8,756 results match your criteria Polycythemia Vera


Immunoblotting-assisted assessment of JAK/STAT and PI3K/Akt/mTOR signaling in myeloproliferative neoplasms CD34+ stem cells.

Methods Cell Biol 2022 26;171:81-109. Epub 2022 Jul 26.

Center for Research and Innovation for Myeloproliferative Neoplasms, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. Electronic address:

Philadelphia-negative myeloproliferative neoplasms (pH-MPNs) origin from the clonal expansion of hematopoietic stem cells with acquired mutations leading to uncontrolled proliferation of differentiated myeloid cells. The main entities of Ph-MPNs are represented by Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Myelofibrosis (MF) that are characterized by microvascular disorders, thrombosis and bleeding, splenomegaly secondary to extramedullary hematopoiesis, various degree of bone marrow fibrosis and a progressive risk of leukemic transformation. Somatic mutations in myeloid genes including JAK2, CALR, and MPL cause the constitutive activation of the Janus Kinase 2 (JAK)/signal transducer and activator of transcription (STAT) pathway that confers proliferative and differentiative advantage to mutated hematopoietic progenitors and ultimately drives the development of a Ph-MPNs phenotype. Read More

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Recurrent and Multiple Intracerebral Hemorrhages in Polycythemia Vera Secondary to Myelofibrosis: A Case Report and Literature Review.

Case Rep Neurol 2022 May-Aug;14(2):274-280. Epub 2022 Jun 7.

Department of Neurology, Juntendo University Faculty of Medicine, Tokyo, Japan.

Polycythemia vera (PV) is one of the myeloproliferative neoplasms and has higher frequency of the V617F mutation. Hemorrhagic stroke is rare in PV, and myelofibrosis is secondary to PV. A 76-year-old Japanese man was diagnosed as PV with the V617F mutation at the age of 63 years. Read More

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Successful Chemical Synovectomy in a Patient with Acquired von Willebrand Syndrome with Chronic Synovitis Due to Recurrent Knee Hemarthrosis: A Case Report.

Rheumatol Ther 2022 Aug 10. Epub 2022 Aug 10.

Department of Pathophysiology and Transplantation, University of Milan, Via Pace, 9, 20122, Milan, Italy.

Acquired von Willebrand syndrome (AVWS) is a rare, non-hereditary bleeding disorder related to heterogeneous medical conditions such as hematological malignancies and cardiovascular and autoimmune diseases. We describe the clinical course of a 62-year-old man with polycythemia vera who experienced post-traumatic knee and leg swelling due to hemarthrosis. He was treated at another center with low molecular weight heparin due to misdiagnosed deep vein thrombosis further exacerbating the ongoing bleeding. Read More

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Advances in Risk Stratification and Treatment of Polycythemia Vera and Essential Thrombocythemia.

Curr Hematol Malig Rep 2022 Aug 6. Epub 2022 Aug 6.

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

Purpose Of Review: Estimating and modifying thrombotic risk is currently the mainstay of care for patients with polycythemia vera (PV) and essential thrombocythemia (ET). In recent years, however, increased attention has shifted towards quality of life and disease modification. In this review, we discuss recent advances in risk stratification, present updated results for ruxolitinib and interferon randomized clinical trials, discuss new approaches in antiplatelet and anticoagulant treatment, and summarize early phase trials of novel agents and emerging therapeutic concepts for the treatment of PV and ET. Read More

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Hematocrit, White Blood Cells, and Thrombotic Events in the Veteran Population With Polycythemia Vera.

Authors:
Tsewang Tashi

Fed Pract 2022 May 16;39(Suppl 2):S43-S46. Epub 2022 Mar 16.

Huntsman Cancer Institute, University of Utah and Salt Lake City Veterans Affairs Medical Center, Salt Lake City, Utah.

Background: Patients with polycythemia vera (PV), a chronic myeloproliferative neoplasm, have a greater morbidity and mortality risk than the general population, largely due to a high incidence of thrombotic events.

Observations: Two recently published retrospective analyses from Parasuraman and colleagues used Veterans Health Administration (VHA) data to replicate, in a real-world population, findings from the prospective, randomized Cytoreductive Therapy in Polycythemia Vera (CYTO-PV) study. In the CYTO-PV study, hematocrit (Hct) level and white blood cell (WBC) count were shown to be independently associated with thrombotic event risk in patients with PV. Read More

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SURPASS-ET: phase III study of ropeginterferon alfa-2b versus anagrelide as second-line therapy in essential thrombocythemia.

Future Oncol 2022 Aug 4. Epub 2022 Aug 4.

UT Health San Antonio Cancer Center, San Antonio, TX 78229, USA.

Patients diagnosed with high-risk essential thrombocythemia (ET) have limited treatment options to reduce the risk of thrombosis and lessen the progression of the disease by targeting the molecular source. Hydroxyurea is the recommended treatment, but many patients experience resistance or intolerance. Anagrelide is an approved second-line option for ET, but concerns of a higher frequency of disease transformation may affect its role as a suitable long-term option. Read More

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Reversible striatal hypermetabolism of acquired chorea associated with polycythemia vera.

Acta Neurol Belg 2022 Aug 3. Epub 2022 Aug 3.

Department of Neurology, Kyungpook National University Hospital, Daegu, 41944, Republic of Korea.

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The prevalence of JAK2 exon12 mutations in Vietnamese patients with JAK2 V617F-negative polycythemia vera: frequent or rare?

Hum Hered 2022 Aug 3. Epub 2022 Aug 3.

Purpose Polycythemia vera is a hematological malignancy characterized by the overproduction of red blood cells in the bone marrow. Pathogenesis of Polycythemia vera was thought to be caused by genetic mutations of the Janus kinase 2 (JAK2) gene, especially the JAK2 V617F and exon 12 mutations since those mutations were found frequently in the patients. The prevalence of JAK2 exon 12 mutations among Polycythemia Vera patients in Vietnam has not been studied yet. Read More

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Delayed diagnosis of polycythaemia vera in an adult female with non-cirrhotic portal hypertension.

Ghana Med J 2022 Mar;56(1):38-41

Department of Haematology, College of Health Sciences, University of Ghana, Accra, Ghana.

Polycythaemia vera (PV) is a rare myeloproliferative neoplasm characterized primarily by erythrocytosis and an increased risk of thrombosis. We report a case of PV in a 60-year-old female with diabetes mellitus (DM) and a past history of recurrent abdominal pain and documented oesophageal varices who was followed up for 2 years as a case of non-cirrhotic portal hypertension of unknown cause. PV was only diagnosed after persistent complaints of vaso-motor symptoms and better scrutiny of full blood count results. Read More

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Second Malignancies among Older Patients with Classical Myeloproliferative Neoplasms Treated with Hydroxyurea.

Blood Adv 2022 Aug 2. Epub 2022 Aug 2.

Yale University, New Haven, Connecticut, United States.

Patients with classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), including polycythemia vera (PV), essential thrombocythemia (ET), primary and secondary myelofibrosis (MF), are known to have an increased risk of second malignancies (SM). Hydroxyurea (HU) is a guideline-recommended cytoreductive therapy for high-risk MPN patients. Controversy exists as to whether HU use is associated with a higher risk of SM including acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). Read More

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The differences of hemogram, myelogram, and driver gene mutations in classic myeloproliferative neoplasms.

Blood Cells Mol Dis 2022 11 20;97:102698. Epub 2022 Jul 20.

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China. Electronic address:

The aim of this study was to explore and compare routine blood features and pathological characteristics of bone marrow tissues in essential thrombocythemia (ET), polycythemia vera (PV), primary myelofibrosis, prefibrotic stage (prePMF) and overt fibrotic stage (overtPMF), and the correlation between common driver gene mutations and clinical manifestations of myeloproliferative neoplasms (MPN). Methods: We analyzed 259 MPN patients treated at Tongji Hospital of Huazhong University of Science and Technology from January 2016 to December 2020. Results: Among ET, PV, prePMF, and overtPMF, the median leukocyte counts of PV and prePMF were significantly higher than those of ET. Read More

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November 2022

Cases in the management of polycythemia vera: the importance of strict hematocrit control.

Authors:
Raajit K Rampal

Clin Adv Hematol Oncol 2022 Jul;20 Suppl 13(7):1-8

Memorial Sloan Kettering Cancer Center, New York, New York.

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Healthcare Resource Utilization in Patients with Myeloproliferative Neoplasms: A Danish Nationwide Matched Cohort Study.

Eur J Haematol 2022 Jul 28. Epub 2022 Jul 28.

Department of Hematology, Zealand University Hospital, Roskilde, Denmark.

Few studies have assessed healthcare resource utilization (HRU) in patients with Philadelphia-negative myeloproliferative neoplasms (MPN) using a matched cohort design. Further, no detailed assessment of HRU in the years preceding an MPN diagnosis exists. We conducted a registry-based nationwide Danish cohort study, including patients with essential thrombocythemia, polycythemia vera, myelofibrosis, and unclassifiable MPN diagnosed between January 2010 and December 2016. Read More

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Prevalence and clinical outcomes of polycythemia vera and essential thrombocythemia with hydroxyurea resistance or intolerance.

Hematology 2022 Dec;27(1):813-819

Faculty of Medicine, Department of Internal Medicine, Chiang Mai University, Chiang Mai, Thailand.

Introduction: To determine the prevalence, clinical outcomes, and factors associated with hydroxyurea (HU) resistance or intolerance among polycythemia vera (PV) and essential thrombocythemia (ET) patients.

Methods: This study was a retrospective cohort study including PV and ET patients diagnosed by WHO criteria and treated with HU between January 2000 and June 2020. Clinical features, laboratory data, and resistance or intolerance of HU were collected. Read More

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December 2022

Hematocrit Self-Testing in Patients with Polycythemia Vera and Other Hematological Conditions: Assessing the Accuracy of the StatStrip Xpress 2 LAC/Hb/Hct Device and User Opinion about the Device in Real-World Clinical Practice.

J Clin Med 2022 Jul 21;11(14). Epub 2022 Jul 21.

Clinic for Medical Oncology and Hematology, Kantonsspital St. Gallen, 9000 St. Gallen, Switzerland.

Maintaining hematocrit (Hct) levels below 45% can reduce morbidity and mortality in patients with polycythemia vera (PV). A device that patients can use to self-monitor Hct levels could enable timely interventions if Hct levels increase above 45%, and could improve quality of life (QoL). This study evaluated the accuracy of the StatStrip Xpress 2 LAC/Hb/Hct meter (Hb/Hct meter) when used by healthcare professionals (HCPs) or patients in clinical practice. Read More

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A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights.

Int J Mol Sci 2022 Jul 17;23(14). Epub 2022 Jul 17.

Dipartimento di Scienze Della Salute Della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy.

Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) represent a group of hematological disorders that are traditionally considered as indistinct slow progressing conditions; still, a subset of cases shows a rapid evolution towards myelofibrotic bone marrow failure. Specific abnormalities in the megakaryocyte lineage seem to play a central role in this evolution, especially in the bone marrow fibrosis but also in the induction of myeloproliferation. In this review, we analyze the current knowledge of prognostic factors of MPNs related to their evolution to myelofibrotic bone marrow failure. Read More

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Severe Acute Respiratory Syndrome Coronavirus 2 Omicron Variant in Patients with Philadelphia-Negative Myeloproliferative Neoplasm: A Single Center Experience.

Oncology 2022 26;100(8):460-466. Epub 2022 Jul 26.

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

Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the viral agent responsible for the coronavirus disease of 2019. The disease is primarily a respiratory illness; however, multisystem involvement is not uncommon. The infection is reported to be more severe in patients with multiple comorbidities and immunocompromised patients. Read More

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Janus kinase (JAK) inhibitors in the treatment of neoplastic and inflammatory disorders.

Authors:
Robert Roskoski

Pharmacol Res 2022 Jul 22;183:106362. Epub 2022 Jul 22.

Blue Ridge Institute for Medical Research, 3754 Brevard Road, Suite 106, Box 19, Horse Shoe, NC 28742, United States. Electronic address:

The Janus kinase (JAK) family of nonreceptor protein-tyrosine kinases consists of JAK1, JAK2, JAK3, and TYK2 (Tyrosine Kinase 2). Each of these proteins contains a JAK homology pseudokinase (JH2) domain that interacts with and regulates the activity of the adjacent protein kinase domain (JH1). The Janus kinase family is regulated by numerous cytokines including interferons, interleukins, and hormones such as erythropoietin and thrombopoietin. Read More

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An Polycythemia Vera Evolve from Acute Myeloid Leukemia? Report of a Case Showing a Simultaneous Minor V617F Mutated Clone.

Mediterr J Hematol Infect Dis 2022 1;14(1):e2022058. Epub 2022 Jul 1.

Department of Biomedicine and Prevention, University of Tor Vergata, Rome, Italy.

The evolution of myeloproliferative neoplasms (MPN) to acute myeloid leukemia (AML) occurs in 2-10% of patients, depending on the MPN subtype, treatment, and follow-up length. The reverse-path from AML to MPN has been rarely reported. We herein present a 75 years old woman with AML, in whom a -V617F positive polycythemia vera (PV) emerged during follow-up, 19 months from the end of consolidation treatment. Read More

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The Outcome of Fatherhood in Patients With Philadelphia-Negative Myeloproliferative Neoplasms: A Single-Institution Experience.

Cureus 2022 Jun 15;14(6):e25953. Epub 2022 Jun 15.

Hematology and Oncology, Hamad General Hospital, Doha, QAT.

Background Fertility is a highly complex subject; it involves more than one individual and has profound psychological and economic implications. Moreover, it is affected by several factors, including age, significant systemic illness in either partner, exposure to environmental toxins, medications, or radiation. In patients with malignancy, fertility is more complicated. Read More

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Osteolytic lesion in polycythemia vera: First report and review of literature.

EJHaem 2022 May 27;3(2):526-532. Epub 2022 Mar 27.

Princess Margaret Cancer Centre University of Health Network Toronto Canada.

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Revisiting the impact of serial therapeutic phlebotomy in polycythaemia on laboratory and clinical parameters using a fixed interval and fixed volume protocol.

Transfus Clin Biol 2022 Jul 11. Epub 2022 Jul 11.

Department of Transfusion Medicine, Government Medical College and Hospital, Chandigarh, India.

Objectives: Therapeutic phlebotomy allows for a controlled and gradual decrease in red cell mass leading to improved blood flow and symptomatic relief in polycythaemia. The present study was aimed to determine the impact of serial fixed volume and fixed interval therapeutic phlebotomy protocol on the laboratory and clinical parameters in patients of polycythaemia.

Material And Methods: This prospective longitudinal study was conducted over 18 months. Read More

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Ischemic enteritis resulting from polycythemia vera.

Clin J Gastroenterol 2022 Jul 13. Epub 2022 Jul 13.

Department of Gastrointestinal Surgery, Osaka City General Hospital, 2-13-22, Miyakojima-hondori, Miyakojima-ku, Osaka, 534-0021, Japan.

Polycythemia vera is a chronic myeloproliferative disorder characterized by clonal proliferation of bone marrow progenitors. We report a case of ischemic enteritis and bowel obstruction resulting from polycythemia vera. A 76-year-old man was admitted to our hospital with abdominal distention. Read More

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Vascular Endothelial Dysfunction in Myeloproliferative Neoplasms and Gene Mutations.

Int Heart J 2022 Jul 14;63(4):661-668. Epub 2022 Jul 14.

Department of Cardiovascular Medicine, Nippon Medical School.

Essential thrombocythemia (ET) and polycythemia vera (PV), are common Philadelphia-negative myeloproliferative neoplasms (MPN). Patients with MPN have a high rate of cardiovascular complications and often have acquired JAK2V617F and CALR genetic mutations. In this study, we aimed to analyze vascular endothelial function in patients with MPN. Read More

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Splenic Infarctions in Polycythaemia Vera are Not Always a Catastrophe.

Eur J Case Rep Intern Med 2022 29;9(6):003370. Epub 2022 Jun 29.

Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.

Splenic infarction can occur as an infrequent thrombotic manifestation in polycythaemia vera (PV) and is usually catastrophic. We describe the case of a middle-aged woman who was diagnosed with PV 3 months before she presented to the emergency department with acute limb ischaemia. A splenic infarction detected on diagnostic imaging during her hospital stay was treated conservatively with modification of her hydroxyurea dose along with pain management, without the need for surgery. Read More

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Lights Off, Lights On: Amaurosis Fugax in Polycythemia.

Cureus 2022 Jun 8;14(6):e25752. Epub 2022 Jun 8.

Ophthalmology, Hospital Kuala Lumpur, Kuala Lumpur, MYS.

There are many causes of amaurosis fugax, including polycythemia. Polycythemia is associated with elevated hematocrit levels and hyperviscosity, which can lead to ocular manifestations. We report a polycythemia patient with amaurosis fugax, who had resolution of ocular symptoms following venesection. Read More

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Clinical characteristics of Japanese patients with polycythemia vera: results of the JSH-MPN-R18 study.

Int J Hematol 2022 Jul 9. Epub 2022 Jul 9.

Department of Hematology, Juntendo University Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.

The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Read More

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A Case Report of Large Bilateral Pulmonary Embolism in a Patient With Janus Kinase 2 (JAK2) Positive Mutation.

Cureus 2022 May 30;14(5):e25482. Epub 2022 May 30.

Cardiology, Royal Free Hospital, London, GBR.

Venous thromboembolism may be the primary presentation in patients with polycythaemia vera (PV) and essential thrombocythemia. Most patients get diagnosed with polycythaemia vera after presenting with venous or arterial thromboembolism in the first place. Most patients tend to develop thrombosis just before or at the time of diagnosis, and this risk decreases over time. Read More

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Bilateral Adrenal Hemorrhage and Adrenal Insufficiency in the Context of Polycythemia Vera: A Case Report and Review of the Literature.

Case Rep Med 2022 22;2022:5335543. Epub 2022 Jun 22.

Isfahan Endocrine and Metabolism Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.

Background: Polycythemia vera (PV) is a myeloproliferative disorder presented with different manifestations. However, bilateral adrenal hemorrhage (BAH) and adrenal insufficiency (AI) are rare manifestations. Herein, we described a patient who suffered from BAH and AI in the context of PV. Read More

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