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Polycythemia vera disorder

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https://www.readbyqxmd.com/read/28028027/emerging-treatments-for-classical-myeloproliferative-neoplasms
#1
Alessandro M Vannucchi, Claire Harrison
There has been a major revolution in the management of patients with myeloproliferative neoplasms (MPN), in particular those with myelofibrosis and extensive splenomegaly and symptomatic burden, following the introduction of the JAK1 and JAK2 inhibitor ruxolitinib. The drug has been later approved also as second line therapy for polycythemia vera (PV). However, the therapeutic armamentarium for MPN is still largely inadequate to cope with the major unmet patients' needs, that include normalization of life span (MF and some PV patients), reduction of cardiovascular complications (mainly PV and essential thrombocythemia (ET)), prevention of hematological progression and improved quality of life (all MPN)...
December 27, 2016: Blood
https://www.readbyqxmd.com/read/28028026/diagnosis-risk-stratification-and-response-evaluation-in-classical-myeloproliferative-neoplasms
#2
Elisa Rumi, Mario Cazzola
Philadelphia-negative classical myeloproliferative neoplasms include polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The 2016 revision of the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues includes new criteria for the diagnosis of these disorders. Somatic mutations in the 3 driver genes, that is, JAK2, CALR, and MPL, represent major diagnostic criteria in combination with hematologic and morphological abnormalities. Polycythemia vera is characterized by erythrocytosis with suppressed endogenous erythropoietin production, bone marrow panmyelosis, and JAK2 mutation...
December 27, 2016: Blood
https://www.readbyqxmd.com/read/27956542/myeloproliferative-neoplasms-version-2-2017-nccn-clinical-practice-guidelines-in-oncology
#3
Ruben Mesa, Catriona Jamieson, Ravi Bhatia, Michael W Deininger, Aaron T Gerds, Ivana Gojo, Jason Gotlib, Krishna Gundabolu, Gabriela Hobbs, Rebecca B Klisovic, Patricia Kropf, Sanjay R Mohan, Stephen Oh, Eric Padron, Nikolai Podoltsev, Daniel A Pollyea, Raajit Rampal, Lindsay A M Rein, Bart Scott, David S Snyder, Brady L Stein, Srdan Verstovsek, Martha Wadleigh, Eunice S Wang, Mary Anne Bergman, Kristina M Gregory, Hema Sundar
Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are a group of heterogeneous disorders of the hematopoietic system collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). The diagnosis and the management of patients with MPNs have evolved since the identification of mutations that activate the JAK pathway (JAK2, CALR, and MPL mutations) and the development of targeted therapies has resulted in significant improvements in disease-related symptoms and quality of life...
December 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27956534/a-polycythemia-vera-jak2-mutation-masquerading-as-a-duodenal-cancer-mutation
#4
Justin Lee, Jennifer Axilbund, W Brian Dalton, Daniel Laheru, Stanley Watkins, David Chu, Karen Cravero, Berry Button, Kelly Kyker-Snowman, Ian Waters, Christopher D Gocke, Josh Lauring, Ben Ho Park
Next-generation sequencing (NGS) is increasingly being used in cancer care to identify both somatic tumor driver mutations that can be targeted for therapy, and heritable mutations in the germline associated with increased cancer risk. This report presents a case of a JAK2 V617F mutation falsely identified as a duodenal cancer mutation via NGS. The patient was found to have a history of polycythemia vera, a disorder with a high incidence of JAK2 somatic mutations. Buccal cell DNA showed heterozygosity for the mutation, suggesting that it was potentially germline...
December 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27892526/comprehensive-gene-expression-meta-analysis-and-integrated-bioinformatic-approaches-reveal-shared-signatures-between-thrombosis-and-myeloproliferative-disorders
#5
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
#6
REVIEW
Alessandro M Vannucchi
Polycythemia vera is a clonal disorder of hematopoietic stem/progenitor cells. It manifests as an expansion of red cell mass. It is the most common chronic myeloproliferative neoplasm. In virtually all cases, it is characterized by a V617F point mutation in JAK2 exon 14 or less common mutations in exon 12. The landmark discovery of the autonomously activated JAK/STAT signaling pathway paved the way for the clinical development of the first target drug, the JAK1 and JAK2 inhibitor ruxolitinib. This is now approved for patients with resistance or intolerance to hydroxyurea...
January 2017: Haematologica
https://www.readbyqxmd.com/read/27787922/bone-health-in-patients-with-hematopoietic-disorders-of-bone-marrow-origin-systematic-review-and-meta-analysis
#7
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/27725592/myeloproliferative-neoplasms-updates-on-molecular-pathophysiology-diagnosis-and-treatment-strategies
#8
Katsuto Takenaka
Myeloproliferative neoplasms (MPNs) are chronic hematopoietic stem cell disorders, including polycythemia vera, essential thrombocytosis, and primary myelofibrosis. The JAK2V617F mutation was identified in 2005, followed by the discovery of the JAK2 exon12, MPNW515 mutation, and CALR mutation. About 90% of patients with BCR/ABL negative MPNs have been shown to have one of these driver mutations. In addition, mutations in epigenetic regulators and RNA splicing genes were found to co-exist with driver mutations and to play critical roles in the disease progression of MPNs...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27710990/coexistence-of-chronic-lymphocytic-leukemia-and-polycythemia-vera-a-case-report-and-review-of-the-literature
#9
Serdal Korkmaz, Sinan Kulakoglu, Hasan Gorkem, Bilal Aygun, Ali Cetinkaya
: Polycythemia vera is a Philadelphia chromosome-negative myeloproliferative neoplasm. Chronic lymphocytic leukemia is a monoclonal expansion of a CD5+ CD19+ B lymphocytes. Chronic myeloproliferative neoplasms may coexist with indolent B-cell malignant lymphomas of various types. The association of chronic lymphocytic leukemia with polycythemia vera is a rare event with only a few cases of coexistence ever reported. We report a 56-year-old man in whom these two disorders were diagnosed concomitantly...
September 2016: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/27697978/practice-patterns-in-the-diagnosis-and-treatment-of-polycythemia-vera-in-the-post-jak2-v617f-discovery-era
#10
Elizabeth M Kander, Alison R Moliterno, Alfred Rademaker, Michael B Streiff, Jerry L Spivak, Brady L Stein
Polycythemia vera (PV) is an acquired clonal hematopoietic stem cell disorder characterized by an overproduction of red blood cells, white blood cells, and platelets; thrombotic and hemorrhagic complications; and an increased risk of transformation to myelofibrosis and acute leukemia. In 1967, the Polycythemia Vera Study Group proposed the optimal approach to diagnosis and treatment of PV, and in 2002, investigators from Johns Hopkins University School of Medicine surveyed the practice patterns of hematologists as they pertained to PV...
October 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27693837/massive-upper-gastrointestinal-bleeding-due-to-splenoportal-axis-thrombosis-in-a-patient-with-a-tested-jak2-mutation-a-case-report-and-review-literature
#11
Isabel Macías
Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10mmHg. Cirrhosis is the most common cause of portal hypertension and thrombosis of the splenoportal axis not associated with liver cirrhosis is the second cause of portal hypertension in the Western world. The primary myeloproliferative disorders are the main cause of portal venous thrombosis and somatic mutation of Janus Kinase 2 gene (JAK2 V617F) can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% primary myelofibrosis...
August 13, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27598581/clinical-characteristics-of-cerebrovascular-pathology-with-patients-suffering-from-ph-negative-myeloproliferative-disease
#12
Marine M Tanashyan, Polina I Kuznetsova, Alla A Shabalina, Anton A Raskurazhev, Olga V Lagoda, Irina N Subortseva, Anait L Melikyan
BACKGROUND: Disturbances of microcirculation play a significant role in the development and progression of both acute and chronic cerebrovascular diseases (CVD) and may be associated with different hemogram abnormalities. One of the reasons of the prothrombogenic state of the endothelium is the increase in the number of blood corpuscles leading to (non-Ph) myeloproliferative disorders (MPD) including essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PM)...
September 7, 2016: Cerebrovascular Diseases Extra
https://www.readbyqxmd.com/read/27500783/myeloproliferative-neoplasms-trends-in-incidence-prevalence-and-survival-in-norway
#13
Christina Roaldsnes, René Holst, Henrik Frederiksen, Waleed Ghanima
BACKGROUND: Polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF) are clonal disorders collectively named as myeloproliferative neoplasms (MPN). Published data on epidemiology of MPN after the discovery of the JAK2 mutation and the 2008 WHO classifications are scarce. We aimed to study the incidence rates, prevalence and survival of MPN in Norway during the period 1993-2012. METHOD: We identified 2453 persons diagnosed with MPN from the Cancer Registry of Norway between 1993 and 2012...
January 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/27494969/-not-available
#14
Matthieu Mosca, Gaëlle Vertenoeil, Katte Rao Toppaldoddi, Isabelle Plo, William Vainchenker
BIOLOGICAL ASPECTS OF JAK/STAT SIGNALING IN BCR-ABL-NEGATIVE MYELOPROLIFERATIVE NEOPLASMS: Myeloproliferative disorders more recently named Myeloproliferative neoplasms (MPN) display several clinical entities: chronic myeloid leukemia (CML), the classical MPN including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and atypical and unclassifiable NMP. The term MPN is mostly used for classical BCR-ABL-negative (myeloproliferative disorder) (ET, PV, PMF). These are clonal diseases resulting from the transformation of an hematopoietic stem cell and leading to an abnormal production of myeloid cells...
June 2016: Bulletin du Cancer
https://www.readbyqxmd.com/read/27486346/clinical-applications-of-therapeutic-phlebotomy
#15
Kyung Hee Kim, Ki Young Oh
Phlebotomy is the removal of blood from the body, and therapeutic phlebotomy is the preferred treatment for blood disorders in which the removal of red blood cells or serum iron is the most efficient method for managing the symptoms and complications. Therapeutic phlebotomy is currently indicated for the treatment of hemochromatosis, polycythemia vera, porphyria cutanea tarda, sickle cell disease, and nonalcoholic fatty liver disease with hyperferritinemia. This review discusses therapeutic phlebotomy and the related disorders and also offers guidelines for establishing a therapeutic phlebotomy program...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27473820/janus-kinase-jak-inhibitors-in-the-treatment-of-inflammatory-and-neoplastic-diseases
#16
REVIEW
Robert Roskoski
The Janus kinase (JAK) family of non-receptor protein-tyrosine kinases consists of JAK1, JAK2, JAK3, and TYK2 (tyrosine kinase-2). Each of these proteins contains a JAK homology pseudokinase (JH2) domain that regulates the adjacent protein kinase domain (JH1). JAK1/2 and TYK2 are ubiquitously expressed whereas JAK3 is found predominantly in hematopoietic cells. The Janus kinase family is regulated by numerous cytokines including interleukins, interferons, and hormones such as erythropoietin, thrombopoietin, and growth hormone...
September 2016: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/27468853/correlation-between-jak2-allele-burden-and-pulmonary-arterial-hypertension-and-hematological-parameters-in-philadelphia-negative-jak2-positive-myeloproliferative-neoplasms-an-egyptian-experience
#17
Mervat M Mattar, Mohammed Abdel Kader Morad, Noha M El Husseiny, Noha H Ali, Doaa M El Demerdash
Myeloproliferative neoplasms are characterized by a common stem cell-derived clonal proliferation, but are phenotypically diverse. JAK2 is mutated (V617F) in more than 90 % of patients with polycythemia vera (PV) and approximately 60 % of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). Pulmonary arterial hypertension (PAH) is a major complication of several hematological disorders. Chronic myeloproliferative disorders associated with PAH have been included in group five for which the etiology is unclear and/or multifactorial...
October 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27429517/is-jak2v617f-mutation-the-only-factor-for-thrombosis-in-philadelphia-negative-chronic-myeloproliferative-neoplasms
#18
Mehmet Sevki Uyanik, Mehmet Baysal, Gulsum Emel Pamuk, Muhammet Maden, Mustafa Akker, Elif Gulsum Umit, Muzaffer Demir, Erkan Aydogdu
The most common genetic disorder in Philadelphia negative chronic myeloproliferative neoplasms is the JAK2-V617F mutation. In the present study, we aimed to determine risk factors for thrombosis in patients with essential thrombocytosis and polycythemia vera. We screened the medical records of 101 patients. Risk factors which may predict thrombosis were recorded. Venous thrombosis (VT) before diagnosis was significantly higher in JAK2 positive patients. VT after diagnosis was similar in JAK2 positive and negative groups, and was significantly higher in elderly patients...
September 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27414763/do-we-need-antiplatelet-therapy-in-thrombocytosis-contra-proposal-for-an-individualized-risk-adapted-treatment
#19
Rüdiger E Scharf
Thrombocytosis is a frequent laboratory finding but not a diagnosis. Therefore, elevated platelet counts (>450 x 10(9)/l) require careful diagnostic work-up to differentiate between reactive thrombocytosis (RT), caused by various conditions, and essential thrombocythemia (ET), a myeloproliferative neoplasm (MPN). In either setting, aspirin is widely used in clinical practice. However, RT (even at platelet counts >1000 x 10(9)/l) has never been shown to cause thrombosis or bleeding due to acquired von Willebrand factor defects in association with high platelet counts...
November 7, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27334391/anesthetic-management-of-a-patient-with-polycythemia-vera-for-neurosurgery
#20
Nodu Shivananda Gautham, Appavoo Arulvelan, Sethuraman Manikandan
Polycythemia vera (PV) is a myeloproliferative disorder characterized by excess red cell clonality. The increased number of red blood cells can lead to increased viscosity of the blood and ultimately compromise the blood supply to the end organs. Thromboembolic and hemorrhagic complications can also develop. Patients with PV presenting with neurological diseases that require surgical intervention are at an increased risk due to various factors, such as immobility, prolonged surgical time, hypothermia and dehydration...
October 2016: Journal of Anesthesia
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