8,157 results match your criteria Polycythemia Vera


Molecular profiling and risk classification of patients with myeloproliferative neoplasms and splanchnic vein thromboses.

Blood Adv 2020 Aug;4(15):3708-3715

Centre d'Investigations Cliniques (CIC) 1427, INSERM, Hôpital Saint-Louis, Université de Paris, Paris, France.

Myeloproliferative neoplasms (MPNs) are the most frequent underlying causes of splanchnic vein thromboses (SVTs). MPN patients with SVTs (MPN-SVT) often have a unique presentation including younger age, female predominance, and low Janus kinase 2 (JAK2) mutation allele burden. This study aimed at identifying risk factors for adverse hematologic outcomes in MPN-SVT patients. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020002414DOI Listing

Cardiovascular Risk in Polycythemia Vera: Thrombotic Risk and Survival: Can Cytoreductive Therapy Be Useful in Patients with Low-Risk Polycythemia Vera with Cardiovascular Risk Factors?

Oncol Res Treat 2020 Aug 7:1-4. Epub 2020 Aug 7.

Hematology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy.

Background/aims: Cardiovascular risk factors are not considered in the current scores for evaluation of the thrombotic risk in myeloproliferative neoplasms, and in polycythemia vera (PV) in particular. Cytoreduction is currently not indicated in low-risk patients with PV, despite the absence or presence of cardiovascular risk factors. Our purpose is to highlight how cardiovascular risk factors in patients with PV increase the thrombotic risk both in low- and high-risk patients. Read More

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http://dx.doi.org/10.1159/000509376DOI Listing

EQ-5D-Derived Health State Utility Values in Hematologic Malignancies: A Catalog of 796 Utilities Based on a Systematic Review.

Value Health 2020 Jul 13;23(7):953-968. Epub 2020 Jul 13.

Department of Experimental and Clinical Pharmacology, Medical University of Warsaw, Poland; HealthQuest Spółka z ograniczoną odpowiedzialnością Sp. k., Warsaw, Poland.

Objectives: We performed a systematic review of health state utility values (HSUVs) obtained using the EQ-5D questionnaire for patients with hematologic malignancies.

Methods: The following databases were searched up to September 2018: MEDLINE, EMBASE, The Cochrane Library, and the EQ-5D publications database on the EuroQol website. Additional references were extracted from reviewed articles. Read More

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http://dx.doi.org/10.1016/j.jval.2020.04.1825DOI Listing

[Acquired von Willebrand syndrome].

Rinsho Ketsueki 2020 ;61(7):809-817

Department of Transfusion Medicine, Nara Medical University.

Von Willebrand disease (VWD) is among the most common inherited bleeding disorders. Interestingly, acquired von Willebrand syndrome (AVWS) is diagnosed much less frequently, but can be identified in association with a substantial number of medical conditions and diseases, including lymphoproliferative (48%), cardiovascular (21%), myeloproliferative (15%), other neoplastic (5%), and autoimmune disorders (2%). Most recently, AVWS has been diagnosed in patients with aortic valve stenosis (AS, 79%) and continuous-flow left ventricular assist devices (LVAD, up to 100%). Read More

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http://dx.doi.org/10.11406/rinketsu.61.809DOI Listing
January 2020

Clonal Hematopoiesis and Mutations of Myeloproliferative Neoplasms.

Authors:
Lasse Kjær

Cancers (Basel) 2020 Jul 28;12(8). Epub 2020 Jul 28.

Department of Hematology, Zealand University Hospital, Vestermarksvej 7-9, DK-4000 Roskilde, Denmark.

Myeloproliferative neoplasms (MPNs) are associated with the fewest number of mutations among known cancers. The mutations propelling these malignancies are phenotypic drivers providing an important implement for diagnosis, treatment response monitoring, and gaining insight into the disease biology. The phenotypic drivers of Philadelphia chromosome negative MPN include mutations in , , and . Read More

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http://dx.doi.org/10.3390/cancers12082100DOI Listing

Cellular and Molecular Aspects of Blood Cell-Endothelium Interactions in Vascular Disorders.

Int J Mol Sci 2020 Jul 27;21(15). Epub 2020 Jul 27.

Faculté de Médecine, Université Denis Diderot Paris, 75013 Paris, France.

In physiology and pathophysiology the molecules involved in blood cell-blood cell and blood cell-endothelium interactions have been identified. Platelet aggregation and adhesion to the walls belonging to vessels involve glycoproteins (GP), GP llb and GP llla and the GP Ib-IX-V complex. Red blood cells (RBCs) in normal situations have little interaction with the endothelium. Read More

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http://dx.doi.org/10.3390/ijms21155315DOI Listing

Use of Interferon Alfa in the Treatment of Myeloproliferative Neoplasms: Perspectives and Review of the Literature.

Cancers (Basel) 2020 Jul 18;12(7). Epub 2020 Jul 18.

Department of Medical Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.

Interferon alfa was first used in the treatment of myeloproliferative neoplasms (MPNs) over 30 years ago. However, its initial use was hampered by its side effect profile and lack of official regulatory approval for MPN treatment. Recently, there has been renewed interest in the use of interferon in MPNs, given its potential disease-modifying effects, with associated molecular and histopathological responses. Read More

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http://dx.doi.org/10.3390/cancers12071954DOI Listing

The Role of Inflammation and Inflammasome in Myeloproliferative Disease.

J Clin Med 2020 Jul 22;9(8). Epub 2020 Jul 22.

Department of Scienze Mediche Chirurgiche e Tecnologie Avanzate "G.F. Ingrassia", University of Catania, 95123 Catania, Italy.

Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are rare hematological conditions known as myeloproliferative neoplasms (MPNs). They are characterized for being negative malignancies and affected patients often present with symptoms which can significantly impact their quality of life. MPNs are characterized by a clonal proliferation of an abnormal hematopoietic stem/progenitor cell. Read More

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http://dx.doi.org/10.3390/jcm9082334DOI Listing

Artificial intelligence-based morphological fingerprinting of megakaryocytes: a new tool for assessing disease in MPN patients.

Blood Adv 2020 Jul;4(14):3284-3294

Department of Cellular Pathology, John Radcliffe Hospital, Oxford University NHS Foundation Trust, Oxford, United Kingdom; and.

Accurate diagnosis and classification of myeloproliferative neoplasms (MPNs) requires integration of clinical, morphological, and genetic findings. Despite major advances in our understanding of the molecular and genetic basis of MPNs, the morphological assessment of bone marrow trephines (BMT) is critical in differentiating MPN subtypes and their reactive mimics. However, morphological assessment is heavily constrained by a reliance on subjective, qualitative, and poorly reproducible criteria. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020002230DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391156PMC

Prevalence and risk factors of high echocardiographic probability of pulmonary hypertension in myeloproliferative neoplasms patients.

Int J Hematol 2020 Jul 23. Epub 2020 Jul 23.

Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 110 Inthawaroros Street, Muang, Chiang Mai, 50200, Thailand.

Pulmonary hypertension (PH) is emerging as a complication of myeloproliferative neoplasms (MPNs). This was a prospective study conducted at Chiang Mai University Hospital. The primary objective was to determine the prevalence of high echocardiographic probability of PH in MPNs patients. Read More

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http://dx.doi.org/10.1007/s12185-020-02952-4DOI Listing

Givinostat: an emerging treatment for polycythemia vera.

Expert Opin Investig Drugs 2020 Jun 21;29(6):525-536. Epub 2020 Jul 21.

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

Introduction: Polycythemia vera (PV), a Philadelphia chromosome-negative myeloproliferative neoplasm, is characterized by panmyelosis, pancytosis, and a mutation. Patients are at increased risk of thrombohemorrhagic events, and progression to myelofibrosis or acute leukemia. Current treatments include aspirin, phlebotomy, and cytoreductive drugs (most commonly hydroxyurea). Read More

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http://dx.doi.org/10.1080/13543784.2020.1761323DOI Listing

JAK2 V617F as a Marker for Long-Term Disease Progression and Mortality in Polycythemia Vera and its Role in Economic Modeling.

J Health Econ Outcomes Res 2020 4;7(1):61-70. Epub 2020 Jun 4.

Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Background: In order to facilitate sound economic evaluations of novel treatments, health-economic models of polycythemia vera (PV) must combine effects on surrogate endpoints in trials with disease progression (DP) and mortality in long-term cohort data.

Objective: We validate an economic model for PV that uses Janus Kinase 2 (JAK2) burden as a surrogate endpoint to predict DP (thrombosis, myelofibrosis, and acute leukemia) and overall survival (OS) based on progression-specific mortality.

Methods: Long-term observational studies that include information about baseline JAK2 burden were identified via PubMed searches and used to validate the model. Read More

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http://dx.doi.org/10.36469/jheor.2020.13083DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7343343PMC

Atypical Myocardial Infarction with Apical Thrombus and Systemic Embolism: A Rare Presentation of Likely JAK2 V617F-Positive Myeloproliferative Neoplasm.

Case Rep Oncol Med 2020 19;2020:9654048. Epub 2020 May 19.

Richmond University Medical Center, USA.

A few types of myeloproliferative neoplasms may be significant for Janus-associated kinase 2 mutation, JAK2 V617F, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The prevalence of JAK2 mutation is low in the general population but higher in patients with myeloproliferative neoplasms. Some patients with JAK2 V617F-positive essential thrombocythemia are asymptomatic, but others may develop hemorrhagic or thromboembolic complications. Read More

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http://dx.doi.org/10.1155/2020/9654048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7338968PMC

Correlation Between PAI-1 Gene 4G/5G Polymorphism and the Risk of Thrombosis in Ph Chromosome-Negative Myeloproliferative Neoplasms.

Clin Appl Thromb Hemost 2020 Jan-Dec;26:1076029620935207

Department of Hematology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian Province, China.

Thrombosis has been recognized as one of the most significant risk factors of high mortality and disability in patients with Philadelphia (Ph) chromosome negative myeloproliferative neoplasms (MPNs). However, the risk factors of thrombotic events in these patients have not been completely understood. In this study, the clinical data of 58 patients with Ph-MPNs were obtained and analyzed, including 34 cases of essential thrombocytopenia (ET), 23 thrombotic events happened in 21 (36%) patients, among which 60% (14 of 23) with cerebral infarction, 17% (4 of 23) with coronary heart disease and 23% (5 of 23) with venous thrombosis. Read More

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http://dx.doi.org/10.1177/1076029620935207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7372617PMC

From leeches to interferon: should cytoreduction be prescribed for all patients with polycythemia vera?

Leukemia 2020 Jul 16. Epub 2020 Jul 16.

Foundation of Clinical Research-FROM, Ospedale Papa Giovanni XXIII, Bergamo, Italy.

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http://dx.doi.org/10.1038/s41375-020-0984-9DOI Listing

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype.

Cancers (Basel) 2020 Jul 14;12(7). Epub 2020 Jul 14.

Department of Internal Medicine V with Hematology, Oncology and Palliative Care, Hospital Hietzing, 1130 Vienna, Austria.

Patients with a myeloproliferative neoplasm (MPN) sometimes show a chronic myelomonocytic leukemia (CMML)-like phenotype but, according to the 2016 WHO classification, a documented history of an MPN excludes the diagnosis of CMML. Forty-one patients with an MPN (35 polycythemia vera (PV), 5 primary myelofibrosis, 1 essential thrombocythemia) and a CMML-like phenotype (MPN/CMML) were comprehensively characterized regarding clinical, hematologic, biologic and molecular features. The white blood cell counts in MPN/CMML patients were not different from CMML patients and PV patients. Read More

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http://dx.doi.org/10.3390/cancers12071891DOI Listing

PRMT5 inhibition modulates E2F1 methylation and gene regulatory networks leading to therapeutic efficacy in JAK2V617F mutant MPN.

Cancer Discov 2020 Jul 15. Epub 2020 Jul 15.

Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center

We investigated the role of PRMT5 in MPN pathogenesis and aimed to elucidate key PRMT5 targets contributing to MPN maintenance. PRMT5 is overexpressed in primary MPN cells and PRMT5 inhibition potently reduced MPN cell proliferation ex vivo. PRMT5 inhibition was efficacious at reversing elevated hematocrit, leukocytosis and splenomegaly in a model of JAK2V617F+ polycythemia vera (PV) and leukocyte and platelet counts, hepatosplenomegaly and fibrosis in the MPLW515L model of myelofibrosis (MF). Read More

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http://dx.doi.org/10.1158/2159-8290.CD-20-0026DOI Listing

Acute oxygenator occlusion in two cases of polycythemia vera: Bailout strategies.

J Card Surg 2020 Jul 15. Epub 2020 Jul 15.

Department of Cardiac Surgery, Medical University of Vienna, Vienna, Austria.

Polycytemia vera (PV) is a rare myeloproliferative neoplasm associated with microcirculatory disturbances, thrombosis and bleeding. Patients suffering from PV have a high risk of perioperative adverse events, but the literature regarding on-pump procedures in PV patients is scarce. We report two cases of acute and severe oxygenator failure during cardiopulmonary bypass and present valid exit scenarios. Read More

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http://dx.doi.org/10.1111/jocs.14876DOI Listing

Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms.

J Clin Med 2020 Jul 8;9(7). Epub 2020 Jul 8.

Department of Clinical and Experimental Medicine, UO Haematology, Azienda Ospedaliero-Universitaria Pisana, 56127 Pisa, Italy.

To define the role of spleen stiffness (SS) and liver stiffness (LS) in myelofibrosis and other Philadelphia (Ph)-negative myeloproliferative neoplasms (MPNs), we studied, by ultrasonography (US) and elastography (ES), 70 consecutive patients with myelofibrosis (MF) (no.43), essential thrombocythemia (ET) (no.10), and polycythemia vera (PV) (no. Read More

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http://dx.doi.org/10.3390/jcm9072149DOI Listing

Impact of World Health Organization (WHO) Revised Criteria-2016 on the Diagnosis of Polycythemia Vera.

Indian J Hematol Blood Transfus 2020 Jul 9;36(3):477-483. Epub 2019 Oct 9.

Department of Pathology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Udupi, Karnataka 576104 India.

The diagnosis of polycythemia vera (PV) requires the integration of clinical and laboratory findings, bone marrow morphologic features, and JAK2 analysis. JAK2V617F (exon 14) mutation is found in 95% of PV cases. In PV, addition of characteristic bone marrow morphology as one of three major diagnostic criteria allowed reduced hemoglobin/hematocrit threshold for diagnosis to 16. Read More

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http://dx.doi.org/10.1007/s12288-019-01202-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7326849PMC
July 2020
0.234 Impact Factor

Necessity to screen and treat latent tuberculosis before ruxolitinib treatment-Ruxolitinib-associated disseminated tuberculosis: A case report and literature review.

IDCases 2020 26;21:e00892. Epub 2020 Jun 26.

Center for Infectious Diseases, Nara Medical University, Shijo-cho, Kashihara, Nara, Japan.

Ruxolitinib, a Janus kinase inhibitor, considerably improves symptoms of patients with polycythemia vera and primary or secondary myelofibrosis. However, its association with the development of infectious complications is a concern. Herein, we report the case of an 80-year-old man with primary myelofibrosis who developed disseminated tuberculosis during treatment with ruxolitinib at 15 mg twice daily and prednisone at 5 mg. Read More

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http://dx.doi.org/10.1016/j.idcr.2020.e00892DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7332526PMC

Aetiology of Myeloproliferative Neoplasms.

Cancers (Basel) 2020 Jul 6;12(7). Epub 2020 Jul 6.

Centre for Health Data Science, University of Aberdeen, Aberdeen AB24 3FX, UK.

Myeloproliferative neoplasms (MPNs) have estimated annual incidence rates for polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis of 0.84, 1.03, and 0. Read More

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http://dx.doi.org/10.3390/cancers12071810DOI Listing

Polycythemia Vera Presenting With Normal Hemoglobin and Hematocrit: A Rare Variant.

Cureus 2020 Jun 2;12(6):e8404. Epub 2020 Jun 2.

Hematology/Oncology, Brookdale University Hospital and Medical Center, Brooklyn, USA.

Polycythemia vera (PV) is a myeloproliferative neoplasm, and its diagnosis requires elevated hemoglobin level (>16.5 mg/dL in men and >16 mg/dL in women), bone marrow characteristics of PV (hypercellularity for age with trilineage growth), and presence of JAK2 (Janus kinase 2) mutations or subnormal erythropoietin level if JAK2 mutation is not present. There exists a subset of patients with normal hemoglobin and hematocrit due to either from dilution of the blood or from coincidental blood loss anemia but these patients still might have underlying PV. Read More

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http://dx.doi.org/10.7759/cureus.8404DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331897PMC

Real-World Outcomes of Ruxolitinib Treatment for Polycythemia Vera.

Clin Lymphoma Myeloma Leuk 2020 May 29. Epub 2020 May 29.

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

Introduction: Ruxolitinib is approved for the treatment of polycythemia vera (PV) with hydroxyurea resistance or intolerance. Approval was based on the phase III RESPONSE trial, which demonstrated efficacy in a highly selected patient population.

Materials And Methods: To characterize the tolerability and outcomes of ruxolitinib outside of a clinical trial, we performed a multi-center retrospective analysis of patients with PV treated with ruxolitinib at 11 participating sites across the United States. Read More

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http://dx.doi.org/10.1016/j.clml.2020.05.019DOI Listing

Blood pressure profile, sympathetic nervous system activity and subclinical target organ damage in patients with polycythemia vera.

Pol Arch Intern Med 2020 Jul 4. Epub 2020 Jul 4.

Introduction: Polycythemia vera (PV) is a rare myeloproliferative disease associated with an increased prevalence of hypertension and increased risk of cardiovascular complications. The precise mechanisms leading to elevation of blood pressure (BP) and secondary target organ damage remains, however, poorly understood.

Objectives: The aims were to: evaluate BP profile, assess sympathetic nervous system and renin-angiotensin system activities and provide a comprehensive assessment of subclinical target organ damage in PV patients. Read More

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http://dx.doi.org/10.20452/pamw.15473DOI Listing

Automated red blood cell depletion: Results from a validation trial of the Fenwal Amicus new red blood cell depletion program.

J Clin Apher 2020 Jul 3. Epub 2020 Jul 3.

Service d'Hémaphérèse Auto-Transfusion, APHM Hôpital de la Conception, Marseille, France.

Purpose: A new software release of the Fenwal Amicus device is now available for red blood cell (RBC) apheresis, offering RBC exchange, RBC depletion (RBCd)/RCB exchange, and RBCd procedures. The main goal of this study was to validate the new RBCd program through the accuracy of the patient's postprocedure hematocrit (HCT) (actual end HCT) to the HCT reported by the device at the end of the procedure (target end HCT). Secondary objectives were to assess the device-related significant adverse events, the patient's cellular losses, and the degree of device induced hemolysis. Read More

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http://dx.doi.org/10.1002/jca.21802DOI Listing

Three myeloproliferative neoplasms: An overview.

Nursing 2020 Aug;50(8):22-30

Jill Brennan-Cook is an assistant clinical professor of nursing at Duke University School of Nursing in Durham, N.C.

A group of rare hematologic cancers, myeloproliferative neoplasms (MPNs) evolve when bone marrow dysfunction causes overproduction of one or more blood cell types. This article explores the diagnosis, treatment, and nursing care of patients diagnosed with one of three classic MPNs: essential thrombocythemia, polycythemia vera, and primary myelofibrosis. Read More

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http://dx.doi.org/10.1097/01.NURSE.0000684184.44195.27DOI Listing

[Brain MRI-findings in Ph - negative myeloproliferative disorders].

Ter Arkh 2019 Jul 15;91(7):29-34. Epub 2019 Jul 15.

Research center of Neurology.

Myeloproliferative disorders (MPD) are accompanied by a high proportion of thrombotic complications, which may lead to cerebrovascular disease (CVD).

Aim: To describe MRI-findings in patients with Ph - negative MPD and evaluate any cerebrovascular disease.

Materials And Methods: We included 104 patients with Ph - negative MPD (age varied between 20 and 58) with clinical correlates of cerebrovascular pathology. Read More

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http://dx.doi.org/10.26442/00403660.2019.07.000329DOI Listing

Cluster-Like Headache Revealing Polycythemia Vera: A Case Report.

Authors:
Cyprian Popescu

Case Rep Neurol 2020 May-Aug;12(2):184-188. Epub 2020 Jun 10.

Victor Pauchet Clinic, Amiens, France.

Herein, we report on a 44-year-old man who presented with cluster headache (CH)-like pain triggered by polycythemia vera (PV). He had severe unilateral head pain attacks lasting about 30 min not associated with cranial autonomic symptoms. After the exclusion of secondary etiologies, the patient was screened for a neoplastic process through biological markers, and the diagnosis of PV was established. Read More

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http://dx.doi.org/10.1159/000508356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315213PMC

Clinical characteristics and brain MRI findings in myeloproliferative neoplasms.

J Neurol Sci 2020 Jun 19;416:116990. Epub 2020 Jun 19.

Department of Neurology, Nippon Medical School, Tokyo, Japan.

Background: Myeloproliferative neoplasms (MPNs) including polycythemia vera (PV) and essential thrombocythemia (ET) have an increased risk of ischemic stroke. However, little is known about brain morphological changes and the cerebral vasculature in MPNs. The aim of the present study is to clarify the prevalence rates of brain infarcts (BIs) on magnetic resonance imaging (MRI) and to assess the detailed clinical and MRI characteristics in those patients. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116990DOI Listing

Management of challenging myelofibrosis after JAK inhibitor failure and/or progression.

Blood Rev 2020 May 30:100716. Epub 2020 May 30.

UT Health San Antonio MD Anderson Cancer Center, University of Texas Health Science Center San Antonio, San Antonio, TX 78249, USA. Electronic address:

The myeloproliferative neoplasms (MPNs) encompass a heterogenous set of diseases that have variable survival, but in the setting of treatment refractory and progressive disease, prognosis has been characteristically poor. JAK inhibition with ruxolitinib or fedratinib therapy has become the first line treatment for symptomatic or intermediate to high risk myelofibrosis. However, after three years of ruxolitinib therapy, approximately half of all patients with myelofibrosis will likely have stopped treatment. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100716DOI Listing

JAK2, CALR, and MPL Mutations in Egyptian Patients With Classic Philadelphia-negative Myeloproliferative Neoplasms.

Clin Lymphoma Myeloma Leuk 2020 May 16. Epub 2020 May 16.

Clinical Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt; Clinical Pathology Department, Oncology Center Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt.

Background: Genetic mutations have been proven to be one of the major criteria in the diagnosis and distinction of different myeloproliferative neoplasm (MPN) subtypes. Therefore, the aim of this study was to determine the molecular profile of Egyptian patients with MPN subtypes and correlate with clinicopathological status.

Methods: A series of 200 patients with MPNs (92 polycythemia vera, 68 essential thrombocythemia, and 40 primary myelofibrosis) were included in this study. Read More

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http://dx.doi.org/10.1016/j.clml.2020.05.011DOI Listing

Long-term efficacy and safety of ruxolitinib in polycythaemia vera - Authors' reply.

Lancet Haematol 2020 07;7(7):e506

Centre d'Investigations Cliniques, Hôpital Saint-Louis, Assitance Publique - Hôpitaux de Paris, Université de Paris, Inserm, Paris 75010, France. Electronic address:

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http://dx.doi.org/10.1016/S2352-3026(20)30141-1DOI Listing

Long-term efficacy and safety of ruxolitinib in polycythaemia vera.

Lancet Haematol 2020 07;7(7):e505-e506

Navitas Clinic Kawasaki, Kanagawa, Japan.

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http://dx.doi.org/10.1016/S2352-3026(20)30102-2DOI Listing

Genomic heterogeneity in myeloproliferative neoplasms and applications to clinical practice.

Blood Rev 2020 May 19:100708. Epub 2020 May 19.

Wellcome Sanger Institute, Hinxton, Cambridgeshire, UK; Cambridge Stem Cell Institute, Jeffrey Cheah Biomedical Centre, Cambridge Biomedical Campus, Puddicombe Way, Cambridge, UK; Department of Haematology, University of Cambridge, Cambridge, UK; Haematopathology and Oncology Diagnostics Service/ Department of Haematology, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, UK. Electronic address:

The myeloproliferative neoplasms (MPN) polycythaemia vera, essential thrombocythaemia and primary myelofibrosis are chronic myeloid disorders associated most often with mutations in JAK2, MPL and CALR, and in some patients with additional acquired genomic lesions. Whilst the molecular mechanisms downstream of these mutations are now clearer, it is apparent that clinical phenotype in MPN is a product of complex interactions, acting between individual mutations, between disease subclones, and between the tumour and background host factors. In this review we first discuss MPN phenotypic driver mutations and the factors that interact with them to influence phenotype. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100708DOI Listing

Myeloproliferative neoplasms treated with hydroxyurea, pegylated interferon alpha-2A or ruxolitinib: clinicohematologic responses, quality-of-life changes and safety in the real-world setting.

Hematology 2020 Dec;25(1):247-257

Department of Medicine, The University of Hong Kong, Hong Kong, People's Republic of China.

Real-world data of responses, quality-of-life (QOL) changes and adverse events in patients with myeloproliferative neoplasms (MPN) on conventional therapy (hydroxyurea ± anagrelide), pegylated interferon alpha-2A (PEG-IFNα-2A) or ruxolitinib are limited. We prospectively studied MPN patients receiving conventional therapy, PEG-IFNα-2A or ruxolitinib. Next-generation sequencing of 69 myeloid-related genes was performed. Read More

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http://dx.doi.org/10.1080/16078454.2020.1780755DOI Listing
December 2020
1.189 Impact Factor

Novel agents for the treatment of polycythemia vera: an insight into preclinical research and early phase clinical trials.

Expert Opin Investig Drugs 2020 Jul 16:1-9. Epub 2020 Jul 16.

Department of Hematology and Oncology, UT Health Science Center San Antonio MD Anderson Cancer Center , San Antonio, Texas, USA.

Introduction: Current treatment for polycythemia vera (PV) is limited and primarily targets thrombosis risk. Agents targeting distinct mechanisms of action within myeloproliferation are undergoing clinical evaluation to optimize efficacy, improve tolerance and augment long term disease complications.

Area Covered: This article reviews the current data from completed early phase clinical trials in PV, either as monotherapy or in combination with the few currently approved agents. Read More

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http://dx.doi.org/10.1080/13543784.2020.1782886DOI Listing

Parathyroid Adenoma as a Rare Cause of Persistent Hypercalcemia in a Female with Polycythemia Vera.

Case Rep Oncol 2020 May-Aug;13(2):578-582. Epub 2020 May 26.

Hamad Medical Corporation, Doha, Qatar.

Polycythemia vera is one of the myeloproliferative neoplasms that is distinguished by the uncontrolled production of blood cells and an increased red cell mass due to acquired mutation. It has many complications and it might increase the risk of other tumors. However, it does not cause hypercalcemia and is rarely associated with parathyroid adenoma. Read More

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http://dx.doi.org/10.1159/000507362DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7275195PMC

Risk of infections in patients with myeloproliferative neoplasms-a population-based cohort study of 8363 patients.

Leukemia 2020 Jun 16. Epub 2020 Jun 16.

Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden.

Infections are a common complication in patients with many hematologic malignancies, however, whether patients with myeloproliferative neoplasms (MPN) also are at an increased risk of infections is largely unknown. To assess the risk of serious infections, we performed a large population-based matched cohort study in Sweden including 8 363 MPN patients and 32,405 controls using high-quality registers between the years 1992-2013 with follow-up until 2015. The hazard ratio (HR) of any infection was 2. Read More

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http://dx.doi.org/10.1038/s41375-020-0909-7DOI Listing

Current management strategies for polycythemia vera and essential thrombocythemia.

Blood Rev 2020 Jun 3:100714. Epub 2020 Jun 3.

CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi, Firenze, DENOTHE Excellence Center, Italy. Electronic address:

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms characterized by increased rate of cardiovascular events, a varying burden of symptoms, and an intrinsic risk of evolution to secondary forms of myelofibrosis and acute leukemia; however, survival is only modestly reduced in most instances. In the last few years, following the description of driver mutations in JAK2, MPL and CALR, the diagnostic criteria for PV and ET were revised, making the identification of very early stages feasible. Scores for identifying patients at different risk of thrombosis were refined, and they largely guide therapeutic decisions. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100714DOI Listing

Sex determines the presentation and outcomes in MPN and is related to sex-specific differences in the mutational burden.

Blood Adv 2020 Jun;4(12):2567-2576

Division of Adult Hematology, Department of Medicine.

The factors underlying the variable presentation and clinical course of myeloproliferative neoplasms (MPNs) remain unclear. The aim of this study was to evaluate the independent effect of sex on MPN presentation and outcomes. A total of 815 patients with essential thrombocytosis, polycythemia vera, or primary myelofibrosis were evaluated between 2005 and 2019, and the association of sex with presenting phenotype, JAK2 V617F burden, progression, and survival was examined. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019001407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322953PMC

Effect of Tc elution in vivo from red cells on red cell volumes measured using autologous Tc-labeled red cells: comparison with Cr method.

Ann Biol Clin (Paris) 2020 Jun;78(3):319-322

Unité de radiopharmacie, Centre Georges François Leclerc, Dijon, France, Département de biologie et de pathologie des tumeurs, Unité de biologie clinique, Centre Georges François Leclerc, Dijon, France.

The purpose of this work was to compare the measured red-cell volume (RCV) using sodium pertechnétate [RCV-Tc] compared to the reference technique using sodium radiochromate [RCV-Cr] and to assess the influence of technetium-99 elution on the RCV-Tc value. Ten patients had simultaneous measurements of RCV-Tc and RCV-Cr. Elution of Tc-99m from red blood cells was 2. Read More

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http://dx.doi.org/10.1684/abc.2020.1547DOI Listing

Paraneoplastic small vessel vasculitis and Takayasu arteritis associated with polycythemia rubra vera.

Int J Rheum Dis 2020 Jul 15;23(7):986-988. Epub 2020 Jun 15.

Department of General Medicine, M.E.S. Medical College, Perinthalmanna, India.

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http://dx.doi.org/10.1111/1756-185X.13882DOI Listing

A Rare Case of Essential Thrombocythemia with Coexisting and Driver Mutations.

J Korean Med Sci 2020 Jun 15;35(23):e168. Epub 2020 Jun 15.

Division of Hematology & Oncology, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea.

Philadelphia-negative (Ph-) classical myeloproliferative neoplasms (MPNs) include polycythemia vera, essential thrombocythemia (ET), and primary myelofibrosis. Somatic driver mutations in the , , and genes serve as major diagnostic criteria of the Ph- MPNs and these mutations occur in a mutually exclusive manner. In this report, we describe the first case of ET harboring double mutations in V617F and . Read More

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http://dx.doi.org/10.3346/jkms.2020.35.e168DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295601PMC

Reduced CXCR4-expression on CD34-positive blood cells predicts outcomes of persons with primary myelofibrosis.

Leukemia 2020 Jun 14. Epub 2020 Jun 14.

Center for the Study of Myelofibrosis, Laboratory of Biochemistry, Biotechnology and Advanced Diagnostics, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation, Pavia, Italy.

The expression of the CXCR4 chemokine receptor on CD34-positive blood cells is reduced in persons with primary myelofibrosis (PMF). We analyzed the relevance of cytofluorimetric assessment of the percentage of CD34-positive blood cells that had a positive CXCR4 surface expression (CD34/CXCR4-se) in a large cohort of subjects with myeloproliferative neoplasms. Mean CD34/CXCR4-se was lower in subjects with PMF compared with those with essential thrombocythemia (ET) or polycythemia vera (PV). Read More

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http://dx.doi.org/10.1038/s41375-020-0926-6DOI Listing

Prognostic models in the myeloproliferative neoplasms.

Authors:
Jacob Grinfeld

Blood Rev 2020 May 30:100713. Epub 2020 May 30.

Department of Paediatric Haematology, Leeds General Infirmary, Great George St, Leeds LS1 3EX, UK. Electronic address:

The management of myelofibrosis (MF) is predominantly supportive, with the use of JAK2 inhibitors or allogeneic stem cell transplantation reserved for patients predicted to have poor overall survival. Identification of these patients is aided by a number of prognostic scoring systems, foremost among them the Dynamic International Prognostic Scoring System (DIPSS). Similarly, the use of cytoreductive therapies in essential thrombocytosis (ET) and polycythemia vera (PV) is targeted to patients identified as at highest risk of thrombosis. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100713DOI Listing

Epidemiology of the classical myeloproliferative neoplasms: The four corners of an expansive and complex map.

Blood Rev 2020 May 22:100706. Epub 2020 May 22.

Section of Hematology, Department of Internal Medicine, Yale University School of Medicine, New Haven, USA; Cancer Outcomes, Public Policy, and Effectiveness Research (COPPER) Center, Yale University, New Haven, USA. Electronic address:

The classical myeloproliferative neoplasms (MPNs), specifically chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF), represent clonal myeloid disorders whose pathogenesis is driven by well-defined molecular abnormalities. In this comprehensive review, we summarize the epidemiological literature and present our own analysis of the most recent the Surveillance, Epidemiology, and End Results (SEER) program data through 2016. Older age and male gender are known risk factors for MPNs, but the potential etiological role of other variables is less established. Read More

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http://dx.doi.org/10.1016/j.blre.2020.100706DOI Listing

Off-label studies on ruxolitinib in dermatology: a review.

J Dermatolog Treat 2020 Jun 9:1-7. Epub 2020 Jun 9.

Division of Dermatology, Harbor-UCLA Medical Center, Torrance, CA, USA.

Ruxolitinib is a Janus kinase (JAK) inhibitor that is FDA-approved for the treatment of myelofibrosis, polycythemia vera, and acute graft-versus-host disease. Its use in treating various dermatologic diseases has been a topic of growing interest due to its favorable safety profile and targeted inhibition of several cytokines that perpetuate inflammatory skin conditions. The PubMed/MEDLINE and ClinicalTrials. Read More

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http://dx.doi.org/10.1080/09546634.2020.1773385DOI Listing