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Essential Thrombocythemia

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https://www.readbyqxmd.com/read/29040257/current-challenges-in-the-management-of-essential-thrombocythemia
#1
Ariel Kleman, Arun K Singavi, Laura C Michaelis
Essential thrombocythemia (ET), an uncommon blood cancer, is one of the classic myeloproliferative neoplasms, a category that also includes polycythemia vera and primary myelofibrosis. All 3 diseases are clonal hematopoietic stem cell disorders. Since 2005, when scientists discovered a molecular aberration driving clonal hematopoiesis in polycythemia vera, our understanding of the genomic underpinnings of these conditions has increased rapidly. Over the last decades, primary prevention of thrombotic and hemorrhagic complications has improved the lives of patients with ET, and the ability to characterize the disease by the presence or absence of molecular mutations has lent precision to our prognostic models...
October 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29022213/high-prevalence-of-heparin-induced-thrombocytopenia-with-thrombosis-among-patients-with-essential-thrombocytemia-carrying-v617f-mutation
#2
Roberto Castelli, Paolo Gallipoli, Riccardo Schiavon, Thomas Teatini, Giorgio Lambertenghi Deliliers, Luigi Bergamaschini
Arterial and venous complications are major causes of morbidity and mortality in myeloproliferative neoplasms (MPNs). MPNs patients, frequently receive heparin. Heparin-induced thrombocytopenia (HIT) is a rare but potentially life-threatening complication resulting in a severe acquired thrombophilic condition. We carried out a retrospective analysis to evaluate occurrence of new thrombotic events during heparin therapy in essential thrombocythemia (ET) patients. We studied 108 ET patients on heparin for treatment of previous thrombotic events or in thromboprophilaxis...
October 11, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28990497/calr-jak2-and-mpl-mutation-status-in-argentinean-patients-with-bcr-abl1-negative-myeloproliferative-neoplasms
#3
Mara Jorgelina Ojeda, Irma Margarita Bragós, Karina Lucrecia Calvo, Gladis Marcela Williams, María Magdalena Carbonell, Arianna Flavia Pratti
OBJECTIVES: To establish the frequency of JAK2, MPL and CALR mutations in Argentinean patients with BCR-ABL1-negative  myeloproliferative neoplasms (MPN) and to compare their clinical and haematological features. METHODS: Mutations of JAK2V617F, JAK2 exon 12, MPL W515L/K and CALR were analysed in 439 Argentinean patients with BCR-ABL1-negative MPN, including 176 polycythemia vera (PV), 214 essential thrombocythemia (ET) and 49 primary myelofibrosis (PMF). RESULTS: In 94...
October 9, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28982745/nccn-guidelines-insights-myeloproliferative-neoplasms-version-2-2018
#4
Ruben A Mesa, Catriona Jamieson, Ravi Bhatia, Michael W Deininger, Christopher D Fletcher, Aaron T Gerds, Ivana Gojo, Jason Gotlib, Krishna Gundabolu, Gabriela Hobbs, Brandon McMahon, Sanjay R Mohan, Stephen Oh, Eric Padron, Nikolaos Papadantonakis, Philip Pancari, Nikolai Podoltsev, Raajit Rampal, Erik Ranheim, Vishnu Reddy, Lindsay A M Rein, Bart Scott, David S Snyder, Brady L Stein, Moshe Talpaz, Srdan Verstovsek, Martha Wadleigh, Eunice S Wang, Mary Anne Bergman, Kristina M Gregory, Hema Sundar
Myeloproliferative neoplasms (MPNs) are a group of heterogeneous disorders of the hematopoietic system that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). PV and ET are characterized by significant thrombohemorrhagic complications and a high risk of transformation to MF and acute myeloid leukemia. The diagnosis and management of PV and ET has evolved since the identification of mutations implicated in their pathogenesis. These NCCN Guideline Insights discuss the recommendations outlined in the NCCN Guidelines for the risk stratification, treatment, and special considerations for the management of PV and ET...
October 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28948488/prognostication-in-philadelphia-chromosome-negative-myeloproliferative-neoplasms-a-review-of-the-recent-literature
#5
REVIEW
Amy Zhou, Amber Afzal, Stephen T Oh
PURPOSE OF REVIEW: The prognosis for patients with Philadelphia chromosome (Ph)-negative myeloproliferative neoplasms (MPNs) is highly variable. All Ph-negative MPNs carry an increased risk for thrombotic complications, bleeding, and leukemic transformation. Several clinical, biological, and molecular prognostic factors have been identified in recent years, which provide important information in guiding management of patients with Ph-negative MPNs. In this review, we critically evaluate the recent published literature and discuss important new developments in clinical and molecular factors that impact survival, disease transformation, and thrombosis in patients with polycythemia vera, essential thrombocythemia, and primary myelofibrosis...
September 25, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28948454/molecular-pathogenesis-of-myeloproliferative-neoplasms-influence-of-age-and-gender
#6
REVIEW
Jeffrey Patterson-Fortin, Alison R Moliterno
The myeloproliferative neoplasms polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) display distinct clinical and pathologic features but are characterized by mutations in JAK2, MPL, and CALR leading to activation of the JAK-STAT pathway. This review addresses the pathogenesis and mechanisms of these mutant alleles and the unique interactions of both of age and gender.
September 25, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28934680/a-phase-1-study-of-the-janus-kinase-2-jak2-v617f-inhibitor-gandotinib-ly2784544-in-patients-with-primary-myelofibrosis-polycythemia-vera-and-essential-thrombocythemia
#7
MULTICENTER STUDY
Srdan Verstovsek, Ruben A Mesa, Mohamed E Salama, Li Li, Celine Pitou, Fabio P Nunes, Gregory L Price, Jennifer L Giles, Deborah N D'Souza, Richard A Walgren, Josef T Prchal
Mutations in Janus kinase 2 (JAK2) are implicated in the pathogenesis of Philadelphia-chromosome negative myeloproliferative neoplasms, including primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Gandotinib (LY2784544), a potent inhibitor of JAK2 activity, shows increased potency for the JAK2(V617F) mutation. The study had a standard 3+3 dose-escalation design to define the maximum-tolerated dose. Primary objectives were to determine safety, tolerability, and recommended oral daily dose of gandotinib for patients with JAK2(V617F)-positive myelofibrosis, essential thrombocythemia, or polycythemia vera...
October 2017: Leukemia Research
https://www.readbyqxmd.com/read/28914569/mutations-in-myeloproliferative-neoplasms-their-significance-and-clinical-use
#8
Fiorella Schischlik, Robert Kralovics
Clonal hematologic diseases of the blood such as polycythemia vera, essential thrombocythemia and primary myelofibrosis belong to the BCR-ABL negative Myeloproliferative Neoplasms (MPN). These diseases are characterized by clonal expansion of hematopoietic precursor cells followed by increased production of differentiated cells of the myeloid lineage. Initiation of clonal hematopoiesis, formation of a clinical phenotype as well as disease progression form part of MPN disease evolution. The disease is driven by acquired somatic mutations in critical pathways such as cytokine signaling, epigenetic regulation, RNA splicing, and transcription factor signaling...
September 25, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28883275/successful-management-of-intraperitoneal-bleeding-with-platelet-apheresis-and-von-willebrand-factor-supplementation-in-a-patient-with-essential-thrombocythemia-and-acquired-von-willebrand-syndrome
#9
Toshimitsu Ueki, Kanako Takeshige, Masahiko Sumi, Mayumi Ueno, Naoaki Ichikawa, Hikaru Kobayashi
A 36-year-old woman with essential thrombocythemia (ET) was admitted to our hospital for acute lower abdominal pain. Given no family history of bleeding disorder, she was diagnosed with acquired von Willebrand syndrome. Despite having a medical history of venous thrombosis, she had never been treated for ET because of her preferences. On admission, CT scan revealed massive hemorrhage in the ascending colon with the leakage of a contrast agent. Furthermore, a delayed enhancement of fluid collection in the Douglas fossa followingcontrast CT indicated bloody ascites...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28827411/ruxolitinib-for-essential-thrombocythemia-refractory-to-or-intolerant-of-hydroxyurea-long-term-phase-2-study-results
#10
LETTER
Srdan Verstovsek, Francesco Passamonti, Alessandro Rambaldi, Giovanni Barosi, Elisa Rumi, Elisabetta Gattoni, Lisa Pieri, Huiling Zhen, Muriel Granier, Albert Assad, Mario Cazzola, Hagop M Kantarjian, Tiziano Barbui, Alessandro M Vannucchi
No abstract text is available yet for this article.
October 12, 2017: Blood
https://www.readbyqxmd.com/read/28819279/application-of-current-prognostic-models-for-primary-myelofibrosis-in-the-setting-of-post-polycythemia-vera-or-post-essential-thrombocythemia-myelofibrosis
#11
A Tefferi, L Saeed, C A Hanson, R P Ketterling, A Pardanani, N Gangat
No abstract text is available yet for this article.
August 18, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28818981/putti-mc-pizzi-m-bertozzi-i-et-al-bone-marrow-histology-for-the-diagnosis-of-essential-thrombocythemia-in-children-a-multicenter-italian-study-blood-2017-129-22-3040-3042
#12
https://www.readbyqxmd.com/read/28808591/immune-thrombocytopenia-and-jak2v617f-positive-essential-thrombocythemia-literature-review-and-case-report
#13
M A Sobas, T Wróbel, K Zduniak, M Podolak-Dawidziak, J Rybka, M Biedroń, M Sawicki, J Dybko, K Kuliczkowski
We present the case where immune thrombocytopenia (ITP) and essential thrombocythemia (ET) sequentially appeared in the space of twenty-one years of follow-up. Impaired platelet production is present in both diseases, but clinical presentation and treatment are different. On the basis of this case history a possible role of autoimmunity as a predisposing factor to myeloproliferation has been discussed.
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28795425/gender-and-survival-in-essential-thrombocythemia-a-two-center-study-of-1-494-patients
#14
Ayalew Tefferi, Silvia Betti, Daniela Barraco, Mythri Mudireddy, Sahrish Shah, Curtis A Hanson, Rhett P Ketterling, Animesh Pardanani, Naseema Gangat, Giacomo Coltro, Paola Guglielmelli, Alessandro M Vannucchi
Based on suggestive information from recent epidemiologic data and earlier retrospective studies, we revisited the effect of gender on survival in 1,494 patients with essential thrombocythemia (ET). The primary study population included 904 patients from the Mayo Clinic (median age 58 years; 65% females); risk distribution, according to the international prognostic score for ET (IPSET), was 23% high, 42% intermediate and 35% low. Multivariable analysis that included IPSET-relevant risk factors identified male sex (HR 1...
August 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28794072/ruxolitinib-versus-best-available-therapy-for-et-intolerant-or-resistant-to-hydroxycarbamide-in-a-randomized-trial
#15
Claire N Harrison, Adam J Mead, Anesh Panchal, Sonia Fox, Christina Yap, Emmanouela Gbandi, Aimee Houlton, Samah Alimam, Joanne Ewing, Marion Wood, Frederick Chen, Jason Coppell, Nicki Panoskaltsis, Steven Knapper, Sahra Ali, Angela Hamblin, Ruben Scherber, Amylou C Dueck, Nicholas C P Cross, Ruben Mesa, Mary Frances McMullin
Treatments for high-risk essential thrombocythemia (ET) address thrombocytosis, disease-related symptoms, as well as risks of thrombosis, hemorrhage, transformation to myelofibrosis and leukemia. Patients resistant/intolerant to hydroxycarbamide (HC) have a poor outlook. MAJIC (ISRCTN61925716) is a randomized phase II trial of ruxolitinib (JAK1/2 inhibitor) vs Best Available Therapy (BAT) in ET and polycythemia vera (PV) patients resistant or intolerant to HC. Here findings of MAJIC-ET are reported, where the modified intention-to-treat population included 58 & 52 patients randomized to receive ruxolitinib or BAT respectively...
August 9, 2017: Blood
https://www.readbyqxmd.com/read/28780729/the-impact-of-myeloproliferative-neoplasms-mpns-on-patient-quality-of-life-and-productivity-results-from-the-international-mpn-landmark-survey
#16
Claire N Harrison, Steffen Koschmieder, Lynda Foltz, Paola Guglielmelli, Tina Flindt, Michael Koehler, Jonathan Mathias, Norio Komatsu, Robert N Boothroyd, Amber Spierer, Julian Perez Ronco, Gavin Taylor-Stokes, John Waller, Ruben A Mesa
Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are myeloproliferative neoplasms (MPNs) associated with high disease burden, reduced quality of life (QOL), and shortened survival. To assess how MPNs affect patients, we conducted a global MPN Landmark survey. This online survey of patients with MPNs and physicians was conducted in Australia, Canada, Germany, Japan, Italy, and the United Kingdom. The survey measured MPN-related symptoms and the impact of MPNs on QOL and the ability to work as well as disease-management strategies...
August 5, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28780601/elevated-plasma-levels-of-procoagulant-microparticles-are-a-novel-risk-factor-for-thrombosis-in-patients-with-myeloproliferative-neoplasms
#17
Yasuhiro Taniguchi, Hirokazu Tanaka, Espinoza J Luis, Kazuko Sakai, Takahiro Kumode, Keigo Sano, Kentarou Serizawa, Shinya Rai, Yasuyoshi Morita, Hitoshi Hanamoto, Kazuo Tsubaki, Kazuto Nishio, Itaru Matsumura
Myeloproliferative neoplasms (MPNs), including polycythemia vera and essential thrombocythemia, are frequently associated with thrombotic complications. Prevention of thrombotic events is thus a primary aim of the current treatment for these disorders. Although it is known that microparticles (MPs), which are small vesicles released from cell membranes and circulate in the blood, directly contribute to thrombosis via their procoagulant activity, potential associations between plasma levels of MPs and the risk of thrombotic events in MPNs have not been reported...
August 5, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28766534/-clinical-features-of-essential-thrombocythemia-and-primary-myelofibrosis-depending-on-the-molecular-characteristics-of-disease
#18
A L Melikyan, I N Subortseva, A B Sudarikov, A M Kovrigina, E A Gilyazitdinova, T I Kolosheinova, A O Abdullaev, S A Treglazova
The aim of the present paper was to evaluate the clinical features and risk of thrombotic events (TE) in patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF), depending on the molecular characteristics of disease. Clinical data and laboratory parameters were analyzed in 50 ET patients and 50 PMF ones who had been followed up at the Department for Standardization of Treatments, National Research Center for Hematology, Ministry of Health of the Russian Federation, from February 2015 to September 2016...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28760302/developmental-therapeutics-in-myeloproliferative-neoplasms
#19
REVIEW
Prithviraj Bose, Srdan Verstovsek
The unprecedented success of the Janus kinase (JAK) 1/2 inhibitor ruxolitinib in myelofibrosis (MF) provided much-needed impetus for clinical drug development for the Philadelphia chromosome-negative myeloproliferative neoplasms. The survival benefit conferred by this agent, along with its marked efficacy with regard to spleen volume and symptom reduction, have made ruxolitinib the cornerstone of drug therapy in MF. However, there remain significant unmet needs in the treatment of patients with MF, and many novel classes of agents continue to be investigated in efforts to build on the progress made with ruxolitinib...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28747560/germline-variations-at-jak2-tert-hbs1l-myb-and-mecom-and-the-risk-of-myeloproliferative-neoplasms-in-taiwanese-population
#20
Yi-Hao Chiang, Yu-Cheng Chang, Huan-Chau Lin, Ling Huang, Chun-Chia Cheng, Wei-Ting Wang, Hung-I Cheng, Nai-Wen Su, Caleb Gon-Shen Chen, Johnson Lin, Yi-Fang Chang, Ming-Chih Chang, Ruey-Kuen Hsieh, Wen-Chien Chou, Ken-Hong Lim, Yuan-Yeh Kuo
Germline variations at JAK2, TERT, HBS1L-MYB and MECOM have been found to associate with myeloproliferative neoplasms (MPNs) in European populations. Whether these germline variations are associated with MPNs in Taiwanese population is obscure. Here we aimed to evaluate the association of five germline variations (JAK2 46/1 haplotype tagged by rs12343867, JAK2 intron 8 rs12339666, TERT rs2736100, HBS1L-MYB rs9376092 and MECOM rs2201862) and the risk of MPNs in Taiwanese population. A total of 178 MPN patients (109 essential thrombocythemia, 54 polycythemia vera and 15 primary myelofibrosis) were enrolled into this study...
July 12, 2017: Oncotarget
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