8,449 results match your criteria Polycythemia Vera


Improving the investigative approach to polycythaemia vera: a critical assessment of current evidence and vision for the future.

Lancet Haematol 2021 Aug;8(8):e605-e612

Division of Hematology and Medical Oncology, Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA. Electronic address:

Polycythaemia vera is a challenging disease to study given its low prevalence and prolonged time-to-event for important clinical endpoints such as thrombosis, progression, and mortality. Although researchers in this space often rise to meet these challenges, there is considerable room for improvement in the analysis of retrospective data, the development of risk-stratification tools, and the design of randomised controlled trials. In this Viewpoint, we review the evidence behind the contemporary approach to risk stratification and treatment of polycythaemia vera. Read More

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Lower response to BNT162b2 vaccine in patients with myelofibrosis compared to polycythemia vera and essential thrombocythemia.

J Hematol Oncol 2021 Jul 29;14(1):119. Epub 2021 Jul 29.

Hematology Unit, Department of Research and Clinical Oncology, IRCCS Regina Elena National Cancer Institute, Via Elio Chianesi 53, 00144, Rome, Italy.

In a population of 42 Philadelphia negative myeloproliferative neoplasm patients, all on systemic active treatment, the likelihood of responding to anti-SARS-CoV-2 BNT162b2 vaccine at 2 weeks after the second dose was significantly lower in the ten patients with myelofibrosis compared to the 32 with essential thrombocythemia (n = 17) and polycythemia vera (n = 15) grouped together, both in terms of neutralizing anti-SARS-CoV-2 IgG titers and seroprotection rates (32.47 AU/mL vs 217.97 AU/mL, p = 0. Read More

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Selective Inhibition of JAK1 Primes STAT5-Driven Human Leukemia Cells for ATRA-Induced Differentiation.

Target Oncol 2021 Jul 29. Epub 2021 Jul 29.

Cancer Biology Program, Vanderbilt University School of Medicine, Nashville, TN, USA.

Background: All-trans retinoic acid (ATRA), a derivate of vitamin A, has been successfully used as a therapy to induce differentiation in M3 acute promyelocytic leukemia (APML), and has led to marked improvement in outcomes. Previously, attempts to use ATRA in non-APML in the clinic, however, have been underwhelming, likely due to persistent signaling through other oncogenic drivers. Dysregulated JAK/STAT signaling is known to drive several hematologic malignancies, and targeting JAK1 and JAK2 with the JAK1/JAK2 inhibitor ruxolitinib has led to improvement in survival in primary myelofibrosis and alleviation of vasomotor symptoms and splenomegaly in polycythemia vera and myelofibrosis. Read More

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NOAC in patients with VTE and polycythemia vera or essential thrombocythemia: a cohort study.

J Cardiovasc Pharmacol 2021 Jul 19. Epub 2021 Jul 19.

Faculty of Medicine and Health Science, Andrzej Frycz Modrzewski Krakow University, Krakow, Poland John Paul II Hospital, Krakow, Poland Institute of Cardiology, Jagiellonian University Medical College, Krakow, Poland.

Abstract: Thrombosis is the most common adverse event in patients with polycythemia vera (PV) and essential thrombocythemia (ET). Little is known about the use of non-vitamin K antagonist oral anticoagulants (NOACs) in patients with myeloproliferative neoplasms (MPN). We sought to evaluate the efficacy and safety of NOAC in a cohort of patients with PV and ET, who experienced venous thromboembolism (VTE). Read More

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Transcatheter Heart Valve Thrombosis in a Patient With Polycythemia Vera Despite Apixaban Therapy for Atrial Fibrillation.

JACC Case Rep 2021 Feb 17;3(2):269-272. Epub 2021 Feb 17.

Department of Thoracic and Cardiovascular Surgery, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.

We present a case of transcatheter heart valve thrombosis in a 76-year-old man with paroxysmal atrial fibrillation on therapeutic anticoagulation with apixaban and polycythemia vera. The incidence of transcatheter heart valve thrombosis in patients with atrial fibrillation and on adequate anticoagulation is not well reported. (). Read More

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February 2021

Spontaneous Recanalization of Coronary Thrombus in a Patient With Polycythemia Vera.

JACC Case Rep 2020 Mar 18;2(3):497-498. Epub 2020 Mar 18.

Department of Cardiology, University and Hospital Fribourg, Switzerland.

Spontaneous recanalized coronary thrombi (SRCT), a rare and under-diagnosed entity, are old thrombus formations characterized by multiple communicating channels. We report the case of a 72-year-old female patient who presented with SRCT in the context of polycythemia vera. Optical coherence tomography (OCT) is the diagnostic method of choice. Read More

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Pediatric Myeloproliferative Neoplasms.

Clin Lab Med 2021 Sep;41(3):529-540

Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA; Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA. Electronic address:

Myeloproliferative neoplasms can present early in life and may present a diagnostic challenge. Very few studies have focused on the diagnosis, prognosis, and therapy for pediatric myeloproliferative neoplasms. This article focuses on chronic myeloid leukemia, essential thrombocythemia, polycythemia vera, and primary myelofibrosis in children. Read More

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September 2021

Clinical and Laboratory Features of JAK2 V617F, CALR, and MPL Mutations in Malaysian Patients with Classical Myeloproliferative Neoplasm (MPN).

Int J Environ Res Public Health 2021 Jul 16;18(14). Epub 2021 Jul 16.

Department of Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Malaysia.

Mutations of JAK2V617F, CALR, and MPL genes confirm the diagnosis of myeloproliferative neoplasm (MPN). This study aims to determine the genetic profile of JAK2V617F, CALR exon 9 Type 1 (52 bp deletion) and Type 2 (5 bp insertion), and MPL W515 L/K genes among Malaysian patients and correlate these mutations with clinical and hematologic parameters in MPN. Mutations of JAK2V617F, CALR, and MPL were analyzed in 159 Malaysian patients using allele-specific polymerase chain reaction, including 76 polycythemia vera (PV), 41 essential thrombocythemia (ET), and 42 primary myelofibrosis (PMF) mutations, and the demographics of the patients were retrieved. Read More

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Polycythemia Vera Presenting as Hemorrhagic Stroke.

J Neurosci Rural Pract 2021 Jul 5;12(3):601-602. Epub 2021 May 5.

Department of Neurology, MOSC Medical College, Kolenchery, Kochi, Kerala, India.

Patients with polycythemia vera are at high risk for vaso-occlusive events including cerebral ischemia and hemorrhage. Cerebral ischemic events are due to increased blood viscosity and platelet activation within the central nervous system arterial vessels. We present a case of a 65-year-old woman who presented to the emergency department with seizures followed by left-sided weakness. Read More

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Thrombotic and hemorrhagic events in 2016 World Health Organization-defined Philadelphianegative myeloproliferative neoplasm.

Korean J Intern Med 2021 Jul 22. Epub 2021 Jul 22.

Division of Hematology/Oncology, Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.

Background/aims: Recent changes in the diagnostic criteria for myeloproliferative neoplasms (MPNs) and increasing patient numbers necessitate updating of the data on vascular events in patients with such disorders.

Methods: In this single-center study, thrombotic and hemorrhagic events were retrospectively analyzed in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or PMF, based on the 2016 World Health Organization diagnostic criteria.

Results: Of a total of 335 consecutive patients (139 ET, 42 pre-PMF, 124 PV, and 30 PMF patients; 192 males and 143 females) of median age 64 years (range, 15 to 91), 112 (33. Read More

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A case of a primary myelofibrosis with progression and related literature review of progression phase genetics.

Int J Lab Hematol 2021 Jul;43 Suppl 1:78-81

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Philadelphia (BCR-ABL)-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN can transform into an accelerated or a blast phase, which is associated with poor response to standard therapy and low overall median survival. We present an interesting case of a patient with a history of PMF and progression and summarize the current studies on genetic features of myeloproliferative neoplasms in blast phase (MPN-BP) with an emphasis on PMF. Read More

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Autoimmune disorders and the risk of thrombotic events in polycythaemia vera.

Leuk Res 2021 Jul 13;110:106667. Epub 2021 Jul 13.

Department of Internal Medicine, General Hospital of Šibenik-Knin County, Šibenik, Croatia.

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Interferon-Alpha 2-a and Its Dual Effect in Treating Two Diseases (Hepatitis C and Polycythemia Vera).

Case Rep Oncol 2021 May-Aug;14(2):851-854. Epub 2021 Jun 11.

Department of Medical Oncology, Hamad Medical Corporation, Doha, Qatar.

Hepatitis C and polycythemia vera (PV) co-existence is not rare; it has been reported in the literature. Treatment with interferon (IFN) has been used to treat both conditions; however, the use of IFN in concomitant hepatitis C infection with PV and its outcome are rarely described in the literature. Here, we report a 56-years-old male patient with hepatitis C virus infection and PV, who was treated with IFN for his chronic hepatitis C, which resulted in significant improvement of HB as well as normalization of his bone marrow and eradication of the clone ( V617F). Read More

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Neuropsychiatric Derangement by Polycythemia Vera: A Case Report of an Unexpected Disease Presentation and Review of the Literature.

Acta Haematol 2021 Jul 9:1-6. Epub 2021 Jul 9.

Department of Geriatric Medicine, Jeroen Bosch Hospital, 's-Hertogenbosch, The Netherlands.

Cerebral infarction as well as other thromboses, headaches, and visual complaints are well-known symptoms of polycythemia vera. However, chorea and neuropsychiatric disturbances are less recognized consequences of this chronic disease. Whereas chorea is a rare but acknowledged symptom of polycythemia vera, neuropsychiatric symptoms have only sporadically been reported. Read More

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The first report of a JAK2 V617F-positive myeloproliferative neoplasm with initial manifestation as a rare pampiniform venous plexus thrombosis and review of the literature.

J Thromb Thrombolysis 2021 Jul 9. Epub 2021 Jul 9.

Department of Pathology, Microbiology, & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.

Pampiniform venous plexus (PVP) thrombosis is exceedingly rare, with fewer than 25 cases described. Thus, the etiology and pathophysiology remain largely unknown. A 38-year-old male with no known risk factors incidentally noted a 10-day history of right testicular discomfort prompting evaluation. Read More

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Diagnosis and management of neutrophilic myeloid neoplasms.

Clin Adv Hematol Oncol 2021 Jul;19(7):450-459

Division of Hematology, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, California.

Chronic neutrophilia is commonly seen with persistent infections, inflammatory disorders, smoking, solid tumors, and specific medications. However, after reactive causes have been excluded, a workup for primary (clonal) neutrophilic disorders, such as myeloproliferative neoplasms (MPNs) and myelodysplastic/myeloproliferative overlap syndromes, should be pursued. Except for chronic myeloid leukemia, which is defined by the presence of the Philadelphia (Ph) chromosome, and the classic Ph chromosome-negative MPNs (polycythemia vera, essential thrombocythemia, and primary myelofibrosis), clonal neutrophilic neoplasms historically have been challenging to diagnose and classify. Read More

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Successful use of phlebotomy to treat severe secondary polycythemia due to chronic lung disease.

Hematol Rep 2021 Jun 9;13(2):8961. Epub 2021 Jun 9.

Department of Medicine, West Virginia University, Morgantown, WV, USA.

Secondary polycythemia (SP) occurs as a result of increase erythropoietin levels most commonly as a result of tissue hypoxia. Symptoms such as erythromelalgia, pruritis, and bleeding, which are frequently seen in polycythemia vera (PV), do not commonly occur in SP. Phlebotomy is considered one of the mainstays of therapy for PV but is rarely used for treatment of SP due to concern about worsening tissue hypoxia. Read More

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[Perspectives on a new therapeutic approach for myeloproliferative neoplasms].

Authors:
Keita Kirito

Rinsho Ketsueki 2021 ;62(6):582-591

Department of Hematology and Oncology, University of Yamanashi.

After the discovery of driver mutations for myeloproliferative neoplasms (MPN), treatment approach for the disease has achieved tremendous progress. Ruxolitinib, a JAK inhibitor, is now widely used for both patients with myelofibrosis and polycythemia vera in several countries, including Japan. Fedratinib, another JAK inhibitor, has been recently approved in the United States. Read More

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VEGF Regulation of Angiogenic Factors via Inflammatory Signaling in Myeloproliferative Neoplasms.

Int J Mol Sci 2021 Jun 22;22(13). Epub 2021 Jun 22.

Department of Molecular Oncology, Institute for Medical Research, University of Belgrade, 11000 Belgrade, Serbia.

Background: Chronic inflammation has been recognized in neoplastic disorders, including myeloproliferative neoplasm (MPN), as an important regulator of angiogenesis.

Aims: We investigated the influence of vascular endothelial growth factor (VEGF) and pro-inflammatory interleukin-6 (IL-6) on the expression of angiogenic factors, as well as inflammation-related signaling in mononuclear cells (MNC) of patients with MPN and V617F positive human erythroleukemic (HEL) cells.

Results: We found that IL-6 did not change the expression of angiogenic factors in the MNC of patients with MPN and HEL cells. Read More

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The Prevalence of Gene Mutations in Patients with --Negative Myeloproliferative Neoplasms (MPN): A Systematic Review and Meta-Analysis.

Cancers (Basel) 2021 Jun 20;13(12). Epub 2021 Jun 20.

Department of Haematology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia.

Multiple recurrent somatic mutations have recently been identified in association with myeloproliferative neoplasms (MPN). This meta-analysis aims to assess the pooled prevalence of gene mutations among patients with MPN. Six databases (PubMed, Scopus, ScienceDirect, Google Scholar, Web of Science and Embase) were searched for relevant studies from inception till September 2020, without language restrictions. Read More

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Molecular pathogenesis of the myeloproliferative neoplasms.

J Hematol Oncol 2021 Jun 30;14(1):103. Epub 2021 Jun 30.

Patrick G Johnston Centre for Cancer Research, Queen's University Belfast, Belfast, UK.

The Philadelphia negative myeloproliferative neoplasms (MPN) compromise a heterogeneous group of clonal myeloid stem cell disorders comprising polycythaemia vera, essential thrombocythaemia and primary myelofibrosis. Despite distinct clinical entities, these disorders are linked by morphological similarities and propensity to thrombotic complications and leukaemic transformation. Current therapeutic options are limited in disease-modifying activity with a focus on the prevention of thrombus formation. Read More

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Repeat exit site infection in peritoneal dialysis patient with polycythemia vera - a case report.

BMC Infect Dis 2021 Jun 30;21(1):624. Epub 2021 Jun 30.

Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Al. Powstańców Wielkopolskich 72, 70-111, Szczecin, Poland.

Background: Infectious complications of peritoneal dialysis (PD) remain a common cause of catheter loss and discontinuation of PD. Exit site infection (ESI) constitutes a significant risk factor for PD-related peritonitis and determination of predisposing states is relevant. We here present a case of repeat ESI due to Pseudomonas aeruginosa in a PD patient with skin changes in the course of polycythemia vera (PV). Read More

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Coexistent antiphospholipid syndrome and myeloproliferative neoplasm.

Lupus 2021 Aug 1;30(9):1502-1508. Epub 2021 Jul 1.

Haemostasis Research Unit, Department of Haematology, University College London, London, UK.

Antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPN) are associated with an increased risk of thrombosis. The optimal management of patients with coexistent APS and MPN has not been defined. A single centre and systematic literature review of patients with coexistent APS and MPN was performed. Read More

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Current Concepts of Pathogenesis and Treatment of Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.

Hamostaseologie 2021 Jun 30;41(3):197-205. Epub 2021 Jun 30.

Division of Hematology, University Hospital Basel, Basel, Switzerland.

Philadelphia chromosome-negative myeloproliferative neoplasms are hematopoietic stem cell disorders characterized by dysregulated proliferation of mature myeloid blood cells. They can present as polycythemia vera, essential thrombocythemia, or myelofibrosis and are characterized by constitutive activation of signaling. They share a propensity for thrombo-hemorrhagic complications and the risk of progression to acute myeloid leukemia. Read More

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Toll-like receptor gene polymorphisms in patients with myeloproliferative neoplasms.

Mol Biol Rep 2021 Jun 30;48(6):4995-5001. Epub 2021 Jun 30.

Graduate Program in Biosciences and Physiopathology of the State University of Maringá, Av. Colombo 5790, bloco T20, sala 109, Maringá, PR, CEP: 87020-900, Brazil.

Toll-like receptors (TLRs) are a family of transmembrane receptors whose signaling control cellular processes of cell proliferation, survival, apoptosis, angiogenesis, remodeling, and repair of tissues. Polymorphisms in TLR genes can change the balance between pro and anti-inflammatory cytokines, modulating the risk of infection, chronic inflammation, and cancer. Although many studies have demonstrated the direct involvement of TLR signaling in the benefit of tumor cells in certain cancers, little is known about the influence of these gene polymorphisms on myeloproliferative neoplasms (MPNs). Read More

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Rupture of splenic artery aneurysm in a man with polycythemia vera and acquired von Willebrand syndrome.

BMJ Case Rep 2021 Jun 24;14(6). Epub 2021 Jun 24.

Radiology, Creighton University School of Medicine, Omaha, Nebraska, USA

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A New High Affinity Hemoglobin Variant: Hb San Francisco-KP (: c.104T>C).

Hemoglobin 2021 Jun 24:1-3. Epub 2021 Jun 24.

Department of Hematology Oncology, Oakland Medical Center, Kaiser Permanente Northern California, Oakland, CA, USA.

The evaluation of erythrocytosis can fail to detect hemoglobin (Hb) variants if a thorough and systemic investigation is not undertaken. Here we report the identification of a novel high-oxygen affinity Hb that was previously misclassified as polycythemia vera (PV). Given that treatment recommendations can vary significantly based on the etiology of erythrocytosis, familiarity with reference laboratories and their methodologies is of crucial importance to conducting a precise consultation, as in the case of our Hb variant, named Hb San Francisco-KP [β34(B16)Val→Ala, : c. Read More

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Renal post-mortem findings in myeloproliferative and myelodysplastic/myeloproliferative neoplasms.

Virchows Arch 2021 Jun 23. Epub 2021 Jun 23.

Institute of Medical Genetics and Pathology, University Hospital Basel, University of Basel, Schönbeinstrasse 40, 4031, PathologyBasel, Switzerland.

Myeloproliferative neoplasms (MPN) are a heterogeneous group of hematological disorders presenting with an increased proliferation in one or several hematological cell lines. Renal manifestations of MPN have not been fully characterized so far. To morphologically assess the potential renal involvement in MPN patients, we analyzed histomorphological findings of a post-mortem cohort (n = 57) with a disease history of Philadelphia-negative MPN including polycythaemia vera, primary myelofibrosis, essential thrombocythemia, or chronic myelomonocytic leukemia (CMML). Read More

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Polycythemia vera as a rare cause of hypertension in a young man.

BMJ Case Rep 2021 Jun 23;14(6). Epub 2021 Jun 23.

Department of Faculty Therapy #2, I M Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia.

Polycythemia vera (PV) is an orphan haematological disease and one of the most common myeloproliferative diseases, with the incidence rate of about 0.4-2.8 cases per 100 000 population per year. Read More

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Hemorrhage in patients with polycythemia vera receiving aspirin with an anticoagulant: a prospective, observational study.

Haematologica 2021 Jun 24. Epub 2021 Jun 24.

Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Atrium Health, Charlotte, NC.

Polycythemia vera (PV) is associated with increased risk of thrombosis and hemorrhage. Aspirin, recommended for primary thromboprophylaxis, is often combined with anticoagulants during management of acute thrombotic events. The safety of dual antiplatelet and anticoagulant therapy is not established in PV. Read More

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