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Mastocytosis

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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
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28612232/midostaurin-first-global-approval
#5
Esther S Kim
Midostaurin (Rydapt(®)) is a multikinase inhibitor being developed by Novartis Pharmaceuticals. In April 2017, midostaurin was approved in the USA for the treatment of adult patients with newly diagnosed, FMS-like tyrosine kinase 3 (FLT3) mutation-positive acute myeloid leukaemia (AML) [in combination with standard cytarabine and daunorubicin induction, and cytarabine consolidation], or aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated haematological neoplasm (SM-AHN) or mast cell leukaemia (MCL) [collectively known as advanced SM]...
June 13, 2017: Drugs
https://www.readbyqxmd.com/read/28610444/midostaurin-for-the-treatment-of-acute-myeloid-leukemia
#6
Mrinal M Patnaik
Midostaurin is a multikinase tyrosine kinase inhibitor acting against targets known to be expressed in hematologic malignancies, especially acute myeloid leukemia. Midostaurin combined with chemotherapy followed by single-agent maintenance therapy elicited statistically significant and clinically meaningful improvement in overall survival versus placebo in patients with newly diagnosed FLT3-mutant acute myeloid leukemia. Although gastrointestinal events were more common with midostaurin, overall the drug was relatively well tolerated...
June 14, 2017: Future Oncology
https://www.readbyqxmd.com/read/28599188/mast-cell-leukemia-mcl-clinico-pathologic-and-molecular-features-and-survival-outcome
#7
Preetesh Jain, Sa Wang, Keyur P Patel, Nawid Sarwari, Jorge Cortes, Hagop Kantarjian, Srdan Verstovsek
Mast cell leukemia (MCL) is a very rare subtype of systemic mastocytosis (SM). We have identified 13 such patients (5.9%) among 218 patients with SM seen at our institution between 1994 and 2016. Patients with MCL had poor survival (median 31.6 months); response to various therapies was rare and not durable. Clinical course may be affected by concurrent associated hematologic neoplasm and different genetic profiles. More research is required to decipher this rare and enigmatic SM subtype.
June 1, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28589908/activation-of-trka-receptor-elicits-mastocytosis-in-mice-and-is-involved-in-the-development-of-resistance-to-kit-targeted-therapy
#8
Min Yang, Zengkai Pan, Kezhi Huang, Guntram Büsche, Friedrich Feuerhake, Anuhar Chaturvedi, Danian Nie, Michael Heuser, Felicitas Thol, Nils von Neuhoff, Arnold Ganser, Zhixiong Li
The neurotrophins (NTs) play a key role in neuronal survival and maintenance. The TRK (tropomyosin-related kinase) tyrosine kinase receptors (TRKA, TRKB, TRKC) are high affinity receptors for NTs. There is increasing data demonstrating an important role of the TRK family in cancer initiation and progression. NTs have been known for many years to promote chemotaxis, maturation, and survival of mast cells. However, the role of NT signaling in the pathogenesis of mastocytosis is not well understood. In this study, we demonstrate that activation of TRKA by its ligand nerve growth factor (NGF) is potent to trigger a disease in mice with striking similarities to human systemic mastocytosis (SM)...
May 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28584020/kit-d816v-induces-src-mediated-tyrosine-phosphorylation-of-mitf-and-altered-transcription-program-in-melanoma
#9
Bengt Phung, Julhash U Kazi, Alicia Lundby, Kristin Bergsteinsdottir, Jianmin Sun, Colin R Goding, Göran Jönsson, Jesper V Olsen, Eiríkur Steingrímsson, Lars Rönnstrand
The oncogenic D816V mutation of the KIT receptor is well characterized in systemic mastocytosis and acute myeloid leukemia. Although KIT(D816V) has been found in melanoma, its function and involvement in this malignancy is not understood. Here we show that KIT(D816V) induces tyrosine phosphorylation of MITF through a triple protein complex formation between KIT, MITF, and SRC family kinases. In turn, phosphorylated MITF activates target genes that are involved in melanoma proliferation, cell-cycle progression, suppression of senescence, survival, and invasion...
June 5, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28579854/acute-myeloid-leukemia-with-inv-16-p13q22-associated-with-hidden-systemic-mastocytosis-case-report-and-review-of-literature
#10
Feryal Abbas Ibrahim Hilmi, Ahmad Al-Sabbagh, Dina Sameh Soliman, Hesham Al Sabah, Omar Mohammad Ismail, Mohamed Yassin, Halima El-Omri
Systemic mastocytosis (SM) is a condition associated with clonal neoplastic proliferation of mast cells. In up to 40% of systemic mastocytosis cases, an associated clonal hematological disease of non-mast cell lineage, such as acute myeloid leukemia (AML), is diagnosed before, simultaneously with, or after the diagnosis of SM. Herein, we report a case of a 30-year-old man diagnosed with AML with inv(16) (p13;q22) CBFB:MYH11. Associated mastocytosis was not noted at diagnosis and was only detected in the bone marrow at time of remission after successful chemotherapy...
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/28570653/improved-efficacy-of-allergen-specific-immunotherapy-by-jak-inhibition-in-a-murine-model-of-allergic-asthma
#11
Antonio Aguilar-Pimentel, Anke Graessel, Francesca Alessandrini, Helmut Fuchs, Valerie Gailus-Durner, Martin Hrabě de Angelis, Dennis Russkamp, Adam Chaker, Markus Ollert, Simon Blank, Jan Gutermuth, Carsten B Schmidt-Weber
BACKGROUND: Allergen-specific immunotherapy (AIT) is the only curative treatment for type-1 allergies, but sometimes shows limited therapeutic response as well as local and systemic side effects. Limited control of local inflammation and patient symptoms hampers its widespread use in severe allergic asthma. OBJECTIVE: Our aim was to evaluate whether AIT is more effective in suppression of local inflammation if performed under the umbrella of short-term non-specific immunomodulation using a small molecule inhibitor of JAK pathways...
2017: PloS One
https://www.readbyqxmd.com/read/28570344/pharmacotherapy-of-mast-cell-disorders
#12
Theo Gülen, Cem Akin
PURPOSE OF REVIEW: Mast cell disorders (MCDs) comprise mastocytosis and disorders referred to as mast cell activation syndrome and are caused by abnormal accumulation and/or activation of mast cells in tissues. Clinical signs and symptoms are protean; therefore, finding suitable treatment options for individual patients entails a challenge for clinicians. The purpose of this manuscript is to review the literature on the available therapeutic interventions in patients with MCD. RECENT FINDINGS: Pharmacotherapy is mainly directed against the effects of mast cells and their mediators...
May 31, 2017: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28559894/multifactorial-modulation-of-food-induced-anaphylaxis
#13
REVIEW
Sara Benedé, María Garrido-Arandia, Laura Martín-Pedraza, Cristina Bueno, Araceli Díaz-Perales, Mayte Villalba
Prevalence of food-induced anaphylaxis increases progressively and occurs in an unpredictable manner, seriously affecting the quality of life of patients. Intrinsic factors including age, physiological, and genetic features of the patient as well as extrinsic factors such as the intake of drugs and exposure to environmental agents modulate this disorder. It has been proven that diseases, such as mastocytosis, defects in HLA, or filaggrin genes, increase the risk of severe allergic episodes. Certain allergen families such as storage proteins, lipid transfer proteins, or parvalbumins have also been linked to anaphylaxis...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28543193/patterns-of-anaphylaxis-after-diagnostic-work-up-a-follow-up-study-of-226-patients-with-suspected-anaphylaxis
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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