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Essential thrombocytosis

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https://www.readbyqxmd.com/read/29790256/platelet-characteristics-in-patients-with-essential-thrombocytosis
#1
Oliver Heidmann Pedersen, Mads Lamm Larsen, Erik Lerkevang Grove, Peter Buur van Kooten Niekerk, Søren Bønløkke, Peter H Nissen, Steen Dalby Kristensen, Anne-Mette Hvas
BACKGROUND: Essential thrombocytosis (ET) is a myeloproliferative disorder characterized by an increased platelet count. ET is associated with an increased risk of thrombosis, and procoagulant features of the disease may include an increased number of reactive reticulated platelets and an increased aggregation potential. We aimed to explore the association between platelet count, platelet turnover and platelet aggregation in patients with ET. METHODS: We included 24 ET patients who discontinued antiplatelet therapy prior to blood sampling...
May 23, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29784641/intrinsic-apoptosis-circumvents-the-functional-decline-of-circulating-platelets-but-does-not-cause-the-storage-lesion
#2
Irina Pleines, Marion Lebois, Pradnya Gangatirkar, Amanda E Au, Rachael M Lane, Katya J Henley, Maria Kauppi, Jason Corbin, Ping Cannon, Jonathan Bernardini, Imala Alwis, Kate E Jarman, Sarah Ellis, Donald Metcalf, Shaun P Jackson, Simone M Schoenwaelder, Benjamin T Kile, Emma C Josefsson
The circulating life span of blood platelets is regulated by the pro-survival protein BCL-XL. It restrains the activity of BAK and BAX, the essential pro-death mediators of intrinsic apoptosis. Disabling the platelet intrinsic apoptotic pathway in mice by deleting BAK and BAX results in a doubling of platelet life span and concomitant thrombocytosis. Apoptotic platelets expose phosphatidylserine (PS) via a mechanism that is distinct from that driven by classical agonists. Whether there is any role for apoptotic PS in platelet function in vivo, however, is unclear...
May 21, 2018: Blood
https://www.readbyqxmd.com/read/29780645/sustained-regression-of-hydroxycarbamide-induced-actinic-keratoses-after-switching-to-anagrelide
#3
Georgios Gaitanis, Dora Gougopoulou, Eleni Kapsali, Ioannis D Bassukas
Hydroxycarbamide (HC) is the first-line treatment for certain myeloproliferative neoplasms, such as polycythemia vera and essential thrombocytosis (ET). In a subset of these patients long-term treatment with HC can result in the development of confluent actinic keratoses (AK) followed by invasive keratinocytic carcinomas ("squamous dysplasia"), preferentially on sun-exposed skin. Discontinuation or dose reduction of HC may result in partial improvement. A 59-year-old farmer after 14 years on HC (2 gr/d) and acetylsalicylic acid (100 mg/d) for ET, was referred for numerous, hyperkeratotic AK on face, scalp, and hands that could not be controlled with repeated ( N = 15) cryosurgery sessions in the previous 3 years...
2018: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/29732039/jak-2-mutation-frequency-in-patients-with-thrombocytosis
#4
Osman Yokus, Habip Gedik
Background: We aimed to investigate the etiologic causes and the existence of Janus kinase 2 mutation (JAK2) in cases with thrombocytosis. Methods: In this retrospective study, patients who were admitted to hematology clinic with thrombocytosis between 2013 and 2015 were investigated in terms of the etiological causes of thrombocytosis and the existence of JAK2 mutation. Results: We retrospectively evaluated 136 cases that underwent JAK2 mutation analysis due to ET preliminary diagnosis in our hematology clinic...
2018: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/29705369/essential-thrombocythemia-and-cardiac-surgery-a-case-series-and-review-of-the-literature
#5
Carmelina Gurrieri, Bradford B Smith, Gregory A Nuttall, Rajiv K Pruthi, Sameh M Said, Mark M Smith
BACKGROUND: Essential thrombocythemia (ET) is a rare myeloproliferative disorder characterized by an unexplained thrombocytosis (>450 x109 /L) and associated vasomotor, thrombotic and hemorrhagic manifestations. While the literature detailing the perioperative management of patients with ET undergoing cardiac surgery is sparse, major perioperative complications have been reported, particularly in poorly controlled patients presenting with platelet counts ≥800 x 109 /L. The purpose of this study was to provide the experience at a large tertiary medical center in managing patients with ET undergoing cardiac surgery, and to summarize the available literature...
April 26, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29683036/assessment-of-endothelial-dysfunction-with-flow-mediated-dilatation-in-myeloproliferative-disorders
#6
Abdulkerim Yildiz, Melike Güryildirim, Mehmet Sezgin Pepeler, Merve Yazol, Suna Özhan Oktar, Kadir Acar
BACKGROUND: Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs. METHODS: Endothelial dysfunction and its associations with other parameters were investigated. A total of 18 patients with polycythemia vera (PV), 24 with essential thrombocytosis (ET), 7 with primary myelofibrosis (PMF), and 30 healthy patients as a control group were included in the study...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29675946/plasma-proteomics-identifies-a-chemokine-storm-in-idiopathic-multicentric-castleman-disease
#7
Sheila K Pierson, Aaron J Stonestrom, Dustin Shilling, Jason Ruth, Christopher S Nabel, Amrit Singh, Yue Ren, Katie Stone, Hongzhe Li, Frits van Rhee, David C Fajgenbaum
Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS)...
April 20, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29668550/pediatric-chronic-myeloid-leukemia-presenting-with-extreme-thrombocytosis-simulating-essential-thrombocythemia
#8
Jessica L Boklan, Alexandra M Walsh, Michelina C de la Maza, Leon L Su, Francisco A Nizzi, Kristian T Schafernak
A 10-year-old boy presented with spontaneous bruising and was found to have extreme thrombocytosis without neutrophilia/shift to immaturity, basophilia or eosinophilia. While the peripheral blood and bone marrow findings initially suggested essential thrombocythemia, BCR-ABL1 translocation was detected and chronic myeloid leukemia, chronic phase, was diagnosed. Apheresis for platelet depletion was performed as a bridge given the delayed effects of medical therapy.
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29656438/renovascular-hypertension-associated-with-jak2-v617f-positive-myeloproliferative-neoplasms-treated-with-angioplasty-2-cases-and-literature-review
#9
Eikan Mishima, Takehiro Suzuki, Yoichi Takeuchi, Kazumasa Seiji, Noriko Fukuhara, Kei Takase, Hideo Harigae, Takaaki Abe, Sadayoshi Ito
Myeloproliferative neoplasms (MPNs) with Janus kinase 2 (JAK2) mutation are associated with a high risk for occlusive vascular diseases. We report 2 cases of renovascular hypertension associated with JAK2 V617F mutation-positive MPNs and provide a literature review. In Case 1, a 63-year-old woman had resistant hypertension, massive proteinuria, and erythrocytosis. Evaluations revealed right renal artery stenosis causing renovascular hypertension and polycythemia vera with JAK2 V617F mutation. Renin-angiotensin system inhibitors and subsequent angioplasty controlled the blood pressure and the proteinuria resolved...
April 2018: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/29602892/essential-thrombocytosis-40-years-after-splenectomy
#10
Oliver Neuhaus, Gabriele Käfer
No abstract text is available yet for this article.
March 30, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29515972/genetic-alterations-in-essential-thrombocythemia-progression-to-acute-myeloid-leukemia-a-case-series-and-review-of-the-literature
#11
Jackline P Ayres-Silva, Martin H Bonamino, Maria E Gouveia, Barbara C R Monte-Mor, Diego F Coutinho, Adelmo H Daumas, Cristiana Solza, Esteban Braggio, Ilana Renault Zalcberg
The genetic events associated with transformation of myeloproliferative neoplasms (MPNs) to secondary acute myeloid leukemia (sAML), particularly in the subgroup of essential thrombocythemia (ET) patients, remain incompletely understood. Deep studies using high-throughput methods might lead to a better understanding of genetic landscape of ET patients who transformed to sAML. We performed array-based comparative genomic hybridization (aCGH) and whole exome sequencing (WES) to analyze paired samples from ET and sAML phases...
2018: Frontiers in Oncology
https://www.readbyqxmd.com/read/29508552/coexistence-of-jak2-or-calr-mutation-is-a-rare-but-clinically-important-event-in-chronic-myeloid-leukemia-patients-treated-with-tyrosine-kinase-inhibitors
#12
K Lewandowski, M Gniot, M Wojtaszewska, Z Kanduła, R Becht, E Paczkowska, E Mędraś, E Wasilewska, M Iwoła
INTRODUCTION: There are 7 designated conditions under the category of myeloproliferative neoplasms (MPN), including chronic myelogenous leukemia (CML) and classical MPN, that is, polycythemia vera (PV), essential thrombocythaemia (ET), and primary myelofibrosis (PMF). Recently, reports about Philadelphia and JAK2 V617F-positive MPN cases have been described in literature. The coexistence of different molecular defects may change the clinical and laboratory manifestation of MPN and may result in an inappropriate interpretation of the response to treatment with tyrosine kinase inhibitors in CML patients...
March 6, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29492207/evaluation-of-vascular-events-in-patients-with-myeloproliferative-syndromes-and-mutations-of-either-the-januskinase-2-or-calreticulin-gene-at-the-university-hospital-krems-from-2008-to-2015
#13
Sarah Hintermair, Elisabeth Zwickl-Traxler, Martin Pecherstorfer, Josef Singer
Myeloproliferative neoplasms (MPN), classified as polycythemia vera (PV), essential thrombocytosis (ET) and myelofibrosis (MF) are stem-cell derived disorders. Mutations in either the januskinase-2 (JAK-2) or the calreticulin (CALR) gene are characteristic for MPN and may result in enhanced proliferation of red blood cells, white blood cells and platelets, and thus increase the risk for vascular events. This study is a retrospective and descriptive analysis of records of patients, who underwent treatment for myeloproliferative syndromes at the Department of Hemato-Oncology of the University hospital Krems from 2008 to the end of 2015...
February 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29472808/pseudohyperkalemia-potassium-released-from-cells-due-to-clotting-and-centrifugation-a-case-report
#14
REVIEW
Tomáš Šálek
Hyperkalemia is a potentially lethal condition. Pseudohyperkalemia should be always excluded before implementing treatment to prevent inappropriate cause of hypokalemia - equally a potentially lethal condition. Here we present a case report of a 62 year female with chronic myeloproliferative disorder, i.e. essential thrombocythemia. The laboratory test results for potassium concentration were 6.3 mmol/L, for platelet count 1305 x109 /L and for leukocyte count 39.8 x109 /L. This was due to a temporary drug withdrawal after a surgical intervention for gastric bleeding...
February 15, 2018: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/29442537/once-versus-twice-daily-aspirin-treatment-in-patients-with-essential-thrombocytosis
#15
Mads Lamm Larsen, Oliver Heidmann Pedersen, Anne-Mette Hvas, Peter Buur van Kooten Niekerk, Søren Bønløkke, Steen Dalby Kristensen, Erik Lerkevang Grove
Insufficient platelet inhibition has been reported in up to 40% of aspirin-treated patients, including patients with essential thrombocytosis. To maintain sufficient platelet inhibition, a shorter dosing interval with aspirin has been suggested. We aimed to investigate the antiplatelet effect of low-dose aspirin given twice-daily compared to standard once-daily dosing in patients with essential thrombocytosis. We included 22 patients, who were treated for 7 days with standard once-daily aspirin (75 mg once-daily) followed by 7 days treatment of twice-daily aspirin (37...
February 14, 2018: Platelets
https://www.readbyqxmd.com/read/29420626/integrated-micro-messenger-rna-regulatory-networks-in-essential-thrombocytosis
#16
Lu Zhao, Song Wu, Erya Huang, Dimitri Gnatenko, Wadie F Bahou, Wei Zhu
Essential thrombocytosis (ET) is a chronic myeloproliferative disorder with an unregulated surplus of platelets. Complications of ET include stroke, heart attack, and formation of blood clots. Although platelet-enhancing mutations have been identified in ET cohorts, genetic networks causally implicated in thrombotic risk remain unestablished. In this study, we aim to identify novel ET-related miRNA-mRNA regulatory networks through comparisons of transcriptomes between healthy controls and ET patients. Four network discovery algorithms have been employed, including (a) Pearson correlation network, (b) sparse supervised canonical correlation analysis (sSCCA), (c) sparse partial correlation network analysis (SPACE), and, (d) (sparse) Bayesian network analysis-all through a combined data-driven and knowledge-based analysis...
2018: PloS One
https://www.readbyqxmd.com/read/29411299/a-rare-calr-variant-mutation-and-a-review-of-calr-in-essential-thrombocythemia
#17
Robert Diep, Ara Metjian
Essential thrombocythemia (ET) is an indolent myeloproliferative neoplasm characterized by megakaryocyte hyperplasia, thrombocytosis, thrombotic and hemorrhagic complications, and potential transformation into myelofibrosis and acute myeloid leukemia. The vast majority of cases are driven by a somatic mutation in JAK2, CALR, or MPL. CALR, a gene that codes for the calcium-binding chaperone calreticulin, is the predominant mutation in patients with non-mutated JAK2 essential thrombocythemia, accounting for 20-25% of the overall somatic mutation frequency in ET...
April 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29405428/the-2014-bcsh-criteria-and-the-2016-who-criteria-for-essential-thrombocythemia-a-comparison-in-a-large-scale-cohort
#18
Tomonori Ochiai, Hajime Yasuda, Marito Araki, Kyohei Misawa, Soji Morishita, Mai Nudejima, Yumi Hironaka, Shuichi Shirane, Yoko Edahiro, Akihiko Gotoh, Akimichi Ohsaka, Norio Komatsu
OBJECTIVE: There are currently 2 representative diagnostic criteria for essential thrombocythemia (ET), the 2014 British Committee for Standards in Hematology Guidelines (BCSH) criteria and the 2016 World Health Organization (WHO) criteria. We compare and discuss the advantages and disadvantages of the 2 criteria. METHOD: We applied the 2 criteria to 403 patients with thrombocytosis and suspected myeloproliferative neoplasms (MPN) and compared patient populations...
February 5, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29397849/-variation-and-clinical-significance-of-lnk-gene-in-essential-thrombocytosis
#19
Run-Mei Tian, Ying Rong, Liu-Song Wu, Yan Jiang, Hai-Yan Qing, Ping Zhu, Yan Chen
OBJECTIVE: To explore the mutation and single nucleotide polymorphism(SNP) of LNK gene in the patients with essential thrombocytosis (ET), and to analyze the relationship between LNK gene variation and the occurrence of ET. METHODS: JAK2V617F mutation was identified by allele-specific PCR. The whole exon of LNK gene was amplified by PCR. The amplified sequences included the Rs3184504 (C/T) and Rs78894077 (A/C/G/T) affecting the expression of amino acids in LNK gene, and the Rs7973120 (A/T) unaffecting the expression of amino acids...
February 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29381999/abdominal-surgery-in-patients-with-essential-thrombocythemia-a-case-report-and-systematic-review-of-literature
#20
REVIEW
Yi Zhu, HongGang Jiang, ZhiHeng Chen, BoHao Lu, JiaMing Wu
INTRODUCTION: Essential thrombocythemia/thrombocytosis (ET) is characterized by increased bleeding and thrombosis risk during the perioperative period. We report the case of a woman with ET and sigmoid colon cancer, in whom the postoperative course was complicated by anastomotic bleeding. A systematic review was conducted to seek guidance for the management of such patient in the perioperative period. METHODS: A systematic literature review was conducted using EMBASE, Medline, and PubMed databases to detect relevant English language articles...
November 2017: Medicine (Baltimore)
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