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Polycythemia vera

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https://www.readbyqxmd.com/read/29143068/patient-characteristics-and-outcomes-in-adolescents-and-young-adults-with-classical-philadelphia-chromosome-negative-myeloproliferative-neoplasms
#1
Prajwal Boddu, Lucia Masarova, Srdan Verstovsek, Paolo Strati, Hagop Kantarjian, Jorge Cortes, Zeev Estrov, Sherry Pierce, Naveen Pemmaraju
Little is known about the outcomes of Philadelphia-negative myeloproliferative neoplasms (MPNs) in adolescents and young adults (AYA). We reviewed all patients with essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF) treated at our institution from 1988 to 2016 who were aged 16 to 39 years (AYA) and described their outcomes in comparison to older MPN population. Of 2206 patients, 185 (8.3%) were identified as AYA: 105 (57%) ET, 43 (23%) PV, and 37 (20%) MF. The median age was 33 years [range, 16-39], and median follow-up time 3 years [range, 0...
November 15, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29128551/transplant-decisions-in-patients-with-myelofibrosis-should-mutations-be-the-judge
#2
REVIEW
Rachel B Salit, H Joachim Deeg
The prognosis of myeloproliferative neoplasms (MPN), including primary myelofibrosis (PMF), polycythemia vera (PV; post-PV MF) and essential thrombocythemia (ET; post-EMF) varies considerably, between these disorders as well as within each diagnosis. Molecular studies have identified "driver mutations", in JAK2, MPL1 and CALR, and additional somatic DNA mutations, including ASXL1, EZH2, IDH1/2 and SRSF2, that affect prognosis differentially. Patients with mutations in CALR (type1) have a better outlook than patients with mutations in JAK2 or MPL, while patients without any of the driver mutations (triple negative) have the shortest life expectancy...
November 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29121538/therapeutic-options-for-leukemic-transformation-in-patients-with-myeloproliferative-neoplasms
#3
REVIEW
Maliha Khan, Rabbia Siddiqi, Naseema Gangat
Approximately 5-10% of patients with Philadelphia chromosome negative myeloproliferative neoplasms (MPN) comprising of essential thrombocythemia, polycythemia vera and primary myelofibrosis) experience transformation to acute myeloid leukemia (AML, ≥20% blasts). Treatment options for post-MPN AML patients are limited, as conventional approaches like standard chemotherapy, fail to offer long-term benefit. Median survival for secondary AML is ∼2.4 months. Post-MPN AML therefore represents an area of urgent clinical need...
October 27, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29100304/germline-variations-at-jak2-tert-hbs1l-myb-and-mecom-and-the-risk-of-myeloproliferative-neoplasms-in-taiwanese-population
#4
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...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29090588/decreased-turnover-aspirin-resistance-by-bidaily-aspirin-intake-and-efficient-cytoreduction-in-myeloproliferative-neoplasms
#5
Andréas Perrier-Cornet, Jean-Christophe Ianotto, Fanny Mingant, Maëla Perrot, Eric Lippert, Hubert Galinat
Essential thrombocythemia (ET) and polycythemia vera (PV) are myeloproliferative neoplasms (MPN) with an increased risk of arterial and venous thrombosis. Aspirin is recommended to reduce this risk, but resistance to antiplatelet therapy seems to hamper its efficacy in some patients. We have previously shown that multiple electrode aggregometry (MEA) was a valuable tool to assess aspirin resistance in MPN. In this study, MEA was used to assess the reduction in aspirin resistance after bi-daily (BID) aspirin intake or cytoreduction...
November 1, 2017: Platelets
https://www.readbyqxmd.com/read/29074595/ruxolitinib-vs-best-available-therapy-for-et-intolerant-or-resistant-to-hydroxycarbamide
#6
RANDOMIZED CONTROLLED TRIAL
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, Robyn 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 2 trial of ruxolitinib (JAK1/2 inhibitor) vs best available therapy (BAT) in ET and polycythemia vera patients resistant or intolerant to HC. Here, findings of MAJIC-ET are reported, where the modified intention-to-treat population included 58 and 52 patients randomized to receive ruxolitinib or BAT, respectively...
October 26, 2017: Blood
https://www.readbyqxmd.com/read/29068570/the-role-of-certain-gene-polymorphisms-involved-in-the-apoptotic-pathways-in-polycythemia-vera-and-essential-thrombocytosis
#7
Gurbet Dogru, Ozlem Izci Ay, Mehmet Emin Erdal, Mustafa Ertan Ay, Anıl Tombak, Umit Karakas
BACKGROUND: Polycythemia vera (PV) and essential thrombocytosis (ET) are hematological disorders characterized by excessive production of mature and functional blood cells. These cellular disorders are thought to be associated with impaired apoptosis, which is one of the major cellular death mechanisms in hematopoietic cells. OBJECTIVES: In this study, our objective was to examine the association between potential polymorphisms of the Bcl 2, Bax, Fas and Fas Ligand genes involved in apoptosis and the occurrence of PV and ET...
August 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29066270/aquagenic-pruritus-in-polycythemia-vera-a-cross-sectional-study
#8
Edyta Lelonek, Łukasz Matusiak, Tomasz Wróbel, Jacek C Szepietowski
No abstract text is available yet for this article.
October 21, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29056075/an-updated-review-of-the-jak1-2-inhibitor-ruxolitininb-in-the-philadelphia-negative-myeloproliferative-neoplasms
#9
Natalia Curto-Garcia, Claire N Harrison
Ruxolitinib (Rux), a JAK1/2 inhibitor, has been approved for patients with myelofibrosis and in polycythemia vera with inadequate response/intolerance to hydroxycarbamide. Studies have demonstrated that Rux improves disease-related symptoms and splenomegaly. A late emerging observation from two Phase III trials was that Rux was associated with survival advantage in comparison with placebo or other available therapies in myelofibrosis. Important data suggest that for polycythemia vera Rux improved control of blood counts...
October 23, 2017: Future Oncology
https://www.readbyqxmd.com/read/29051279/expansion-of-epor-negative-macrophages-besides-erythroblasts-by-elevated-epor-signaling-in-erythrocytosis-mouse-models
#10
Jieyu Wang, Yoshihiro Hayashi, Asumi Yokota, Zefeng Xu, Yue Zhang, Rui Huang, Xiaomei Yan, Hongyun Liu, Liping Ma, Mohammad Azam, James P Bridges, Jose A Cancelas, Theodosia A Kalfa, Xiuli An, Zhijian Xiao, Gang Huang
Activated EPO receptor (EPOR) signaling causes erythrocytosis. The important role of macrophages for the erythroid expansion and differentiation process has been reported, both in baseline and stress erythropoiesis. However, the significance of EPOR signaling for regulation of macrophages contributing to erythropoiesis has not been fully understood. Here we show that EPOR signaling activation quickly expands both erythrocytes and macrophages in vivo in mouse models of primary and secondary erythrocytosis. To mimic the chimeric condition and expansion of the disease clone in the polycythemia vera patients, we combined Cre-inducible Jak2V617F/+ allele with LysM-Cre allele which expresses in mature myeloid cells and some of the HSC/Ps (LysM-Cre;Jak2V617F/+ mice)...
October 19, 2017: Haematologica
https://www.readbyqxmd.com/read/29047144/mecom-hbs1l-myb-thrb-rarb-jak2-and-tert-polymorphisms-defining-the-genetic-predisposition-to-myeloproliferative-neoplasms-a-study-on-939-patients
#11
Adrian P Trifa, Claudia Bănescu, Anca S Bojan, Cristian M Voina, Ștefana Popa, Simona Vișan, Alina D Ciubean, Florin Tripon, Delia Dima, Viola M Popov, Ștefan C Vesa, Mihaela Andreescu, Tünde Török-Vistai, Romeo G Mihăilă, Nicoleta Berbec, Ioan Macarie, Andrei Coliță, Maria Iordache, Alina C Cătană, Marius F Farcaș, Ciprian Tomuleasa, Kinga Vasile, Cristina Truică, Adriana Todincă, Lavinia Pop-Muntean, Raluca Manolache, Horia Bumbea, Ana-Maria Vlădăreanu, Mihaela Gaman, Cristina M Ciufu, Radu A Popp
Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical myeloproliferative neoplasms (MPN), characterized by specific somatic mutations in JAK2, CALR or MPL genes. JAK2 46/1 and TERT rs2736100 polymorphisms are known to significantly predispose to MPN. This study aimed to establish the additional contribution of the recently described MECOM rs2201862, HBS1L-MYB rs9376092 and THRB-RARB rs4858647 polymorphisms to the occurrence of MPN. These three polymorphisms, along with JAK2 46/1 and TERT rs2736100 were genotyped in 939 MPN patients (454 with ET, 337 with PV and 148 with PMF) and 483 controls...
October 19, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29040257/current-challenges-in-the-management-of-essential-thrombocythemia
#12
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/29034503/detection-of-a-new-heterozygous-germline-etv6-mutation-in-a-case-with-hyperdiploid-acute-lymphoblastic-leukemia-all
#13
Nicolas Duployez, Wadih Abou Chahla, Sophie Lejeune, Alice Marceau-Renaut, Guillaume Letizia, Thomas Boyer, Sandrine Geffroy, Pauline Peyrouze, Nathalie Grardel, Brigitte Nelken, Gérard Michel, Yves Bertrand, Claude Preudhomme
ETV6 is a target of recurrent aberrations in sporadic and familial acute lymphoblastic leukemia (ALL). Here, we report on a new pedigree with a germline ETV6 mutation in which the index patient and his father developed high-hyperdiploid (HeH)-ALL and polycythemia vera at age 13 and 51 respectively. The index patient achieved durable complete remission without transplantation but had persistent moderate thrombocytopenia without bleeding tendency. In order to determine the prevalence of ETV6 alterations in HeH-ALL, we screened 81 unrelated subjects with HeH-ALL by single nucleotide polymorphism-array and high-throughput sequencing for the ETV6 gene...
October 16, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29033774/hemothorax-following-uncomplicated-endoscopic-variceal-sclerotherapy-and-ligation-for-esophageal-varices
#14
Tomoko Ochiai, Yukiomi Nakade, Rena Kitano, Shunsuke Kato, Kazumasa Sakamoto, Tadahisa Inoue, Yuji Kobayashi, Norimitsu Ishii, Tomohiko Ohashi, Yoshio Sumida, Kiyoaki Ito, Haruhisa Nakao, Chihiro Furuta, Motoki Yano, Masashi Yoneda
Endoscopic variceal sclerotherapy and ligation are standard treatment modalities used for the management of esophageal varices. Reportedly, sclerotherapy and ligation are associated with complications such as hematuria, pulmonary thrombus formation, pleural effusion, renal dysfunction, and esophageal stenosis. However, hemothorax following sclerotherapy and ligation has not yet been reported. We treated a patient who presented with liver cirrhosis and polycythemia vera and later developed hemothorax following the above-mentioned procedures...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29032412/prognostication-and-initiation-of-therapy-in-polycythemia-vera-do-we-have-it-right
#15
REVIEW
Brady L Stein, Srdan Verstovsek
ᅟ.
October 14, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/29017808/a-novel-technique-for-duplex-guided-office-based-interventions-for-patients-with-acute-arteriovenous-fistula-occlusion
#16
Afsha Aurshina, Enrico Ascher, Anil Hingorani, Natalie Marks
OBJECTIVE: The purpose of the study was to aggressively salvage acutely occluded arteriovenous fistulas (AVFs) using duplex imaging as the sole imaging modality for percutaneous transluminal angioplasty of acutely thrombosed AVF. METHODS: Over a period of 12 months, 14 patients with acute thrombosis of their AVFs underwent 18 procedures in a single center for AVF salvage. All 14 patients presented with chronic renal failure, and six were diabetic. All patients were treated under duplex guidance alone...
October 7, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28990497/calr-jak2-and-mpl-mutation-status-in-argentinean-patients-with-bcr-abl1-negative-myeloproliferative-neoplasms
#17
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
#18
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/28956263/ruxolitinib-is-effective-and-safe-in-japanese-patients-with-hydroxyurea-resistant-or-hydroxyurea-intolerant-polycythemia-vera-with-splenomegaly
#19
Keita Kirito, Kenshi Suzuki, Koichi Miyamura, Masahiro Takeuchi, Hiroshi Handa, Shinichiro Okamoto, Brian Gadbaw, Kyosuke Yamauchi, Taro Amagasaki, Kazuo Ito, Masayuki Hino
Ruxolitinib, a potent JAK1/JAK2 inhibitor, was found to be superior to the best available therapy (BAT) in controlling hematocrit, reducing splenomegaly, and improving symptoms in the phase 3 RESPONSE study of patients with polycythemia vera with splenomegaly who experienced an inadequate response to or adverse effects from hydroxyurea. We report findings from a subgroup analysis of Japanese patients in RESPONSE (n = 18). The composite response rate (hematocrit control and spleen response) was higher in patients receiving ruxolitinib (50...
September 27, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28949941/polycythemia-vera-from-new-modified-diagnostic-criteria-to-new-therapeutic-approaches
#20
Margherita Maffioli, Barbara Mora, Francesco Passamonti
Polycythemia vera (PV) is a Philadelphia chromosome-negative chronic myeloproliferative neoplasm that is associated with a Janus kinase 2 (JAK2) mutation in most cases. The most recent update to the World Health Organization diagnostic criteria for PV was published in 2016. These were the modifications with the greatest effect: (1) lowering the hemoglobin threshold, allowing a diagnosis of PV at 16.5 g/dL in males and at 16.0 g/dL in females and (2) introducing a hematocrit cutoff (49% in males and 48% in females)...
September 2017: Clinical Advances in Hematology & Oncology: H&O
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