161/response-and-progression-on-midostaurin-in-advanced-systemic-mastocytosis-kit-d816v-and-other-molecular-markers
#13
Mohamad Jawhar, Juliana Schwaab, Nicole Naumann, Hans-Peter Horny, Karl Sotlar, Torsten Haferlach, Georgia Metzgeroth, Alice Fabarius, Peter Valent, Wolf-Karsten Hofmann, Nicholas C P Cross, Manja Meggendorfer, Andreas Reiter
In advanced systemic mastocytosis (advSM), disease evolution is often triggered by activating KIT mutations (D816V in >80% of cases) and by additional mutations, e.g. in SRSF2, ASXL1 and/or RUNX1 (S/A/R(pos), >60% of cases). In a recently reported phase-II-study, midostaurin, a multikinase/KIT inhibitor, demonstrated an overall response rate (ORR) of 60% in advSM but biomarkers predictive of response are lacking. We evaluated the impact of molecular markers (KIT D816V, S/A/R(pos)) at baseline and during follow-up in 38 midostaurin-treated advSM patients...
April 19, 2017: Blood
https://www.readbyqxmd.com/read/28408849/a-retrospective-epidemiological-study-of-skin-diseases-among-pediatric-population-attending-a-tertiary-dermatology-referral-center-in-northern-greece
#14
Efstratios Vakirlis, Grigorios Theodosiou, Zoe Apalla, Michael Arabatzis, Elizabeth Lazaridou, Elena Sotiriou, Aimilios Lallas, Demetrios Ioannides
BACKGROUND: The incidence of skin diseases in children is influenced by hereditary, social, and environmental factors. The objective of this study was to determine the incidence of pediatric dermatoses at a University Hospital in Northern Greece. PATIENTS AND METHODS: We reviewed epidemiologic data of 940 patients, aged 0-18 years, who were referred to the outpatient clinic of a University Hospital between January 2013 and December 2015. Demographic data and the frequency of the various diagnoses in various age groups were studied...
2017: Clinical, Cosmetic and Investigational Dermatology
https://www.readbyqxmd.com/read/28408397/the-il-31-il-31-receptor-axis-general-features-and-role-in-tumor-microenvironment
#15
REVIEW
Elisa Ferretti, Anna Corcione, Vito Pistoia
IL-31 is a recently identified cytokine with a well-defined role in the pathogenesis of pruritus. IL-31, whose production is induced by IL-4 and IL-33, binds a heterodimeric receptor (R) composed of the exclusive IL-31RA chain and the shared oncostatin M R. Signaling through the IL-31R involves the MAPK, PI3K/AKT and Jak/STAT pathways. Different variants and isoforms of IL-31RA with different signaling activities have been identified. IL-31 is produced predominantly by circulating Th2 lymphocytes and skin-homing CLA(+)CD45RO(+) T cells...
April 13, 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/28401108/a-case-of-lymphocytic-variant-hypereosinophilic-syndrome-with-sub-diagnostic-systemic-mastocytosis
#16
Preetesh Jain, Sa A Wang, C Cameron Yin, Yasmin Abaza, Srdan Verstovsek, Zeev Estrov
No abstract text is available yet for this article.
March 2017: Blood Research
https://www.readbyqxmd.com/read/28386644/mutational-profiling-in-the-peripheral-blood-leukocytes-of-patients-with-systemic-mast-cell-activation-syndrome-using-next-generation-sequencing
#17
Janine Altmüller, Britta Haenisch, Amit Kawalia, Markus Menzen, Markus M Nöthen, Heide Fier, Gerhard J Molderings
Mast cell activation syndrome (MCAS) and systemic mastocytosis (SM) are two clinical systemic mast cell activation disease variants. Few studies to date have investigated the genetic basis of MCAS. The present study had two aims. First, to investigate whether peripheral blood leukocytes from MCAS patients also harbor somatic mutations in genes implicated in SM using next-generation sequencing (NGS) technology and a relatively large MCAS cohort. We also addressed the question, whether some of the previously as somatic reported mutations are indeed germline mutations...
April 6, 2017: Immunogenetics
https://www.readbyqxmd.com/read/28386107/constitutively-active-abl-family-kinases-tel-abl-and-tel-arg-harbor-distinct-leukemogenic-activities-in-vivo
#18
A Yokota, H Hirai, T Shoji, T Maekawa, K Okuda
ABL (ABL1) and ARG (ABL2) are highly homologous to each other in overall domain structure and amino-acid sequence, with the exception of their C termini. As with ABL, translocations that fuse ARG to ETV6/TEL have been identified in patients with leukemia. To assess the in vivo leukemogenic activity of constitutively active ABL and ARG, we generated a bone marrow (BM) transplantation model using the chimeric forms TEL/ABL and TEL/ARG, which have comparable kinase activities. TEL/ABL rapidly induced fatal myeloid leukemia in recipient mice, whereas recipients of TEL/ARG-transduced cells did not develop myeloid leukemia, instead, they succumbed to a long-latency infiltrative mastocytosis that could be adoptively transferred to secondary recipients...
May 5, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28382662/successful-targeted-treatment-of-mast-cell-activation-syndrome-with-tofacitinib
#19
Lawrence B Afrin, Roger W Fox, Susan L Zito, Leo Choe, Sarah C Glover
Mast cell (MC) activation syndrome (MCAS) is a collection of illnesses of inappropriate MC activation with little to no neoplastic MC proliferation, distinguishing it from mastocytosis. MCAS presents as chronic, generally inflammatory multisystem polymorbidity likely driven in most by heterogeneous patterns of constitutively activating mutations in MC regulatory elements, posing challenges for identifying optimal mutation-targeted treatment in individual patients. Targeting commonly affected downstream effectors may yield clinical benefit independent of upstream mutational profile...
April 6, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28369700/in-utero-presentation-of-aggressive-systemic-mastocytosis-in-a-neonate
#20
A Huang, N Fiadorchanka, K Brar, J L Balderacchi, S A Glick
Mastocytosis is a clinically heterogenous disease characterized by mast cell hyperplasia in skin, bone marrow, and/or visceral organs. Cutaneous mastocytosis (CM) is more frequently observed in children, while indolent systemic mastocytosis (ISM) is more commonly observed in adults. Aggressive systemic presentation, particularly, of the neonate, is exceptionally rare. We present a rare case of congenital aggressive systemic mastocytosis (ASM). The patient was a 37-week old male, born by Cesarean section due to hepatosplenomegaly and ascites diagnosed in-utero, who exhibited extensive cutaneous and systemic manifestations of mastocytosis at birth...
March 30, 2017: British Journal of Dermatology
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