keyword
MENU ▼
Read by QxMD icon Read
search

Polycythemia vera disorder

keyword
https://www.readbyqxmd.com/read/27892526/comprehensive-gene-expression-meta-analysis-and-integrated-bioinformatic-approaches-reveal-shared-signatures-between-thrombosis-and-myeloproliferative-disorders
#1
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
#2
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/27787922/bone-health-in-patients-with-hematopoietic-disorders-of-bone-marrow-origin-systematic-review-and-meta-analysis
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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...
August 8, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27494969/-not-available
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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...
July 14, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27334391/anesthetic-management-of-a-patient-with-polycythemia-vera-for-neurosurgery
#16
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
https://www.readbyqxmd.com/read/27263782/identification-of-human-erythroid-lineage-committed-progenitors
#17
Yasuo Mori, Koichi Akashi, Irving L Weissman
Elucidating the developmental pathway leading to erythrocytes and being able to isolate their progenitors is crucial to understanding and treating disorders of red cell imbalance such as anemia, myelodysplastic syndrome, and polycythemia vera. Endoglin (CD105) is a key marker for purifying mouse erythroid lineage-committed progenitors (EPs) from bone marrow. Herein, we show that human EPs can also be isolated from adult bone marrow. We identified three subfractions that possessed different expression patterns of CD105 and CD71 within the previously defined human megakaryocyte/erythrocyte progenitor (hMEP; Lineage-CD34(+)CD38(+)IL-3Rα(-)CD45RA(-)) population...
May 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27196481/erdheim-chester-disease-with-multiorgan-involvement-following-polycythemia-vera-a-case-report
#18
Alessandra Iurlo, Lorenzo Dagna, Daniele Cattaneo, Nicola Orofino, Paola Bianchi, Giulio Cavalli, Claudio Doglioni, Umberto Gianelli, Agostino Cortelezzi
Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by the migration and infiltration of lipid-laden CD68, CD1a and S100 histiocytes to various target organs, which leads to the disruption of physiological tissue architecture and reactive fibrosis, and thus impairs organ function.We describe the first case of a patient with Erdheim-Chester disease with multiorgan involvement developed after 6 years from polycythemia vera diagnosis. During the follow-up, an abdominal ultrasound scan revealed the presence of dense, bilateral perinephric infiltration...
May 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27154187/minihepcidin-peptides-as-disease-modifiers-in-mice-affected-by-%C3%AE-thalassemia-and-polycythemia-vera
#19
Carla Casu, Paraskevi Rea Oikonomidou, Huiyong Chen, Vijay Nandi, Yelena Ginzburg, Princy Prasad, Robert E Fleming, Yatrik M Shah, Erika V Valore, Elizabeta Nemeth, Tomas Ganz, Brian MacDonald, Stefano Rivella
In β-thalassemia and polycythemia vera (PV), disordered erythropoiesis triggers severe pathophysiological manifestations. β-Thalassemia is characterized by ineffective erythropoiesis, reduced production of erythrocytes, anemia, and iron overload and PV by erythrocytosis and thrombosis. Minihepcidins are hepcidin agonists that have been previously shown to prevent iron overload in murine models of hemochromatosis and induce iron-restricted erythropoiesis at higher doses. Here, we show that in young Hbb(th3/+) mice, which serve as a model of untransfused β-thalassemia, minihepcidin ameliorates ineffective erythropoiesis, anemia, and iron overload...
July 14, 2016: Blood
https://www.readbyqxmd.com/read/27134742/recent-advances-in-understanding-myelofibrosis-and-essential-thrombocythemia
#20
REVIEW
William Vainchenker, Stefan N Constantinescu, Isabelle Plo
The classic BCR-ABL-negative myeloproliferative neoplasms (MPNs), a form of chronic malignant hemopathies, have been classified into polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). ET and PMF are two similar disorders in their pathogenesis, which is marked by a key role of the megakaryocyte (MK) lineage. Whereas ET is characterized by MK proliferation, PMF is also associated with aberrant MK differentiation (myelodysplasia), leading to the release of cytokines in the marrow environment, which causes the development of myelofibrosis...
2016: F1000Research
keyword
keyword
43113
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"