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Essential Thrombocythemia

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https://www.readbyqxmd.com/read/28314843/quantitative-proteome-heterogeneity-in-myeloproliferative-neoplasm-subtypes-and-association-with-jak2-mutation-status
#1
Nuria Socoro-Yuste, Cokic Vladan P, Julie Mondet, Isabelle Plo, Pascal Mossuz
Apart from well-known genetic abnormalities, several studies have reported variations in protein expression in Philadelphia negative (Ph-) Myeloproliferative Neoplasm (MPN) patients that could contribute towards their clinical phenotype. In this context, a quantitative mass spectrometry proteomics protocol was used to identify differences in the granulocyte proteome with the goal to characterize the pathogenic role of aberrant protein expression in MPNs. LC MS/MS (LTQ Orbitrap) coupled to iTRAQ labeling showed significant and quantitative differences in protein content among various MPN subtypes [polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF)], and according to the genetic status of JAK2 (JAK2V617F presence and JAK2V617F allele burden)...
March 17, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28301098/pharmacokinetics-of-a-novel-anagrelide-extended-release-formulation-in-healthy-subjects-food-intake-and-comparison-with-a-reference-product
#2
Petro E Petrides, Christian Schoergenhofer, Rudolf Widmann, Bernd Jilma, Christoph S Klade
Anagrelide is an established therapy for essential thrombocythemia. Common adverse effects have been linked to peak plasma concentrations of anagrelide and its 3OH metabolite. Our study was performed to investigate the pharmacokinetics (PK) of a novel anagrelide extended-release (AER) formulation and its active metabolites. Thirty healthy volunteers were randomized to receive either 2 mg AER (under fasting and fed conditions) or 2 mg commercially available reference product (CARP) in an open-label, 3-way crossover trial with washout periods of 6 days...
March 16, 2017: Clinical Pharmacology in Drug Development
https://www.readbyqxmd.com/read/28285408/essential-thrombocythemia-presenting-as-acute-coronary-syndrome-case-reports-and-literature-review
#3
Nanqing Xiong, Wen Gao, Junjie Pan, Xinping Luo, Haiming Shi, Jian Li
For the patients with essential thrombocythemia (ET), systemic thrombosis presents as one of the most dangerous complications. It's been widely accepted that acute coronary syndrome (ACS) is a kind of thrombotic diseases. However, there are very few case reports about ET first presenting as ACS. For some patients diagnosed as ACS, but without markedly elevated platelet, underlying ET was missed. And there are some controversies in the principles and target of treatment in those patients. We reported three cases of ACS, in which the patients who did not have common risk factors for coronary artery diseases and presented only mild atherosclerotic stenosis during coronary angiography, one of which had recurrent coronary artery thrombosis...
March 11, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28279030/-clinical-evaluation-of-the-revised-international-prognostic-score-of-thrombosis-for-essential-thrombocythemia-ipset-thrombosis-in-a-cohort-of-746-chinese-adult-patients
#4
R F Fu, H Y Li, F Xue, X F Liu, W Liu, Y T Huang, Y F Chen, L Y Zhang, L Zhang, R C Yang
Objective: To evaluate the role of the revised International Prognostic Score of Thrombosis (IPSET-thrombosis) in predicting the occurrence of thrombotic events in Chinese patients with essential thrombocythemia (ET) and to develop a thrombosis predicting model more applicable to Chinese ET patients. Methods: Medical records of 746 adult patients with an initial diagnosis of ET were retrospectively analyzed. Results: The median age at diagnosis was 52 (18-87) years, with 305 males and 441 females. According to the revised IPSET-thrombosis model, the number of very low-, low-, intermediate-, and high-risk patients were 271 (36...
February 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28261298/fas-fasl-gene-polymorphisms-in-turkish-patients-with-chronic-myeloproliferative-disorders
#5
Fusun Gediz Ozdemirkiran, Sinem Nalbantoglu, Zafer Gokgoz, Bahriye Kadriye Payzin, Filiz Vural, Seckin Cagirgan, Afig Berdeli
INTRODUCTION: Chronic myeloproliferative disorders (CMPD) are chronic myeloid hematological disorders, characterized by increased myeloid cell proliferation and fibrosis. Impaired apoptotic mechanisms, increased cell proliferation, uncontrolled hematopoietic cell proliferation and myeloaccumulation may contribute to the pathogenesis of CMPD. The aim of our study was to show the possible role of FAS/FASL gene polymorphisms in CMPD pathogenesis and investigate the association with clinical parameters and susceptibility to disease...
March 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28260027/pstat5-and-erk-exhibit-different-expression-in-myeloproliferative-neoplasms
#6
Ewa Wiśniewska-Chudy, Łukasz Szylberg, Grzegorz Dworacki, Ewa Mizera-Nyczak, Andrzej Marszałek
Myeloproliferative neoplasms (MPNs) are clonal hematopoietic progenitor cell disorders characterized by the proliferation of one or more hematopoietic lineages. The classical MPNs include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) entities. These disorders are characterized by bone marrow morphology typical for each disease, and by the presence of JAK2V617F mutation in the marrow and blood. However, JAK2V617F cannot account for the phenotypic heterogeneity of MPNs because approximately half of all cases of ET and PMF show no evidence of this molecular marker...
February 24, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28259826/medical-management-of-extensive-arterial-thromboembolism-in-a-patient-with-essential-thrombocythemia-and-warfarin-failure
#7
Aisha K Ahmed, Alicia Youssef, Nedaa Skeik
Chronic myeloproliferative disorders (CMPD) share a stem cell-derived clonal myeloproliferation. This group of disorders includes essential thrombocythemia (ET), polycythemia vera (PV), chronic myeloid leukemia (CML), and primary myelofibrosis (PMF), with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis(1). These disorders can be associated with genetic mutations affecting protein tyrosine kinases, resulting in different configurations of abnormal signal transduction. The Janus tyrosine kinase 2 (JAK2) mutation can be used as a key diagnostic tool for diagnosing MPDs, specifically, ET, PV, and PMF(2)...
March 1, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28259616/observational-retrospective-study-of-vascular-modulator-changes-during-treatment-in-essential-thrombocythemia
#8
Andrea Piccin, Michael Steurer, Clemens Feistritzer, Ciaran Murphy, Elva Eakins, Muriel Van Schilfgaarde, Daisy Corvetta, Angela Maria Di Pierro, Irene Pusceddu, Luigi Marcheselli, Roberto Gambato, Martin Langes, Dino Veneri, Omar Perbellini, Enrica Pacquola, Michele Gottardi, Filippo Gherlinzoni, Andrea Mega, Martina Tauber, Guido Mazzoleni, Elisa Piva, Mario Plebani, Mauro Krampera, Günther Gastl
Essential thrombocythemia (ET) patients are at risk of developing thrombotic events. Qualitative platelet (PLT) abnormalities and activation of endothelial cells (ECs) and PLTs are thought to be involved. Microparticles (MPs) can originate from PLTs (PMPs), ECs (EMPs), or red cells (RMPs). Previous studies have indicated that MPs contribute to ET pathophysiology. Endothelial modulators (eg, nitric oxide [NO], adrenomedullin [ADM], and endothelin-1 [ET-1]) are also involved in the pathophysiology of this condition...
February 14, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/28251679/risk-factors-for-vascular-complications-and-treatment-patterns-at-diagnosis-of-2389-pv-and-et-patients-real-world-data-from-the-swedish-mpn-registry
#9
Khadija Abdulkarim, Jan Samuelsson, Peter Johansson, Björn Andréasson
OBJECTIVE: The study mainly aimed at investigating possible correlations between peripheral blood counts, erythropoietin (EPO), JAK2 V617F mutation and vascular complications prior to diagnosis of a population-based cohort of newly diagnosed patients with myeloproliferative neoplasms (MPN). METHOD: The study comprises 1105 patients with polycythemia vera (PV) and 1284 patients with essential thrombocythemia (ET) registered in the Swedish MPN Registry. RESULTS: Vascular complications, prior to diagnosis, were registered in 37% of PV patients...
March 2, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28248312/clinical-impact-of-bone-marrow-morphology-for-the-diagnosis-of-essential-thrombocythemia-comparison-between-the-bcsh-and-the-who-criteria
#10
H Gisslinger, G Jeryczynski, B Gisslinger, A Wölfler, S Burgstaller, V Buxhofer-Ausch, M Schalling, M-T Krauth, A-I Schiefer, C Kornauth, I Simonitsch-Klupp, C Beham-Schmid, L Müllauer, J Thiele
No abstract text is available yet for this article.
March 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28247057/novel-hematological-parameters-for-the-evaluation-of-patients-with-myeloproliferative-neoplasms-the-immature-platelet-and-reticulocyte-fractions
#11
Paolo Strati, Prithviraj Bose, Lindsey Lyle, Katie Gaw, Lingsha Zhou, Sherry A Pierce, Julie Huynh-Lu, Cheryl F Hirsch-Ginsberg, Daniel E Bueso-Mendoza, Carlos E Bueso-Ramos, Srdan Verstovsek
New automated hematology analyzers have led to the availability of novel hematological parameters, including the immature platelet fraction (IPF) and the immature reticulocyte fraction (IRF), both of potential interest in patients with myeloproliferative neoplasms (MPNs). We performed a prospective analysis of 217 patients with MPN, including 32 (15%) with essential thrombocythemia (ET), 43 (20%) with polycythemia vera (PV), and 142 (65%) with myelofibrosis (MF); the IPF and IRF were measured by the Sysmex XN analyzer...
February 28, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28245393/-mutation-of-calr-gene-in-patients-with-chronic-myeloproliferative-neoplasm-and-its-clinical-significance
#12
Qin Tang, Xiu-Wen Zhang, Lei Xia, Nai-Ke Jiang
OBJECTIVE: To analyze the CARL gene mutation in the patients with chronic myeloproliferative neoplasm(MPN) and to explore the clinical significance of CALR mutation. METHODS: The peripheral blood of patients was collected and the genomic DNA was exacted, the 9 exon of CALR gene and the fragment of human thrombopoetic receptor(MPL) gene were amplified by PCR, the mutation of CALR and MPL genes was detected by using the direct sequencing, the JAK2 V617F mutation was detected by using allele spicific PCR...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28241944/cerebrovascular-events-as-presenting-manifestations-of-myeloproliferative-neoplasm
#13
E Ong, F Barraco, N Nighoghossian, A Praire, V Desestret, L Derex, A Vighetto, D Biotti
AIM: To determine the incidence and main characteristics of cerebrovascular events as the presenting manifestations of myeloproliferative neoplasm (MPN). METHODS: The Hematology in Lyon (HEMILY) registry is a prospective database (763 patients) of all cases of MPN diagnosed since 2005 in the Rhône-Alpes district of France. The MPN cases were divided into four groups: polycythemia vera (PV); essential thrombocythemia (ET); myelofibrosis (MF); and atypical MPN. The ischemic stroke subtype was classified according to TOAST criteria...
November 2016: Revue Neurologique
https://www.readbyqxmd.com/read/28237966/drosophila-model-of-myeloproliferative-neoplasm-reveals-a-feed-forward-loop-in-the-jak-pathway-mediated-by-p38-mapk-signalling
#14
Ana Terriente-Félix, Lidia Pérez, Sarah J Bray, Angel R Nebreda, Marco Milán
Myeloproliferative neoplasms (MPNs) of the Philadelphia-negative class comprise polycythemia vera, essential thrombocythemia and primary myelofibrosis (PMF). They are associated with aberrant amounts of myeloid lineage cells in the blood, and in the case of overt PMF, with the development of myelofibrosis in the bone marrow and the failure to produce normal blood cells. These diseases are usually caused by gain-of-function mutations in the kinase JAK2 Here we use Drosophila to investigate the consequences of activation of the JAK2 ortholog in hematopoiesis...
February 24, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28237691/vegf-a-svegfr-1-and-svegfr-2-in-bcr-abl-negative-myeloproliferative-neoplasms
#15
Grażyna Gadomska, Katarzyna Stankowska, Joanna Boinska, Robert Ślusarz, Marzena Tylicka, Małgorzata Michalska, Anna Jachalska, Danuta Rość
BACKGROUND AND OBJECTIVE: Data from the literature indicate the relationship between the bone marrow microvessel density and the blood parameters of angiogenesis. The aim of this study was to evaluate selected parameters of angiogenesis (VEGF-A, sVEGFR-1, and sVEGFR-2) and their correlations with white blood cells, platelets, and red blood cells. MATERIALS AND METHODS: The study included 72 patients (mean age, 61.84 years) with myeloproliferative neoplasms (MPNs): essential thrombocythemia (ET) (n=46), polycythemia vera (PV) (n=19), and primary myelofibrosis (PMF) (n=7)...
February 2, 2017: Medicina
https://www.readbyqxmd.com/read/28228104/driver-mutations-jak2v617f-mplw515l-k-or-calr-pentraxin-3-and-c-reactive-protein-in-essential-thrombocythemia-and-polycythemia-vera
#16
Federico Lussana, Alessandra Carobbio, Silvia Salmoiraghi, Paola Guglielmelli, Alessandro Maria Vannucchi, Barbara Bottazzi, Roberto Leone, Alberto Mantovani, Tiziano Barbui, Alessandro Rambaldi
BACKGROUND: The driver mutations JAK2V617F, MPLW515L/K and CALR influence disease phenotype of myeloproliferative neoplasms (MPNs) and might sustain a condition of chronic inflammation. Pentraxin 3 (PTX3) and high-sensitivity C-reactive protein (hs-CRP) are inflammatory biomarkers potentially useful for refining prognostic classification of MPNs. METHODS: We evaluated 305 with essential thrombocythemia (ET) and 172 polycythemia vera (PV) patients diagnosed according to the 2016 WHO criteria and with full molecular characterization for driver mutations...
February 22, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28219560/a-novel-approach-to-essential-thrombocythemia-and-cardiac-surgery
#17
Bradford B Smith, Gregory A Nuttall, Rajiv K Pruthi, David L Joyce, Matthew S Schuldes, Mark M Smith
Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by persistent thrombocytosis (>450 × 10(9)/L). Patients with ET are at risk for thrombotic or hemorrhagic complications especially during cardiovascular operations. We discuss a patient with ET requiring coronary artery bypass grafting with a presenting platelet count of 1631 × 10(9)/L. Platelet reduction with an intraoperative autologous transfusion technique decreased the platelet count to 758 × 10(9)/L before initiation of cardiopulmonary bypass...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28219223/-jak2-v617f-positive-mutation-transformed-to-calreticulin-mutation-in-one-patient-with-essential-thrombocythemia
#18
S Y Wang, L J Zhang, Y X Liu, G M Liu, J Z Fu, F Y Wang, X L Xie, Z Y Cheng
No abstract text is available yet for this article.
January 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28211153/the-prognostic-relevance-of-serum-lactate-dehydrogenase-and-mild-bone-marrow-reticulin-fibrosis-in-essential-thrombocythemia
#19
Mythri Mudireddy, Daniela Barraco, Curtis A Hanson, Animesh Pardanani, Naseema Gangat, Ayalew Tefferi
The 2016 World Health Organization (WHO) diagnostic criteria for myeloproliferative neoplasms (MPN) underscore the prognostically-relevant distinction between essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (pre-PMF). In addition, leukocytosis has been identified as an important prognostic marker in otherwise WHO-defined ET. However, controversy remains regarding the objectivity of morphologic criteria in distinguishing ET from pre-PMF and the precise prognostic cutoff values for leukocytosis...
February 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28205126/estimation-of-diagnosis-and-prognosis-in-et-by-assessment-of-calr-and-jak2-v617f-mutations-and-laboratory-findings-a-meta-analysis
#20
REVIEW
N Saki, R Shirzad, F Rahim, A Saki Malehi
BACKGROUND: Essential thrombocythemia (ET) is a benign disease with slow progress in which thrombosis is a cause of mortality. JAK2(V617F) and calreticulin (CALR) are the most frequent mutations in this disease. In this systematic review and meta-analysis, we compared the prevalence of JAK2(V617F) and CALR mutations in ET and examined the incidence of thrombosis and other hematologic indices. METHODS: After choosing MeSH keywords, including essential thrombocythemia, JAK2(V617F), calreticulin, prognosis, and diagnosis, as well as searching Medline/PubMed and Scopus, 12 papers were selected...
February 15, 2017: Clinical & Translational Oncology
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