7,771 results match your criteria Polycythemia Vera


ruxolitinib (JAKAVI°) and polycythaemia vera Inconclusive evaluation.

Authors:

Prescrire Int 2016 Oct;25(175):229-231

Patients with polycythaemia vera, a myeloproliferative syndrome, are at increased risk of thrombotic events. Marrow fibrosis and transformation to acute leukaemia can also occur after several years. Treatment is based on phlebotomy and aspirin at low (antiplatelet) doses, sometimes combined with hydroxycarbamide, a cytotoxic drug. Read More

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October 2016

Tuberculosis and atypical mycobacterial infections in ruxolitinib treated patients with primary or secondary myelofibrosis or polycythemia vera.

Int J Infect Dis 2019 Jan 10. Epub 2019 Jan 10.

Department of Oncology and Hematology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Université de Strasbourg, Inserm UMR-S1113/IRFAC, Strasbourg, France.

Ruxolitinib is a JAK-1/JAK-2 inhibitor indicated in the treatment of polycythemia vera and primary or secondary myelofibrosis. Only one (0.2%) patient was diagnosed with tuberculosis among the 485 patients receiving ruxolitinib in the four pivotal trials. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S12019712193001
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http://dx.doi.org/10.1016/j.ijid.2019.01.002DOI Listing
January 2019
1 Read

Young versus old age at diagnosis confers distinct genomic profiles in patients with polycythemia vera.

Leukemia 2019 Jan 11. Epub 2019 Jan 11.

Division of Hematology, Washington University School of Medicine, St. Louis, MO, USA.

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http://dx.doi.org/10.1038/s41375-018-0349-9DOI Listing
January 2019

Recurrent ischemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms.

Eur J Neurol 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology & Stroke, and Hertie-Institute for Clinical Brain Research, Eberhard-Karls University of Tübingen, Tübingen, Germany.

Background: Myeloproliferative neoplasms (MPN) - polycythemia vera, essential thrombocythemia and primary myelofibrosis - are associated with increased risk for ischemic cerebrovascular events (ICVE). Due to their low prevalence, MPN often remain undiagnosed as the cause of ICVE.

Methods: Case records at the University of Tübingen between 2014 and 2017 were screened to identify patients with MPN-related ICVE. Read More

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http://doi.wiley.com/10.1111/ene.13907
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http://dx.doi.org/10.1111/ene.13907DOI Listing
January 2019
1 Read

WT1 gene is overexpressed in myeloproliferative neoplasms, especially in myelofibrosis.

Blood Cells Mol Dis 2018 Dec 20;75:35-40. Epub 2018 Dec 20.

Université Angers, UFR Santé, Angers, France; CHU Angers, Laboratoire d'Hématologie, Angers, France; INSERM, CRCINA, Université de Nantes, Université d'Angers, France; Fédération Hospitalo-Universitaire 'Grand Ouest Against Leukemia' (FHU GOAL), France. Electronic address:

Classical Philadelphia-negative myeloproliferative neoplasms include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF). They are characterized by the presence of driver mutations of JAK2, CALR or MPL genes. Overexpression of WT1 is used as a marker of minimal residual disease in acute myeloid leukemia, especially after allogeneic stem cell transplantation (SCT). Read More

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http://dx.doi.org/10.1016/j.bcmd.2018.12.004DOI Listing
December 2018
1 Read

A Case of Chronic Myelogenous Leukemia Occurring in a Patient Treated for Essential Thrombocythemia.

Am J Case Rep 2019 Jan 3;20:10-14. Epub 2019 Jan 3.

Department of Hematology and Medical Oncology, Emory University, Atlanta, GA, USA.

BACKGROUND Essential thrombocythemia (ET) is one of the BCR-ABL gene fusion negative chronic myeloproliferative disorders (MPDs), which also include polycythemia vera (PV), and myelofibrosis. Few clinical cases have reported the progression of ET to chronic myelogenous leukemia (CML) with the expression of the BCR-ABL gene. This report describes such a case and includes a review of other reported cases of CML co-occurring with BCR-ABL-negative chronic MPDs. Read More

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http://dx.doi.org/10.12659/AJCR.911854DOI Listing
January 2019
1 Read

A CBC algorithm combined with immature platelet fraction is able to identify JAK2 V617F mutation-positive polycythaemia vera patients.

Int J Lab Hematol 2019 Jan 2. Epub 2019 Jan 2.

Haematology Laboratory, Medlab Central, Palmerston North Hospital, Palmerston North, New Zealand.

Introduction: The recent lowering of the World Health Organisation (WHO) haemoglobin (Hb) and haematocrit (HCT) thresholds for diagnosis of polycythaemia vera (PV) has markedly increased the proportion of complete blood counts (CBC) that could be referred for haematologist review for comment, including advice on JAK2 V617F mutation analysis. The utility of an algorithm based on CBC indices and immature platelet fraction (IPF) to select cases for haematologist review was investigated.

Methods: IPF analysis was performed on 22 patients with known JAK2 V617F mutation and 41 patients who were negative for this mutation previously tested because of suspicion of PV. Read More

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http://dx.doi.org/10.1111/ijlh.12967DOI Listing
January 2019
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Mutation patterns in essential thrombocythemia, polycythemia vera and secondary myelofibrosis.

Leuk Lymphoma 2019 Jan 2:1-5. Epub 2019 Jan 2.

a Laboratoire d'Oncologie Prédictive , Institut Paoli-Calmettes, Centre de Recherche en Cancérologie de Marseille , Inserm U1068 CNRS UMR 7258 , Marseille , France.

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http://dx.doi.org/10.1080/10428194.2018.1522437DOI Listing
January 2019
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Clinicopathological and molecular features of SF3B1-mutated myeloproliferative neoplasms.

Hum Pathol 2018 Dec 27. Epub 2018 Dec 27.

Department of Pathology, Massachusetts General Hospital, 02114, Boston, MA, USA; Harvard Medical School, 02114, Boston, MA, USA. Electronic address:

The introduction of next-generation sequencing has broadened the genetic landscape of myeloproliferative neoplasms (MPNs) beyond JAK2, MPL and CALR. However, the biological role and clinical impact of most other mutations is not well defined. We interrogated 101 genes in 143 BCR-ABL1-negative MPNs in chronic phase from two large institutions. Read More

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http://dx.doi.org/10.1016/j.humpath.2018.11.022DOI Listing
December 2018
1 Read

A case report of cerebral infarction caused by polycythemia vera.

Medicine (Baltimore) 2018 Dec;97(52):e13880

Department of Radiology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, China.

Rationale: Polycythemia vera (PV) is a cloned erythrocytotic disease manifested by high proliferation and apoptosis in the bone marrow. The clinical symptoms of PV are occult. In practice, patients with cerebral infarction caused by PV are prone to misdiagnosis and missed diagnosis. Read More

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http://dx.doi.org/10.1097/MD.0000000000013880DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314744PMC
December 2018

Treatment of polycythemia vera.

Authors:
Petr Dulíček

Vnitr Lek 2018 ;64(10):955-960

Polycythemia vera is a chronic myeloproliferative neoplasm characterized by hematopoietic stem cell-derived clonal myeloproliferation resulting in erythrocytosis, leukocytosis and thrombocytosis. Survival is reduced compared with general population. Main reasons of death include thrombohemorrhagic complications, fibrotic progression and leuk-aemic transformation. Read More

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January 2018
2 Reads

EVALUATION OF BURDENSOME SYMPTOMS IN PATIENTS WITH RADIATION6ASSOCIATED AND SPONTANEOUS MYELOPROILIFERATIVE NEOPLASMS WITH THE USE OF OPTIMIZED SELF-ASSESSMENT MPN-SAF TSS.

Probl Radiac Med Radiobiol 2018 Dec;23:510-516

State Institution «National Research Center for Radiation Medicine of the National Academy of Medical Sciences of Ukraine», 53 Melnykova str., Kyiv, 04050, UkraineBogomolets National Medical University, 13, Tarasa Shevchenko Blvd, Kyiv, 01601, Ukraine.

Objective: To investigate the intensity of burdensome symptoms using self-assessment MPN-SAF TSS in patientswith radiation-associated and spontaneous myeloproiliferative neoplasms (MPNs).

Materials And Methods: The study included 89 patients with radiation-associated and spontaneous MPNs, the bur-densome symptoms of MPN were determined using MPN-SAF TSS.

Results: The average score for complaints in patients with radiation-associated MPNs was significantly higher thanin patients with spontaneous MPNs - 43. Read More

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http://dx.doi.org/10.33145/2304-8336-2018-23-510-516DOI Listing
December 2018
1 Read

β-catenin and PPAR-γ levels in bone marrow of myeloproliferative neoplasm: an immunohistochemical and ultrastructural study.

Ultrastruct Pathol 2018 Dec 24:1-10. Epub 2018 Dec 24.

a Department of Molecular Oncology , Institute for Medical Research, University of Belgrade , Belgrade , Serbia.

In accordance with increased proliferation in myeloproliferative neoplasm (MPN), the goal is to evaluate the immunoexpression of: β-catenin, PPAR-γ and Ki67 protein, to compare them with bone marrow ultrastructural characteristics in patients with MPN. Immunoexpression and electron microscopy of bone marrow was analyzed in 30 Ph-negative MPN patients, including per 10 patients with polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The quantity of β-catenin immunoreactive cells was significantly higher in PV then in ET (p < 0. Read More

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http://dx.doi.org/10.1080/01913123.2018.1558323DOI Listing
December 2018
1 Read

Genetic studies in the evaluation of myeloproliferative neoplasms.

Semin Hematol 2019 Jan 28;56(1):7-14. Epub 2018 May 28.

University of Pennsylvania, Department of Pathology and Laboratory Medicine, Division of Hematopathology, Philadelphia, PA.

Myeloproliferative neoplasms that include the specific entities of chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera, essential thrombocythemia, and primary myelofibrosis are characterized by the clonal expansion of hematopoietic precursor cells and consequent neoplastic production of mature cells of myeloid, erythroid, and/or megakaryocytic lineage. Genetic studies, encompassing both cytogenetic and molecular testing, play a central and ever increasing role in the assessment of these neoplasms and are the focus of this review. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.004DOI Listing
January 2019
3.274 Impact Factor

IL-6 stimulation of DNA replication is JAK1/2 mediated in cross-talk with hyperactivated ERK1/2 signaling.

Cell Biol Int 2018 Dec 20. Epub 2018 Dec 20.

Department of Molecular Oncology, Institute for Medical Research, University of Belgrade, Belgrade, Serbia.

Myeloproliferative neoplasms (MPNs) are developing resistance to therapy by JAK1/2 inhibitor ruxolitinib. To explore the mechanism of ruxolitinib's limited effect, we examined the JAK1/2 mediated induction of proliferation related ERK1/2 and AKT signaling by proinflammatory interleukin-6 (IL-6) in MPN granulocytes and JAK2V617F mutated human erythroleukemia (HEL) cells. We found that JAK1/2 or JAK2 inhibition prevented the IL-6 activation of STAT3 and AKT pathways in polycythemia vera and HEL cells. Read More

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http://dx.doi.org/10.1002/cbin.11084DOI Listing
December 2018
1 Read
1.933 Impact Factor

Oxidative and nitrosative stress in myeloproliferative neoplasms: the impact on the AKT / mTOR signaling pathway.

J BUON 2018 Sep-Oct;23(5):1481-1491

Deparment of Molecular Biology, Institute for Medical Research, University of Belgrade, Belgrade, Serbia.

Purpose: A common feature of malignancies is increased reactive oxygen species (ROS) and reactive nitrogen species (RNS). We analyzed the influence of oxidative and nitrosative stress on the activation of AKT/mTOR signaling pathway in myeloproliferative neoplasms (MPN).

Methods: Oxidative stress-induced gene expression in circulatory CD34+ cells of MPN patients was studied by microarray analysis. Read More

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December 2018
2 Reads

The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia.

Exp Hematol Oncol 2018 17;7:31. Epub 2018 Dec 17.

Department of Haematology, Sunway Medical Centre, Kuala Lumpur, Malaysia.

Background: The evolution of molecular studies in myeloproliferative neoplasms (MPN) has enlightened us the understanding of this complex disease consisting of polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The epidemiology is well described in the western world but not in Asian countries like Malaysia.

Materials And Methods: This retrospective national registry of MPN was conducted from year 2009 to 2015 in Malaysia. Read More

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https://ehoonline.biomedcentral.com/articles/10.1186/s40164-
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http://dx.doi.org/10.1186/s40164-018-0124-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296047PMC
December 2018
11 Reads

The Role of Advanced Practitioners in Optimizing Clinical Management and Support of Patients With Polycythemia Vera.

J Adv Pract Oncol 2018 Jan-Feb;9(1):56-66. Epub 2018 Jan 1.

The University of Arizona Cancer Center, Tucson, Arizona; and University of Colorado Anschutz Medical Campus, Aurora, Colorado.

Mr. M, a 65-year-old male, presented to his primary care physician with progressive fatigue, difficulty sleeping, and daily headaches for the past 3 weeks. His headaches were not associated with visual disturbances, cognitive deficits, or nausea/vomiting, and he had no history of migraines. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296415PMC
January 2018
1 Read

Advanced forms of MPNs are accompanied by chromosomal abnormalities that lead to dysregulation of TP53.

Blood Adv 2018 Dec;2(24):3581-3589

Tisch Cancer Institute, Division of Hematology/Oncology, and.

The Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and the prefibrotic form of primary myelofibrosis (PMF), frequently progress to more overt forms of MF and a type of acute leukemia termed MPN-accelerated phase/blast phase (MPN-AP/BP). Recent evidence indicates that dysregulation of the tumor suppressor tumor protein p53 (TP53) commonly occurs in the MPNs. The proteins MDM2 and MDM4 alter the cellular levels of TP53. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018024018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306879PMC
December 2018
5 Reads

Safety and efficacy of combined ruxolitinib and decitabine in accelerated and blast-phase myeloproliferative neoplasms.

Blood Adv 2018 Dec;2(24):3572-3580

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

Myeloproliferative neoplasms (MPN), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, have a propensity to evolve into accelerated and blast-phase disease (MPN-AP/BP), carrying a dismal prognosis. Conventional antileukemia therapy has limited efficacy in this setting. Thus, MPN-AP/BP is an urgent unmet clinical need. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018019661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306885PMC
December 2018
1 Read

[A case of gout secondary to primary myelofibrosis].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1117-1119

Department of Rheumatology and Immunology,Peking University First Hospital, Beijing 100034, China.

A 52-year-old man was referred to our department with a 2-year history of polyarthritis. He was diagnosed as gout due to acute arthritis of bilateral feet dorsum 2 years ago,but he didn't receive any standard treatment. 1 year ago,there were more and more joints evolved during the gout attack, and many subcutaneous nodules occurred. Read More

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December 2018
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The ruxolitinib effect: understanding how molecular pathogenesis and epigenetic dysregulation impact therapeutic efficacy in myeloproliferative neoplasms.

J Transl Med 2018 Dec 17;16(1):360. Epub 2018 Dec 17.

Centre for Medical Education, Queen's University Belfast, Belfast, UK.

The myeloproliferative neoplasms (MPN), polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are linked by a propensity to thrombosis formation and a risk of leukaemic transformation. Activation of cytokine independent signalling through the JAK/STAT cascade is a feature of these disorders. A point mutation in exon 14 of the JAK2 gene resulting in the formation of the JAK2 V617F transcript occurs in 95% of PV patients and around 50% of ET and PMF patients driving constitutive activation of the JAK/STAT pathway. Read More

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http://dx.doi.org/10.1186/s12967-018-1729-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296062PMC
December 2018
1 Read

Do All Patients With Polycythemia Vera or Essential Thrombocythemia Need Cytoreduction?

J Natl Compr Canc Netw 2018 Dec;16(12):1539-1545

Department of Medicine, and Division of Hematology and Oncology, Northwestern University Feinberg School of Medicine; and Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois.

Polycythemia vera (PV) and essential thrombocythemia (ET) are Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs), characterized by expansion of normal blood counts, bleeding, thrombosis, and the potential for transformation to myelofibrosis (MF) or acute myeloid leukemia (AML). The primary goals of treatment for MPNs are to reduce the risk of thrombosis, alleviate systemic symptom burden (eg, fatigue, pruritus, microvascular symptoms, and symptomatic splenomegaly), and to prevent transformation to MF/AML. Preventing transformation is clearly important, but not expected with current therapies. Read More

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http://dx.doi.org/10.6004/jnccn.2018.7073DOI Listing
December 2018

Novel therapeutic approaches in polycythemia vera.

Clin Adv Hematol Oncol 2018 Nov;16(11):750-757

Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Polycythemia vera (PV) is the most common Philadelphia chromosome-negative myeloproliferative neoplasm. Whereas low-risk patients are treated with aspirin and phlebotomy, high-risk patients receive cytoreductive therapy, which most commonly consists of hydroxyurea in the United States. Concerns about the long-term safety of hydroxyurea, as well as a desire for more efficacious and targeted therapy, have led to the development of novel therapies for high-risk patients with PV. Read More

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November 2018

Heterogeneity in myeloproliferative neoplasms: Causes and consequences.

Adv Biol Regul 2018 Nov 22. Epub 2018 Nov 22.

MRC Molecular Haematology Unit, MRC Weatherall Institute of Molecular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford, OX3 9DS, United Kingdom; NIHR Biomedical Research Centre, Churchill Hospital, Oxford, UK. Electronic address:

Myeloproliferative neoplasms (MPNs) are haematopoietic stem cell-derived clonal disorders characterised by proliferation of some or all myeloid lineages, depending on the subtype. MPNs are classically categorized into three disease subgroups; essential thrombocythaemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF). The majority (>85%) of patients carry a disease-initiating or driver mutation, the most prevalent occurring in the janus kinase 2 gene (JAK2 V617F), followed by calreticulin (CALR) and myeloproliferative leukaemia virus (MPL) genes. Read More

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http://dx.doi.org/10.1016/j.jbior.2018.11.007DOI Listing
November 2018
1 Read

Detection of Exon 12 and 14 Mutations in Janus Kinase 2 Gene Including a Novel Mutant in V617F Negative Polycythemia Vera Patients from Pakistan.

J Cancer 2018 21;9(23):4341-4345. Epub 2018 Oct 21.

Cancer and Medical Genetics, CAMS-A, King Saud Bin Abdulaziz University for Health Sciences & King Abdullah International Medical Research Centre (KAIMRC), King Abdulaziz Medical City, National Guard Health Affairs, Al Ahsa, Saudi Arabia.

The most frequently reported genetic aberration among polycythemia vera (PV) patients is a gain of function mutation V617F in exon 14 of Janus kinase 2 (JAK2) gene. However in many investigations, V617F negative PV patients have been reported to harbor mutations in JAK 2 exon 12. We investigated 24 patients with PV (diagnosed following 2016 WHO guidelines) to detect V617F mutation through allele specific PCR. Read More

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http://www.jcancer.org/v09p4341.htm
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http://dx.doi.org/10.7150/jca.27414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277640PMC
October 2018
7 Reads
2.639 Impact Factor

Indications and use of therapeutic phlebotomy in polycythemia vera: which role for erythrocytapheresis?

Leukemia 2019 Jan 5;33(1):279-281. Epub 2018 Dec 5.

Fondazione Policlinico Universitario A. Gemelli IRCCS and Istituto di Ematologia, Università Cattolica, Roma, Italy.

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http://dx.doi.org/10.1038/s41375-018-0304-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326952PMC
January 2019
1 Read

Differences in presenting features, outcome and prognostic models in patients with primary myelofibrosis and post-polycythemia vera and/or post-essential thrombocythemia myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study.

Semin Hematol 2018 Oct 5;55(4):248-255. Epub 2018 Jun 5.

Institute of Hematology "L. and A. Seràgnoli", Sant'Orsola-Malpighi University Hospital, Bologna, Italy.

Recently, the myelofibrosis secondary to PV and ET prognostic model (MYSEC-PM) was introduced to assess prognosis in myelofibrosis (MF) secondary to polycythemia vera and essential thrombocythemia (post-PV and post-ET MF), replacing the International Prognostic Scoring System (IPSS) and/or Dynamic IPSS (DIPSS) that was applied for primary MF (PMF). In a cohort of 421 ruxolitinib (RUX)-treated patients (post-PV and post-ET MF: 44.2%), we evaluated the following: (1) disease phenotype, responses, and toxicity to RUX; and (2) performance of the MYSEC-PM in post-PV or post-ET MF. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.013DOI Listing
October 2018
2 Reads

Somatic Mutations in Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.

Semin Hematol 2018 Oct 17;55(4):215-222. Epub 2018 Apr 17.

Serviço de Hematologia e Transplantação de Medula, Hospital de Santa Maria, Lisboa, Portugal.

Myeloproliferative neoplasms (MPN) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN are characterized by clonal proliferation of myeloid progenitors leading to erythrocytosis, thrombocytosis, or leukocytosis, and risk of hemorrhagic and thrombotic events, as well as myelofibrosis and blast transformation. The discovery of somatic mutations in MPN, namely JAK2 V617F, JAK2 exon 12, MPL, and CALR mutations, has permitted a more specific approach to diagnosis and treatment. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.04.005DOI Listing
October 2018
3 Reads

Different effect of hydroxyurea and phlebotomy on prevention of arterial and venous thrombosis in Polycythemia Vera.

Blood Cancer J 2018 Nov 26;8(12):124. Epub 2018 Nov 26.

Department of Experimental and Clinical Medicine, Center of Research and Innovation of Myeloproliferative neoplasms (CRIMM), AOU Careggi, University of Florence, Florence, Italy.

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http://dx.doi.org/10.1038/s41408-018-0161-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6255832PMC
November 2018
2 Reads

Polycythemia Rubra Vera Presenting as Unilateral Clubbing Due to Left Subclavian Artery Thrombosis.

J Assoc Physicians India 2018 May;66(5):90-91

Professor, Govt. Medical College, Calicut, Kerala.

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May 2018
12 Reads

Unique Case of Myeloproliferative Neoplasm with Two Rare Clonal Abnormalities: Rare JAK2 Exon 12 Mutation and Rare e14a3 (b3a3) BCR/ABL Fusion Transcript.

Acta Haematol 2019 21;141(1):23-27. Epub 2018 Nov 21.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas,

Myeloproliferative neoplasms (MPNs) are clonal disorders divided into Philadelphia (Ph) chromosome-positive chronic myeloid leukemia (CML) or Ph chromosome-negative MPNs. Co-occurrence of these disease entities is very rare and typically involves presence of common p190 or p210 BCR/ABL fusion transcript (responsible for CML) along with JAK2V617F mutation (most common driver mutation in Ph-negative MPNs). Because of the rarity of such cases, it is not clear if the outcomes are any different in these patients. Read More

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http://dx.doi.org/10.1159/000494427DOI Listing
November 2018
2 Reads

Analysis of clinical characteristics of bone marrow proliferative tumor progression to acute myeloid leukemia.

Cancer Biomark 2018 ;23(4):469-472

Objective: This study aims to analyze Chinese patients who developed acute leukemia after being diagnosed and treated for Philadelphia chromosome (Ph)-negative chronic myeloproliferative neoplasms (MPNs), and compare the findings of this series with similar studies from literature.

Methods: Nine patients who progressed to leukemia after being diagnosed with MPN were included into the present study. Clinical data including age, treatment modalities and duration of use in the myeloproliferative phase, latency to leukemic transformation (LT), characteristics of leukemia, chemotherapy administration, and survival after LT were examined. Read More

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http://dx.doi.org/10.3233/CBM-171145DOI Listing
January 2018
1 Read

Frequency and etiology of pulmonary hypertension in patients with myeloproliferative neoplasms.

Eur J Haematol 2018 Nov 19. Epub 2018 Nov 19.

Department of Haematology, Odense University Hospital, Odense, Denmark.

Objective: Pulmonary hypertension (PH) has been reported to be associated with myeloproliferative neoplasms (MPN), affecting 5%-48% of MPN patients. With the aims to describe the prevalence of PH in Ph-MPN patients and explore the cause in identified subjects, we performed a prospective cohort study of Ph-MPN patients.

Method: Transthoracic echocardiography (TTE) was performed on all patients. Read More

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http://doi.wiley.com/10.1111/ejh.13197
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http://dx.doi.org/10.1111/ejh.13197DOI Listing
November 2018
9 Reads

JAK-inhibitors in dermatology. Current evidence and future applications.

J Dermatolog Treat 2018 Nov 15:1-22. Epub 2018 Nov 15.

a Department of Dermatology , Medical University of Warsaw , Poland , 00-008 Warsaw, Koszykowa 82a.

The Janus kinase (JAK) and signal transducer and activator of transcription (STAT) pathway is a ubiquitous intracellular signaling network. Selective JAK-inhibitors have anti-inflammatory properties and have been approved in many countries for the treatment of rheumatoid arthritis (tofacitinib, baricitinib) and myelofibrosis or polycythemia vera (ruxolitinib). The aim of the publication was to summarize and critically analyze the efficacy and safety of JAK-inhibitors in skin diseases, such as psoriasis, alopecia areata, atopic dermatitis and vitiligo. Read More

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http://dx.doi.org/10.1080/09546634.2018.1546043DOI Listing
November 2018
3 Reads

Expression of CD markers in JAK2 positive myeloproliferative neoplasms: Prognostic significance.

Oncol Rev 2018 Jul 2;12(2):373. Epub 2018 Oct 2.

Thalassemia and Hemoglobinopathy Research Center, Research Institute of Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterized by the presence of JAK2 mutation. Thrombohemorrhagic as well as autoimmune or inflammatory phenomena are common clinical outcomes of these disorders. Recent studies have shown that abnormality in frequency and function of blood cells manifested by an alteration in CD markers' expression patterns play a key role in these complications. Read More

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https://www.oncologyreviews.org/index.php/or/article/view/37
Publisher Site
http://dx.doi.org/10.4081/oncol.2018.373DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199554PMC
July 2018
10 Reads

Safety of Therapeutic Plasma Exchange for the Treatment of Guillain-Barré Syndrome in Polycythemia Vera.

Neurologist 2018 Nov;23(6):185-187

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York.

Polycythemia vera (PV) is a risk factor for systemic thromboses and ischemic stroke. This has been attributed to blood hyperviscosity, the result of increased blood cell production. Intravenous immunoglobulin, which is indicated for the treatment of numerous hematologic and neurological conditions also causes increased serum viscosity and has been associated with ischemic strokes in the setting of PV. Read More

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http://dx.doi.org/10.1097/NRL.0000000000000197DOI Listing
November 2018
3 Reads

Macular perfusion velocities in the ocular ischaemic syndrome.

Acta Ophthalmol 2018 Oct 27. Epub 2018 Oct 27.

Department of Ophthalmology, Rigshospitalet, Glostrup, Denmark.

Purpose: To assess retinal perfusion in eyes with unilateral ocular ischaemic syndrome (OIS) and to compare with control subjects.

Methods: Retrospective case series. Linear blood flow velocities in macular vessels were estimated using motion-contrast fundus photography in eight patients with unilateral OIS (eight OIS eyes, seven fellow eyes) and 12 control subjects. Read More

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http://doi.wiley.com/10.1111/aos.13950
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http://dx.doi.org/10.1111/aos.13950DOI Listing
October 2018
12 Reads

Jak2V617F and Dnmt3a loss cooperate to induce myelofibrosis through activated enhancer-driven inflammation.

Blood 2018 Dec 26;132(26):2707-2721. Epub 2018 Oct 26.

QIMR Berghofer Medical Research Institute, Brisbane, QLD, Australia.

Myeloproliferative neoplasms (MPNs) are a group of blood cancers that arise following the sequential acquisition of genetic lesions in hematopoietic stem and progenitor cells (HSPCs). We identify mutational cooperation between Jak2V617F expression and Dnmt3a loss that drives progression from early-stage polycythemia vera to advanced myelofibrosis. Using in vivo, clustered regularly interspaced short palindromic repeats (CRISPR) with CRISPR-associated protein 9 (Cas9) disruption of Dnmt3a in Jak2V617F knockin HSPC, we show that Dnmt3a loss blocks the accumulation of erythroid elements and causes fibrotic infiltration within the bone marrow and spleen. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-04
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http://dx.doi.org/10.1182/blood-2018-04-846220DOI Listing
December 2018
15 Reads

The impact of phlebotomy and hydroxyurea on survival and risk of thrombosis among older patients with polycythemia vera.

Blood Adv 2018 Oct;2(20):2681-2690

Department of Chronic Disease Epidemiology, School of Public Health.

Current guidelines recommend therapeutic phlebotomy for all polycythemia vera (PV) patients and additional cytoreductive therapy (eg, hydroxyurea [HU]) for high-risk PV patients. Little is known about the impact of these therapies in the real-world setting. We conducted a retrospective cohort study of older adults diagnosed with PV from 2007 to 2013 using the linked Surveillance, Epidemiology, and End Results-Medicare database. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018021436DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199652PMC
October 2018
3 Reads

Takotsubo Cardiomyopathy Associated with Polycythemia Vera.

Case Rep Cardiol 2018 20;2018:4542925. Epub 2018 Sep 20.

Maharajgunj Medical Campus, Tribhuvan University, Kathmandu, Nepal.

Takotsubo cardiomyopathy is characterized by transient apical ballooning with preserved basal ventricular function triggered by physical or emotional stressors. We present a case of a 75-year-old man referred to our facility for the management of acute myocardial infarction later diagnosed as takotsubo cardiomyopathy. We believe platelet-mediated adrenaline release from massive thrombocytosis might have been the precipitating factor for the pathogenesis of takotsubo cardiomyopathy. Read More

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https://www.hindawi.com/journals/cric/2018/4542925/
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http://dx.doi.org/10.1155/2018/4542925DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171202PMC
September 2018
8 Reads

Management of Polycythemia Vera: A Survey of Canadian Physician Practice Patterns.

Clin Lymphoma Myeloma Leuk 2018 Aug 31. Epub 2018 Aug 31.

Division of Hematology/Oncology, St. Michael's Hospital, University of Toronto, Toronto, Canada.

Background: The 2016 World Health Organization (WHO) revised classification criteria for the diagnosis of polycythemia vera (PV) allows for an earlier detection of masked PV. The literature is scarce about the clinical uptake of new diagnostic algorithms for PV. In a cohort of Canadian hematologists, we aimed to identify how the revised 2016 WHO diagnostic criteria of PV are being incorporated into hematology practice, and if the treatment of PV is comparable to the approaches outlined by the Canadian Myeloproliferative Neoplasm Group. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S21522650183023
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http://dx.doi.org/10.1016/j.clml.2018.07.297DOI Listing
August 2018
8 Reads

Smoking is associated with increased risk of myeloproliferative neoplasms: A general population-based cohort study.

Cancer Med 2018 Nov 14;7(11):5796-5802. Epub 2018 Oct 14.

National Institute of Public Health, University of Southern Denmark, Copenhagen, Denmark.

Background: Former studies on smoking as a risk factor for Philadelphia-negative myeloproliferative neoplasms (MPNs) have mainly been carried out in women's cohorts and studies with various definitions of MPNs. Herein, we conducted a cohort study with register-based follow-up of a general population from Denmark, to validate and substantiate prior observations.

Methods: In the Danish Health Examination Survey cohort, we used the Cox proportional-hazards model adjusted for age, sex, body mass index, and level of education, to calculate hazard ratios (HRs), to investigate, whether daily smokers or occasional/ex-smokers had an increased risk of MPNs compared to never-smokers. Read More

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http://doi.wiley.com/10.1002/cam4.1815
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http://dx.doi.org/10.1002/cam4.1815DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246929PMC
November 2018
9 Reads

Concurrent Polycythemia of Undetermined Etiology and Smouldering Plasma Cell Myeloma.

Case Rep Pathol 2018 16;2018:8781721. Epub 2018 Sep 16.

Department of Pathology and Laboratory Medicine, University of Rochester, Rochester, NY, USA.

The combination of polycythemia and plasma cell myeloma occurring concurrently is very rare and few cases have been reported in the literature. Further, the vast majority of these cases are cases of polycythemia vera and myeloma. Here, we present a case of polycythemia of undetermined etiology and myeloma. Read More

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https://www.hindawi.com/journals/cripa/2018/8781721/
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http://dx.doi.org/10.1155/2018/8781721DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6165604PMC
September 2018
9 Reads

Cladribine treatment for Erdheim-Chester disease involving the central nervous system and concomitant polycythemia vera: A case report.

J Clin Exp Hematop 2018 Dec 10;58(4):161-165. Epub 2018 Oct 10.

Erdheim-Chester disease (ECD), a rare form of non-Langerhans cell histiocytosis, is characterized by the infiltration of foamy CD68 and CD1a histiocytes into multiple organ systems. Central nervous system (CNS) involvement has recently been reported to be a poor prognostic factor when treating ECD with interferon alpha. We report the case of a 66-year-old Japanese patient with ECD involving the CNS who harbored the BRAF V600E mutation and also concomitantly developed polycythemia vera with the JAK2 V617F mutation. Read More

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https://www.jstage.jst.go.jp/article/jslrt/advpub/0/advpub_1
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http://dx.doi.org/10.3960/jslrt.18015DOI Listing
December 2018
6 Reads

Classification and Personalized Prognosis in Myeloproliferative Neoplasms.

N Engl J Med 2018 10;379(15):1416-1430

From the Wellcome-MRC Cambridge Stem Cell Institute and Cambridge Institute for Medical Research (J.G., C.E.M., F.L.N., A.R.G., P.J.C.), the Department of Haematology, University of Cambridge (J.G., E.J.B., C.M., J.C., C.E.M., F.L.N., A.R.G.), and the Department of Haematology, Cambridge University Hospitals NHS Foundation Trust (J.G., E.J.B., A.L.G., C.M., J.C., A.R.G.), Cambridge, Wellcome Trust Sanger Institute, Wellcome Trust Genome Campus (J.N., D.C.W., N.A., E.P., G.G., L.O., S.O., J.W.T., A.P.B., N.W., P.J.C.), and the European Molecular Biology Laboratory, European Bioinformatics Institute (R.C., M.G.), Hinxton, Big Data Institute, University of Oxford, Oxford (D.C.W.), the Department of Haematology, Queen's University Belfast, Belfast (M.F.M.), and the Department of Haematology, Guy's and St. Thomas' NHS Foundation Trust, London (C.N.H.) - all in the United Kingdom; the Center for Molecular Oncology and the Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York (E.P., G.G.); the Department of Hematology, Zealand University Hospital, Roskilde, and the University of Copenhagen, Copenhagen (C.L.A., H.C.H.); and the Department of Experimental and Clinical Medicine, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy (P.G., A.M.V.).

Background: Myeloproliferative neoplasms, such as polycythemia vera, essential thrombocythemia, and myelofibrosis, are chronic hematologic cancers with varied progression rates. The genomic characterization of patients with myeloproliferative neoplasms offers the potential for personalized diagnosis, risk stratification, and treatment.

Methods: We sequenced coding exons from 69 myeloid cancer genes in patients with myeloproliferative neoplasms, comprehensively annotating driver mutations and copy-number changes. Read More

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http://www.nejm.org/doi/10.1056/NEJMoa1716614
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http://dx.doi.org/10.1056/NEJMoa1716614DOI Listing
October 2018
14 Reads

Influence of Blood Count, Cardiovascular Risks, Inherited Thrombophilia, and JAK2 V617F Burden Allele on Type of Thrombosis in Patients With Philadelphia Chromosome Negative Myeloproliferative Neoplasms.

Clin Lymphoma Myeloma Leuk 2018 Sep 10. Epub 2018 Sep 10.

School of Medicine, University of Zagreb, Zagreb, Croatia; Center for Medical Experts, Zagreb, Croatia.

Introduction: Thrombosis is the most common complication in Philadelphia chromosome negative (Ph-) myeloproliferative neoplasms patients.

Patients And Methods: In a cohort of 258 Ph- myeloproliferative neoplasm patients, the difference between patients with and without thrombosis was analyzed according to genetic thrombophilia factors, JAK2 V617F status and burden allele, blood count, cardiovascular risk factors and age. Patients were also divided in polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) subgroups as well as by the type of thrombosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S21522650183104
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http://dx.doi.org/10.1016/j.clml.2018.08.020DOI Listing
September 2018
13 Reads

[Variation of STAT3 Gene in Myleproliferative Neoplasms and Its Significance].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Oct;26(5):1430-1436

Department of Pediatrics, Affiliated Hospital of Zunyi Medical college, Zunyi 563003, Guizhou Province, China.E-mail:

Objective: To detect the mutation and single nucleotide polymorphisms of STAT3 gene in the patients with myeloproliferative neoplasms (MPN), and to analyze the correlation between STAT3 gene and the subtypes of MPN.

Methods: A total of 147 patients with MPN were selected, including 28 patients with polycythaemia vera (PV), 46 patients with essential thrombocythemia (ET), 10 patients with primary myelofibrosis (PMF), and 63 patients with chronic myeloid leukemia (CML); and 88 healthy persons were used as normal control. DNA of all cases was extracted from bone marrow or peripheral blood, and JAK2V617F gene mutation was detected by allele-specific PCR, then 23 exons of STAT3 gene were amplified by PCR. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.05.029DOI Listing
October 2018
9 Reads