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https://www.readbyqxmd.com/read/28707720/efficacy-and-safety-of-anagrelide-in-childhood-essential-thrombocythemia
#1
Ayako Kanai, Hirokazu Morokawa, Kazuyuki Matsuda, Ei Shimazaki, Yozo Nakazawa
No abstract text is available yet for this article.
July 14, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28702412/megakaryocytic-morphology-in-janus-kinase-2-v617f-positive-myeloproliferative-neoplasm
#2
Shuchi Ghai, Sharada Rai
CONTEXT: Alterations in megakaryocyte morphology are the hallmark of myeloproliferative neoplasms (MPNs). These neoplasm are also associated with Janus kinase 2 (JAK2) V617F mutation in nearly 95% patients with polycythemia vera (PV), 40% patients of essential thrombocythemia (ET) and 50% patients of myelofibrosis (MF). The utility of megakaryocyte morphology in these disorders in correlation with JAK2 V617F remains unresolved. AIMS: The aim of the study was to assess the morphology of megakaryocytes in bone marrow aspirates (BMAs) and bone marrow biopsies of patients of BCR-ABL negative MPNs with JAK2 V617F mutation...
April 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28692109/acute-transmural-myocardial-infarction-by-coronary-embolism-in-a-patient-with-jak2-v617f-positive-essential-thrombocythemia
#3
Johannes Rotta Detto Loria, Justyna Rawluk, Tobias Krauss, Christoph Bode, Martin Moser, Thomas Helbing
No abstract text is available yet for this article.
July 10, 2017: Hämostaseologie
https://www.readbyqxmd.com/read/28688466/leukemic-transformation-in-myeloproliferative-neoplasms-a-literature-review-on-risk-characteristics-and-outcome
#4
REVIEW
Meera Yogarajah, Ayalew Tefferi
Myeloproliferative neoplasms (MPNs) operationally include essential thrombocythemia, polycythemia vera, primary myelofibrosis (PMF), and prefibrotic PMF. All 4 MPN variants might progress into blast-phase disease (MPN-BP). For essential thrombocythemia, reported risk factors for leukemic transformation include advanced age, extreme thrombocytosis, anemia, leukocytosis, and sequence variants/mutations involving TP53 and EZH2 (for expansion of gene symbols, see www.genenames.org); for polycythemia vera, advanced age, leukocytosis, abnormal karyotype, mutations involving SRSF2 and IDH2, and treatment with pipobroman, chlorambucil, or P32; and for PMF, increased blast percentage, thrombocytopenia, abnormal karyotype, triple-negative driver mutational status, and sequence variants/mutations involving SRSF2, RUNX1, CEBPA, and SH2B3...
July 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#5
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28676668/the-calreticulin-calr-exon-9-mutations-are-promising-targets-for-cancer-immune-therapy
#6
M O Holmström, E Martinenaite, S M Ahmad, Ö Met, C Friese, L Kjær, C H Riley, P Thor Straten, I M Svane, H C Hasselbalch, M H Andersen
The CALR exon 9 mutations are found in approximately 30% of patients with essential thrombocythemia and primary myelofibrosis. Recently, we reported spontaneous immune responses against the CALR mutations. Here, we describe that CALR-mutant (CALRmut)-specific T-cells are able to specifically recognize CALRmut cells. First, we established a T-cell culture specific for a CALRmut epitope. These specific T-cells were able to recognize several epitopes in the CALRmut C-terminus. Next, we established a CALRmut specific CD4(+) T-cell clone by limiting dilution...
July 5, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28669446/inflammation-and-myeloproliferative-neoplasms
#7
REVIEW
Federico Lussana, Alessandro Rambaldi
Myeloproliferative neoplasms (MPN) include three main entities: Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Myelofibrosis (MF). MPN represent a unique model of the relationship between the clonal development of a hematologic malignancy and chronic inflammation. The neoplastic clone is the main driver of this inflammatory reaction as demonstrated by the curative effect of allogeneic stem cell transplantation which leads not only to a complete restore of the hematopoiesis, but also to regression of bone marrow fibrosis...
June 29, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28662484/high-platelet-count-at-diagnosis-is-a-protective-factor-for-thrombosis-in-patients-with-essential-thrombocythemia
#8
Roberto Latagliata, Marco Montanaro, Michele Cedrone, Ambra Di Veroli, Francesca Spirito, Cristina Santoro, Sabrina Leonetti Crescenzi, Raffaele Porrini, Jonny Di Giandomenico, Nicoletta Villivà, Antonio Spadea, Angela Rago, Cinzia De Gregoris, Atelda Romano, Barbara Anaclerico, Marianna De Muro, Stefano Felici, Massimo Breccia, Enrico Montefusco, Antonino Bagnato, Giuseppe Cimino, Ignazio Majolino, Maria Gabriella Mazzucconi, Giuliana Alimena, Alessandro Andriani
To assess the role of platelet (PLT) count for thrombotic complications in Essential Thrombocythemia (ET), 1201 patients followed in 11 Hematological centers in the Latium region were retrospectively evaluated. At multivariate analysis, the following factors at diagnosis were predictive for a worse Thrombosis-free Survival (TFS): the occurrence of previous thrombotic events (p=0.0004), age>60years (p=0.0044), spleen enlargement (p=0.042) and a lower PLT count (p=0.03). Receiver Operating Characteristic (ROC) analyses based on thrombotic events during follow-up identified a baseline platelet count of 944×10(9)/l as the best predictive threshold: thrombotic events were 40/384 (10...
June 21, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28655032/age-related-macular-degeneration-in-patients-with-chronic-myeloproliferative-neoplasms
#9
Marie Bak, Torben Lykke Sørensen, Esben Meulengracht Flachs, Ann-Dorthe Zwisler, Knud Juel, Henrik Frederiksen, Hans Carl Hasselbalch
Importance: It has been suggested that systemic inflammation increases the risk of age-related macular degeneration (AMD). Given that chronic immune modulation is present in patients with myeloproliferative neoplasms (MPNs), the risk of AMD in these patients may be increased. Objective: To compare the risk of AMD in patients with MPNs with the risk of AMD in matched controls from the general population. Design, Setting, and Participants: A nationwide population-based cohort study using Danish registers was conducted of all patients in Denmark who received a diagnosis between January 1, 1994, and December 31, 2013, of essential thrombocythemia, polycythemia vera, myelofibrosis, or unclassifiable MPNs...
June 22, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28654952/are-antiplatelet-agents-beneficial-in-essential-thrombocythemia-maybe-yes-probably-no
#10
Joel S Bennett
No abstract text is available yet for this article.
June 27, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28653329/acquired-rhd-mosaicism-identifies-fibrotic-transformation-of-thrombopoietin-receptor-mutated-essential-thrombocythemia
#11
Celina Montemayor-Garcia, Rebecca Coward, Maher Albitar, Rupa Udani, Prachi Jain, Eleftheria Koklanaris, Minoo Battiwalla, Siobán Keel, Harvey G Klein, A John Barrett, Sawa Ito
BACKGROUND: Acquired copy-neutral loss of heterozygosity has been described in myeloid malignant progression with an otherwise normal karyotype. CASE REPORT: A 65-year-old woman with MPL-mutated essential thrombocythemia and progression to myelofibrosis was noted upon routine pretransplant testing to have mixed field reactivity with anti-D and an historic discrepancy in RhD type. The patient had never received transfusions or transplantation. RESULTS: Gel immunoagglutination revealed group A red blood cells and a mixed-field reaction for the D phenotype, with a predominant D-negative population and a small subset of circulating red blood cells carrying the D antigen...
June 26, 2017: Transfusion
https://www.readbyqxmd.com/read/28641646/-clinical-characteristics-and-long-term-outcome-of-125-chinese-young-patients-with-essential-thrombocythemia
#12
Rong-Feng Fu, Xiao-Fan Liu, Wei Liu, Yue-Ting Huang, Yun-Fei Chen, Hui-Yuan Li, Man-Kai Ju, Ren-Chi Yang, Lei Zhang
OBJECTIVE: To investigate the clinical characteristics and long-term outcome of Chinese young patients (≤40 years) with essential thrombocythemia(ET), and to develop a thrombosis predicting model specific for young patients with ET, so as to provide a new evidence for risk stratification and treatment. METHODS: Medical records of 125 Chinese young patients with newly diagnosed of ET were retrospectively analyzed. RESULTS: The median age at diagnosis was 32 (18-40) years old, with 37 males and 88 females...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28632284/benefits-and-risks-of-antithrombotic-therapy-in-essential-thrombocythemia-a-systematic-review
#13
Derek K Chu, Christopher M Hillis, Darryl P Leong, Sonia S Anand, Deborah M Siegal
Background: Patients with essential thrombocythemia (ET) are at high risk for both thrombosis and hemorrhage. Purpose: To evaluate the risks and benefits of antithrombotic therapy in adults with ET. Data Sources: Multiple databases, including MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials, through 4 March 2017. Study Selection: Randomized and observational studies of antiplatelet or anticoagulant therapy, published in any language and reporting thrombotic or hemorrhagic events...
June 27, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28622623/a-phase-2-study-of-momelotinib-a-potent-jak1-and-jak2-inhibitor-in-patients-with-polycythemia-vera-or-essential-thrombocythemia
#14
Srdan Verstovsek, Stephane Courby, Martin Griesshammer, Ruben A Mesa, Carrie Baker Brachmann, Jun Kawashima, Julia D Maltzman, Lixin Shao, Yan Xin, Daniel Huang, Ashish Bajel
Momelotinib is a potent inhibitor of JAK1 and JAK2 that demonstrated efficacy in patients with primary and secondary myelofibrosis. This phase 2, open-label, randomized study evaluated the efficacy and safety of oral once-daily momelotinib (100mg and 200mg) for the treatment of polycythemia vera (PV) and essential thrombocythemia (ET). The primary endpoint for PV was overall response rate (ORR), defined as the proportion of patients with hematocrit <45%, white blood cell count <10×10(9)/L, platelet count ≤400×10(9)/L, and resolution of palpable splenomegaly, each lasting ≥4 weeks...
May 30, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28604261/genomics-of-clonal-evolution-in-a-case-of-essential-thrombocythemia
#15
Armin Rashidi, Andrew C Nelson, Michael A Linden, Daniel J Weisdorf, Michelle M Dolan, Celalettin Ustun
No abstract text is available yet for this article.
June 11, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28601551/patients-with-post-essential-thrombocythemia-and-post-polycythemia-vera-differ-from-patients-with-primary-myelofibrosis
#16
Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Kate J Newberry, Taghi Manshouri, Jorge Cortes, Hagop M Kantarjian, Srdan Verstovsek
Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56% (n=616) patients had died...
June 2, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28589759/leukemic-transformation-in-patients-with-myeloproliferative-neoplasms-a-population-based-retrospective-study
#17
Rajesh Shrestha, Smith Giri, James O Armitage, Vijaya Raj Bhatt
AIM: This study determined the epidemiology of developing leukemic transformation in patients with myeloproliferative neoplasms (MPN). METHODS: We utilized the Surveillance, Epidemiology and End Results 13 database to identify 83 cases of leukemic transformation in MPN (n = 9335). RESULTS: The 5-year cumulative incidence of leukemic transformation was higher in male versus female (2.17 vs 1.09%, p < 0.001), and in myelofibrosis (2.19%; 95% CI: 1...
June 7, 2017: Future Oncology
https://www.readbyqxmd.com/read/28589084/calreticulin-in-essential-thrombocythemia-stressing-out-the-megakaryocyte-nucleus
#18
Francisco Jose Iborra, Petros Papadopoulos
Calreticulin (CALR) is a multifaceted protein primarily involved in intracellular protein control processes. The identification of CALR mutations in essential thrombocythemia (ET) and primary myelofibrosis that are mutually exclusive with the JAK2 V617F mutation has stirred an intensive research interest about the molecular functions of CALR and its mutants in myeloproliferative neoplasms (MPNs) and its diagnostic/prognostic value. The recently characterized protein-protein interaction of CALR mutants and MPL receptor has advanced our knowledge on the functional role of CALR mutants in thrombocythemia but it has also uncovered limitations of the current established research models...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28587547/a-mutation-in-ptpn11-may-drive-leukemic-transformation-in-a-case-of-essential-thrombocythemia
#19
Belinda B Guo, James Liang, Richard J N Allcock, Bob Mirzai, Bradley Augustson, Rebecca Howman, Kathryn A Fuller, Wendy N Erber
No abstract text is available yet for this article.
June 7, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28585070/thromboses-and-hemorrhages-are-common-in-mpn-patients-with-high-jak2v617f-allele-burden
#20
Irene Bertozzi, Giulia Bogoni, Giacomo Biagetti, Elena Duner, Anna Maria Lombardi, Fabrizio Fabris, Maria Luigia Randi
The most common causes of morbidity and mortality in myeloproliferative neoplasms (MPN) are thrombotic and hemorrhagic complications. The JAK2V617F mutation, commonly found in MPN, correlates with several clinical and laboratory characteristics even if the relevance of JAK2V617F allele burden in the natural history of these diseases is unclear. In this study we searched, a relation between thrombotic and hemorrhagic complications and JAK2V617F allele burden level in MPN patients. We evaluated 253 consecutive MPN [121 essential thrombocythemia (ET), 124 polycythemia vera (PV), and 8 primary myelofibrosis (PMF)] patients in whom the JAK2V617F allele burden was available, all studied and followed (median 8...
June 6, 2017: Annals of Hematology
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