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https://www.readbyqxmd.com/read/27913526/update-from-the-latest-who-classification-of-mpns-a-user-s-manual
#1
Francesco Passamonti, Margherita Maffioli
The 2016 multiparameter World Health Organization (WHO) classification for Philadelphia-negative myeloproliferative neoplasms (MPNs) integrates clinical features, morphology, and genetic data to diagnose polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The main novelties are: (1) the reduction of the hemoglobin (Hb) level threshold to diagnose PV, now established at 16.5 g/dL for men and 16 g/dL for women (based on the identification of MPN patients with PV-consistent bone marrow [BM] features and a Hb level lower than that established in the 2008 WHO classification for PV); (2) the recognition of prefibrotic/early PMF, distinguishable from ET on the basis of BM morphology, an entity having a higher tendency to develop overt myelofibrosis or acute leukemia, and characterized by inferior survival; (3) the central role of BM morphology in the diagnosis of ET, prefibrotic/early PMF, PMF, and PV with borderline Hb values; megakaryocyte number and morphology (typical in ET, atypical in both PMF forms) accompanied by a new distinction of reticulin fibrosis grade in PMF (grade 1 in prefibrotic/early PMF and grade 2-3 in PMF) constitute diagnostic criteria; and (4) the inclusion of all mutually exclusive MPN driver mutations (JAK2, CALR, and MPL) as major diagnostic criteria in ET and PMF; 10% to 15% of these patients are triple negative, and in these cases the search for an additional clonal marker (eg, mutations in ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, and SF3B1) is warranted...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27911124/the-impact-of-interferon-alpha2-on-hla-genes-in-patients-with-polycythemia-vera-and-related-neoplasms
#2
Vibe Skov, Caroline Hasselbalch Riley, Mads Thomassen, Lasse Kjær, Thomas Stauffer Larsen, Ole Weis Bjerrum, Torben A Kruse, Hans Carl Hasselbalch
Gene expression profiling in Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) have unraveled significant deregulation of several immune and inflammation genes of potential importance for clonal evolution. Other mechanisms might be downregulation of major histocompatibility class I and II genes used by tumor cells to escape antitumor T-cell-mediated immune responses. Several genes encoding human leukocyte antigen (HLA) class I and II molecules have been shown to be significantly downregulated...
December 2, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27892678/characterization-and-prognosis-significance-of-jak2-v617f-mpl-and-calr-mutations-in-philadelphia-negative-myeloproliferative-neoplasms
#3
Roongrudee Singdong, Teerapong Siriboonpiputtana, Takol Chareonsirisuthigul, Adcharee Kongruang, Nittaya Limsuwanachot, Tanasan Sirirat, Suporn Chuncharunee, Budsaba Rerkamnuaychoke
Background: The discovery of somatic acquired mutations of JAK2 (V617F) in Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) has not only improved rational disease classification and prognostication but also brings new understanding insight into the pathogenesis of diseases. Dosage effects of the JAK2 (V617F) allelic burden in Ph-negative MPNs may partially influence clinical presentation, disease progression, and treatment outcome...
January 10, 2016: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/27892526/comprehensive-gene-expression-meta-analysis-and-integrated-bioinformatic-approaches-reveal-shared-signatures-between-thrombosis-and-myeloproliferative-disorders
#4
Prabhash Kumar Jha, Aatira Vijay, Anita Sahu, Mohammad Zahid Ashraf
Thrombosis is a leading cause of morbidity and mortality in patients with myeloproliferative disorders (MPDs), particularly polycythemia vera (PV) and essential thrombocythemia (ET). Despite the attempts to establish a link between them, the shared biological mechanisms are yet to be characterized. An integrated gene expression meta-analysis of five independent publicly available microarray data of the three diseases was conducted to identify shared gene expression signatures and overlapping biological processes...
November 28, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27884974/from-leeches-to-personalized-medicine-evolving-concepts-in-the-management-of-polycythemia-vera
#5
Alessandro Maria Vannucchi
Polycythemia vera (PV), a clonal disorders of hematopoietic stem/progenitor cells that manifests with prevalent expansion of red cell mass, is the most frequent among chronic myeloproliferative neoplasms (MPN). It is characterized by a V617F point mutation in JAK2 exon 14 , or less common mutations in exon 12, in virtually all cases. The landmark discovery of autonomously activated JAK/STAT signaling pathway paved the way for the clinical development of the first target drug, the JAK1 and JAK2 inhibitor ruxolitinib, that is now approved for patients with resistance or intolerance to hydroxyurea...
November 24, 2016: Haematologica
https://www.readbyqxmd.com/read/27880982/safety-and-efficacy-of-ruxolitinib-in-splanchnic-vein-thrombosis-associated-with-myeloproliferative-neoplasms
#6
Lisa Pieri, Chiara Paoli, Umberto Arena, Fabio Marra, Fabio Mori, Mery Zucchini, Stefano Colagrande, Alessandro Castellani, Arianna Masciulli, Vittorio Rosti, Valerio De Stefano, Silvia Betti, Guido Finazzi, Maria Luisa Ferrari, Elisa Rumi, Marco Ruggeri, Ilaria Nichele, Paola Guglielmelli, Rajmonda Fjerza, Carmela Mannarelli, Tiziana Fanelli, Lucia Merli, Giuditta Corbizi Fattori, Margherita Massa, Giuseppe Cimino, Alessandro Rambaldi, Giovanni Barosi, Mario Cazzola, Tiziano Barbui, Alessandro M Vannucchi
Splanchnic vein thrombosis (SVT) is one of the vascular complications of myeloproliferative neoplasms (MPN). We designed a phase 2 clinical trial to evaluate safety and efficacy of ruxolitinib in reducing splenomegaly and improving disease-related symptoms in patients with MPN-associated SVT. Patients, diagnosed with myelofibrosis in 12 cases, polycythemia vera in 5 and essential thrombocythemia in 4, received ruxolitinib for 24 weeks in the core study period. Spleen volume was assessed by magnetic resonance imaging (MRI) and splanchnic vein circulation by echo-Doppler analysis...
November 23, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27875375/management-of-venous-thromboembolism-in-myeloproliferative-neoplasms
#7
Tiziano Barbui, Valerio De Stefano
PURPOSE OF REVIEW: Venous thromboembolism is frequent in chronic myeloproliferative neoplasms (MPN). The current strategy for its management includes cytoreductive therapy and antithrombotic prophylaxis, but many issues remain uncertain. In this review, the risk factors and prevention of recurrences will be discussed. RECENT FINDINGS: Around one-third of patients with polycythemia vera and essential thrombocythemia experience a major thrombosis at diagnosis or during follow-up...
November 21, 2016: Current Opinion in Hematology
https://www.readbyqxmd.com/read/27866579/polycythemia-and-thrombocytosis
#8
REVIEW
Aric Parnes, Arvind Ravi
Myeloproliferative neoplasms (MPNs) are diseases of excess cell proliferation from bone marrow precursors. Two classic MPNs, polycythemia vera (PV) and essential thrombocytosis (ET), are conditions of excess proliferation of red blood cells and platelets, respectively. Although PV and ET involve different cells in the myeloid lineage, their clinical presentations have shared features, consistent with overlapping mutations in growth factor signaling. The management of both diseases involves minimizing the risk of thrombotic and hemorrhagic complications...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27858230/efficacy-and-safety-of-jak-inhibitor-inc424-in-patients-with-primary-and-post-polycythemia-vera-or-post-essential-thrombocythemia-myelofibrosis-in-the-chinese-population
#9
Xin Du, Daobin Zhou
A phase II study (A2202) was performed to evaluate the efficacy and safety of JAK inhibitor ruxolitinib in 63 Chinese MF patients. Ruxolitinib was given twice a day (bid) at a starting dose of 15 mg (n = 25) or 20 mg (n = 38) based on a baseline platelet count. About 94.7% of the patients achieved a reduction in spleen size, 27.0% of which exhibited significant reduction (≥ 35%) at week 24. Significant improvement in debilitating constitutional symptoms, as assessed by MFSAF v2.0, was observed in patients treated with ruxolitinib...
November 17, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27848280/intra-arterial-thrombolysis-of-digital-artery-occlusions-in-a-patient-with-polycythemia-vera
#10
Philipp Jud, Franz Hafner, Thomas Gary, Leyla Ghanim, Rainer Lipp, Marianne Brodmann
There are limited therapeutic options for the resolution of digital artery occlusions. Intra-arterial thrombolysis with anticoagulative and thrombolytic drugs successfully restored the blood flow in the affected digital arteries.
November 18, 2016: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/27830999/the-potential-role-of-hematocrit-control-on-symptom-burden-among-polycythemia-vera-patients-insights-from-the-cyto-pv-and-mpn-saf-patient-cohorts
#11
Robyn Marie Scherber, Holly Lynn Geyer, Amylou C Dueck, Heidi E Kosiorek, Guido Finazzi, Riccardo Cavazzina, Arianna Masciulli, Marco Scarano, Alessandro M Vannucchi, Ruben A Mesa, Tiziano Barbui
Current guidelines suggest that polycythemia vera (PV) patients maintain a strict hematocrit less than 45%. However, to date, little is known about the relationship between HCT control and PV- related symptom burden. In this study, PV patient data was analyzed from the CYTO PV trial (n = 224) and the MPN-SAF study cohort (n = 645). No significant differences in symptom burden were seen at the 6 and 12 month follow-up when evaluating prospective hematocrit control in the CYTO PV cohort. Patients in the MPN-SAF cohort with a worst item score of greater than 5/10 on the Myeloproliferative Neoplasm Symptom Total Symptom Score had a significantly lower mean hematocrit (p = ...
November 10, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27822101/the-danish-national-chronic-myeloid-neoplasia-registry
#12
REVIEW
Marie Bak, Else Helene Ibfelt, Thomas Stauffer Larsen, Dorthe Rønnov-Jessen, Niels Pallisgaard, Ann Madelung, Lene Udby, Hans Carl Hasselbalch, Ole Weis Bjerrum, Christen Lykkegaard Andersen
AIM: The Danish National Chronic Myeloid Neoplasia Registry (DCMR) is a population-based clinical quality database, introduced to evaluate diagnosis and treatment of patients with chronic myeloid malignancies. The aim is to monitor the clinical quality at the national, regional, and hospital departmental levels and serve as a platform for research. STUDY POPULATION: The DCMR has nationwide coverage and contains information on patients diagnosed at hematology departments from January 2010 onward, including patients with essential thrombocythemia, polycythemia vera, myelofibrosis, unclassifiable myeloproliferative neoplasms, chronic myelomonocytic leukemia, and chronic myeloid leukemia...
2016: Clinical Epidemiology
https://www.readbyqxmd.com/read/27812134/quantification-of-igf-1-receptor-may-be-useful-in-diagnosing-polycythemia-vera-suggestion-to-be-added-to-be-one-of-the-minor-criterion
#13
Jen C Wang, Guanfang Shi, Stacey Baptiste, Maryna Yarotska, Hemant Sindhu, Ching Wong, Madhumati Kalavar, Vladimir Gotlieb, Andrei Bandarchuk, Hui Chen
Endogenous erythroid colony (EEC) formation is one of the minor criteria for diagnosing polycythemia vera (PV) according to 2008 WHO diagnostic criteria. But EEC requires bone marrow aspiration and sophisticated laboratory procedures; therefore, practically it is rarely used to diagnose PV. Insulin-like growth factor 1 receptor (IGF-1R) was found to be constitutively phosphorylated and was responsible for the EEC formation in PV; therefore, we measured IGF-1R levels in the peripheral blood of 26 PV patients and compared them with those of 33 patients with secondary polycythemia and 29 normal controls...
2016: PloS One
https://www.readbyqxmd.com/read/27810993/evaluation-of-bone-marrow-morphology-is-essential-for-assessing-disease-status-in-recombinant-interferon-alpha-treated-polycythemia-vera-patients
#14
Elizabeth Margolskee, Spencer Krichevsky, Attilio Orazi, Richard T Silver
No abstract text is available yet for this article.
November 3, 2016: Haematologica
https://www.readbyqxmd.com/read/27801320/-analysis-of-prognostic-factors-in-chinese-patients-with-post-polycythemia-vera-myelofibrosis-and-post-essential-thrombocythemia-myelofibrosis
#15
M Chen, Z F Xu, J Q Xu, B Li, P H Zhang, T J Qin, Y Zhang, J Y Wang, H L Zhang, L W Fang, L J Pan, N B Hu, S Q Qu, Z J Xiao
Objective: To evaluate the performances of the prognostic scoring systems devised for primary myelofibrosis(PMF)and the new developed MYSEC- PM(Mysec Prognostic Model)and investigate the risk factors in Chinese patients with post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis(post- PV/ET MF). The most widely used prognostic scoring systems in PMF included the International Prognostic Scoring System(IPSS), dynamic International Prognostic Scoring System(DIPSS), refined DIPSS(DIPSS plus), modified IPSS for Chinese(IPSS-Chinese), and modified DIPSS for Chinese(DIPSS- Chinese)...
October 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27801313/-interpretation-of-the-chinese-expert-consensus-on-the-diagnosis-and-treatment-of-polycythemia-vera-2016
#16
Z X Shi, Z J Xiao
No abstract text is available yet for this article.
October 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27787922/bone-health-in-patients-with-hematopoietic-disorders-of-bone-marrow-origin-systematic-review-and-meta-analysis
#17
Kieran Steer, Mariya Stavnichuk, Martin Morris, Svetlana V Komarova
Blood cell production and bone homeostasis are physically interlinked systems that exhibit active cross-talk. We examined how bone health is affected in patients with hematopoietic disorders due to abnormal proliferation of bone marrow cells. The electronic databases Medline, Embase, PubMed, BIOSIS Previews, Web of Science, and Cochrane were searched for studies presenting numerical values for trabecular bone volume or bone mineral density in control and patients with hematopoietic disorders. We identified 5 studies for beta-thalassemia, 6 for sickle cell anemia, 2 for polycythemia vera and essential thrombocythemia, 3 for chronic myelogenous leukemia, 6 for myelofibrosis, 5 for multiple myeloma, and 4 studies each for systemic mastocytosis, lymphocytic leukemia, and hemochromatosis...
October 27, 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27784744/genetic-and-pharmacologic-targeting-of-alox5-attenuates-jak2v617f-induced-polycythemia-vera-in-mice
#18
Yaoyu Chen, Yi Shan, Min Lu, Ngoc DeSouza, Zhiru Guo, Ronald Hoffman, Ai-Bing Liang, Shaoguang Li
Myeloproliferative neoplasms such as polycythemia vera (PV) which are associated with the JAK mutation V617F remain uncurable, despite progress in the use of JAK2 inhibitors for treatment of some of these diseases. In this study, we employed mice that undergo JAK2V617F-induced PV as a tool to explore new candidate targets for therapy. Our investigations focused on the lipid metabolic enzyme arachidonate 5-lipoxygenase (Alox5), which we found to be strongly upregulated by JAK2V617F in hematopoietic cells in vitro and in vivo...
October 26, 2016: Cancer Research
https://www.readbyqxmd.com/read/27783966/reduced-left-ventricular-strain-is-related-to-blood-parameters-in-patients-with-polycythemia-vera
#19
Piotr Dobrowolski, Anna Klisiewicz, Aleksander Prejbisz, Anna Sikorska, Jakub Wójcicki, Jacek Lewandowski, Anna M Kaszuba, Marek Kabat, Maria Kordybach-Prokopiuk, Andrzej Więcek, Jerzy Windyga, Andrzej Januszewicz, Piotr Hoffman
No abstract text is available yet for this article.
October 18, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27774824/inhibitors-of-jak-family-kinases-an-update-on-the-patent-literature-2013-2015-part-1
#20
Jason G Kettle, Annika Åstrand, Matthew Catley, Neil P Grimster, Magnus Nilsson, Qibin Su, Richard Woessner
Introduction Janus kinases (JAKs) are a family of four enzymes; JAK1, JAK2, JAK3 and tyrosine kinase 2 (TYK2) that are critical in cytokine signalling and are strongly linked to both cancer and inflammatory diseases. There are currently two launched JAK inhibitors for the treatment of human conditions: tofacitinib for Rheumatoid arthritis (RA) and ruxolitinib for myeloproliferative neoplasms including intermediate or high risk myelofibrosis and polycythemia vera. Areas covered This review covers patents claiming activity against one or more JAK family members in the period 2013-2015 inclusive, and covers 95 patents from 42 applicants, split over two parts...
October 24, 2016: Expert Opinion on Therapeutic Patents
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