4,916 results match your criteria Thrombocytosis Essential


Blood Cell Disorders and the Nervous System.

Continuum (Minneap Minn) 2020 Jun;26(3):659-674

Purpose Of Review: This article discusses the epidemiology, diagnosis, treatment, and prevention of neurologic complications of common and rare blood cell disorders.

Recent Findings: A growing number of preventive treatment options are available for stroke in sickle cell disease. Paroxysmal nocturnal hemoglobinuria and immune thrombocytopenia can lead to stroke. Read More

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http://dx.doi.org/10.1212/CON.0000000000000858DOI Listing

Thrombopoietin is required for full phenotype expression in a JAK2V617F transgenic mouse model of polycythemia vera.

PLoS One 2020 1;15(6):e0232801. Epub 2020 Jun 1.

Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America.

The myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis and primary myelofibrosis are hematopoietic stem cell disorders and share driver mutations that either directly activate the thrombopoietin receptor, MPL, or activate it indirectly through gain-of-function mutations in the gene for JAK2, its cognate tyrosine kinase. Paradoxically, MPL surface expression in hematopoietic stem cells is also reduced in the myeloproliferative neoplasms due to abnormal post-translational glycosylation and premature destruction of JAK2, suggesting that the myeloproliferative neoplasms are disorders of MPL processing since MPL is the only hematopoietic growth factor receptor in hematopoietic stem cells. To examine this possibility, we genetically manipulated MPL expression and maturation in a JAK2V617F transgenic mouse model of polycythemia vera. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0232801PLOS

The role of fedratinib for the treatment of patients with primary or secondary myelofibrosis.

Ther Adv Hematol 2020 15;11:2040620720925201. Epub 2020 May 15.

Division of Hematology and Oncology, UT Health San Antonio MD Anderson, Mail Code 8026, 6th Floor Urschel Tower, 7979 Wurzbach Rd, San Antonio, TX 78229-3900.

Myelofibrosis (MF) is a chronic myeloid neoplasm characterized by either primary myelofibrosis, or secondary MF following essential thrombocythemia or polycythemia vera. Historically, therapy has been symptom directed; however, in 2011, the first janus kinase inhibitor (JAK-i) - ruxolitinib - was approved for treatment. This medication was found to be effective in reduction of symptom burden and spleen size; however, the median duration of response is about 3 years. Read More

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http://dx.doi.org/10.1177/2040620720925201DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232117PMC

Essential thrombocythemia: a hemostatic view of thrombogenic risk factors and prognosis.

Mol Biol Rep 2020 May 30. Epub 2020 May 30.

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

Essential thrombocythemia (ET) is a classical myeloproliferative neoplasm that is susceptible to hypercoagulable state due to impaired hemostatic system, so that thrombotic complications are the leading cause of mortality in ET patients. The content used in this article has been obtained by the PubMed database and Google Scholar search engine from English-language articles (2000-2019) using the following keywords: "Essential thrombocythemia," "Thrombosis," "Risk factors" and "Hemostasis. In this neoplasm, the count and activity of cells such as platelets, leukocytes, endothelial cells, as well as erythrocytes are increased, which can increase the risk of thrombosis through rising intercellular interactions, expression of surface markers, and stimulation of platelet aggregation. Read More

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http://dx.doi.org/10.1007/s11033-020-05536-xDOI Listing

An agenda for future research projects in polycythemia vera and essential thrombocythemia.

Haematologica 2020 May 28. Epub 2020 May 28.

Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy.

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http://dx.doi.org/10.3324/haematol.2019.246207DOI Listing

Mutant specific anti calreticulin antibody (CAL2) immunohistochemistry as a screening test for calreticulin (CALR) mutation testing.

Int J Lab Hematol 2020 May 28. Epub 2020 May 28.

Department of Hematology, SGPGI, Lucknow, Uttar Pradesh, India.

Background: About 50 different CALR frameshift mutations have been identified in BCR-ABL1 negative MPN, all leading to the development of common new protein C terminus. Antibody targeting this terminal epitope can be useful to identify this driver mutation using immunohistochemistry.

Materials And Methods: CALR mutation analysis was carried out in 51 JAK2V617F negative cases, PMF (n = 22) and ET (n = 29). Read More

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http://dx.doi.org/10.1111/ijlh.13242DOI Listing
May 2020
1.870 Impact Factor

Limb gangrene: The first sign of essential thrombocythemia.

Indian J Med Res 2020 Apr;151(4):380-381

Division of Hematology, Faculty of Medicine, Adnan Menderes University, Aydın, Turkey.

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http://dx.doi.org/10.4103/ijmr.IJMR_8_19DOI Listing

MDS/MPN-RS-T justified inclusion as a unique disease entity?

Best Pract Res Clin Haematol 2020 Jun 17;33(2):101147. Epub 2020 Jan 17.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 428, Houston, TX, 77030, USA. Electronic address:

Myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a disease entity characterized by anemia, bone marrow dysplasia with ring sideroblasts and persistent thrombocytosis ≥450 × 10/L with proliferation of large and morphologically atypical megakaryocytes. Although initially recognized by the World Health Organization only as a provisional entity, next generation sequencing has identified recurrent somatic mutations in SF3B1, JAK2 and other genes providing further evidence of the clonal nature of this disease and the need to recognize it as a separate entity. Despite its overlapping features with MDS with ring sideroblasts and essential thrombocythemia, MDS/MPN-RS-T is characterized by specific clinical features and distinct survival outcomes. Read More

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http://dx.doi.org/10.1016/j.beha.2020.101147DOI Listing

Anagrelide in Essential Thrombocythemia (ET): Results from 150 patients over 25 years by the 'Ph1-negative Myeloproliferative Neoplasms Latium Group'.

Eur J Haematol 2020 May 22. Epub 2020 May 22.

Ematologia, Azienda Ospedaliera Universitaria Policlinico Umberto I, Roma, Italia.

Background And Aims: Anagrelide is a drug effective in reducing platelet counts in Essential Thrombocythemia (ET) and Ph1-negative Myeloproliferative Neoplasms. The aim of this study was to evaluate the real-life use of anagrelide in ET patients followed over 25 years at the Haematological Institutes belonging to "Ph1-negative Myeloproliferative Neoplasms Latium Group".

Patients And Methods: Eligibility criteria were: diagnosis of ET and treatment with anagrelide. Read More

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http://dx.doi.org/10.1111/ejh.13454DOI Listing

Myelofibrosis and Portal Hypertension: The Case for Primary Variceal Screening.

ACG Case Rep J 2020 Feb 2;7(2):e00333. Epub 2020 Mar 2.

Division of Gastroenterology, Massachusetts General Hospital and Harvard Medical School, Boston, MA.

Myelofibrosis is a hematologic condition that predisposes to the formation of large and small portal venous clots. Portal injury is believed to underlie the mechanism of development of noncirrhotic portal hypertension in this population. We describe a patient with myelofibrosis, proven portal hypertension, and extramedullary hematopoiesis with no imaging or pathologic evidence of microvascular or macrovascular portal clot. Read More

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http://dx.doi.org/10.14309/crj.0000000000000333DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7209799PMC
February 2020

Ectopic PD-L1 expression in JAK2 (V617F) myeloproliferative neoplasm patients is mediated via increased activation of STAT3 and STAT5.

Hum Cell 2020 May 19. Epub 2020 May 19.

Department of Biochemistry, Maulana Azad Medical College and Associated Hospitals, Bahadur Shah Zafar Marg, New Delhi, 110002, India.

Escalated PD-L1 expression has been identified during malignant transformation in a number of cancer types and helps cancer cells escape an effective anti-tumor immune response. The mechanisms underlying escalated production of PD-L1 in many cancers, however, are still far from clear. We studied PD-L1, STAT3 and STAT5 mRNA expression using qRT-PCR in 72 BCR/ABL1 negative myeloproliferative neoplasm (MPN) patients (39 polycythemia vera and 33 essential thrombocythemia). Read More

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http://dx.doi.org/10.1007/s13577-020-00370-6DOI Listing

Pulmonary hypertension in patients with Philadelphia-negative myeloproliferative neoplasms: a single-center retrospective analysis of 225 patients.

Blood Res 2020 May 20. Epub 2020 May 20.

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

Background: The prevalence of pulmonary hypertension (PH) in myeloproliferative neoplasms (MPNs) varies among studies. We analyzed the prevalence of PH in Korean patients with Philadelphia-negative (Ph⎺) MPNs.

Methods: Medical records of patients with Ph⎺ MPNs [essential thrombocythemia (ET), polycythemia vera (PV), or primary myelofibrosis (PMF)] visiting a single hospital between 1993 and 2019 were reviewed retrospectively. Read More

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http://dx.doi.org/10.5045/br.2020.2020001DOI Listing

Platelet Toll-Like Receptors Mediate Thromboinflammatory Responses in Patients With Essential Thrombocythemia.

Front Immunol 2020 30;11:705. Epub 2020 Apr 30.

Institute of Medical Research A. Lanari, School of Medicine, University of Buenos Aires, Buenos Aires, Argentina.

Essential thrombocythemia (ET) is comprised among chronic myeloproliferative neoplasms (MPN) and is caused by driver mutations in 2, , and , which lead to megakaryocyte proliferation and prominent thrombocytosis. Thrombosis remains the main cause of morbidity in ET and is driven by the interplay between blood cells, the endothelium, the clotting cascade, and host-derived inflammatory mediators. Platelet activation plays a key role in the thrombotic predisposition, although the underlying mechanisms remain poorly defined. Read More

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http://dx.doi.org/10.3389/fimmu.2020.00705DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203216PMC

Essential Thrombocythaemia with Concomitant Waldenström Macroglobulinaemia: Case Report and Literature Review.

Onco Targets Ther 2020 23;13:3431-3435. Epub 2020 Apr 23.

Department of Hematology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang 318000, People's Republic of China.

Essential thrombocythaemia (ET) and Waldenström macroglobulinaemia (WM) are two distinct disorders. Studies have reported several cases of myeloproliferative neoplasms (MPNs) with concomitant plasma cell dyscrasia. However, there were no reported cases of ET with concomitant WM to date. Read More

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http://dx.doi.org/10.2147/OTT.S245950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186880PMC

Acquired Angioedema as the Presenting Feature of a -Positive Essential Thrombocytosis.

JCO Oncol Pract 2020 May 18:JOP1900779. Epub 2020 May 18.

Inova Fairfax Hospital, Falls Church, VA.

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http://dx.doi.org/10.1200/JOP.19.00779DOI Listing

Disease-Specific Derangement of Circulating Endocannabinoids and -Acylethanolamines in Myeloproliferative Neoplasms.

Int J Mol Sci 2020 May 11;21(9). Epub 2020 May 11.

Institute of Hematology "L. e A. Seràgnoli", Department of Experimental, Diagnostic and Specialty Medicine (DIMES), School of Medicine, University of Bologna, 40138 Bologna, Italy.

Growing evidence highlights the endocannabinoid (EC) system involvement in cancer progression. Lipid mediators of this system are secreted by hematopoietic cells, including the ECs 2-arachidonoyl-glycerol (2AG) and arachidonoyl-ethanolamide (AEA), the 2AG metabolite 1AG, and members of N-acylethanolamine (NAE) family-palmitoyl-ethanolamide (PEA) and oleoyl-ethanolamide (OEA). However, the relevance of the EC system in myeloproliferative neoplasms (MPN) was never investigated. Read More

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http://dx.doi.org/10.3390/ijms21093399DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7246996PMC

Thrombocytosis and Thrombosis: Is There Really a Correlation?

Curr Hematol Malig Rep 2020 May 12. Epub 2020 May 12.

Division of Hematology-Oncology, Department of Medicine, Northwestern University Feinberg School of Medicine, 676 N. Saint Clair, Suit 850, Chicago, IL, 60611, USA.

Purpose Of Review: Thrombocytosis is common to all myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis. Despite the traditionally held belief amongst many clinicians that thrombocytosis correlates with thrombosis risk, there is little evidence in the literature to support that claim. Herein we critically analyze the literature to better understand the relationship between thrombocytosis and risk of thrombosis in MPN. Read More

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http://dx.doi.org/10.1007/s11899-020-00588-zDOI Listing

[Cardiac Surgery for Mitral Regurgitation Associated with Essential Thrombocythemia].

Kyobu Geka 2020 May;73(5):353-357

Department of Cardiovascular Surgery, Shizuoka City Shizuoka Hospital, Shizuoka, Japan.

An 80-year-old woman with essential thrombocythemia was diagnosed with severe mitral regurgitation and moderate tricuspid regurgitation. Preoperatively, she had been treated with hydroxycarbamide and low-dose aspirin since her platelet count was high( 96.2×104/μl). Read More

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Loss-of-function polymorphism in reduces risk of V617F somatic mutation and myeloproliferative neoplasm: A Mendelian randomization study.

EClinicalMedicine 2020 Apr 19;21:100280. Epub 2020 Feb 19.

Department of Clinical Biochemistry, Copenhagen University Hospital, Herlev and Gentofte Hospital, Borgmester Ib Juuls Vej 1, DK-2730 Herlev, Denmark.

Background: Whether inflammation is independently associated with development of V617F mutation and myeloproliferative neoplasm is not clear. We tested the hypothesis that a loss-of-function polymorphism in (marked by rs4537545) reduces risk of V617F mutation and myeloproliferative neoplasm in a Mendelian randomization study.

Methods: We genotyped 107,969 Danes from the Copenhagen General Population Study for the rs4537545 genotype, where the T-allele is associated with impaired interleukin-6 receptor signaling and reduced inflammation. Read More

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http://dx.doi.org/10.1016/j.eclinm.2020.100280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7201035PMC

Mutant Calreticulin in the Myeloproliferative Neoplasms.

Hemasphere 2020 Feb 15;4(1):e333. Epub 2020 Jan 15.

Wellcome-MRC Cambridge Stem Cell Institute, Cambridge, United Kingdom and Department of Haematology, University of Cambridge.

Mutations in the gene for calreticulin () were identified in the myeloproliferative neoplasms (MPNs) essential thrombocythaemia (ET) and primary myelofibrosis (MF) in 2013; in combination with previously described mutations in and , driver mutations have now been described for the majority of MPN patients. In subsequent years, researchers have begun to unravel the mechanisms by which mutant CALR drives transformation and to understand their clinical implications. Mutant CALR activates the thrombopoietin receptor (MPL), causing constitutive activation of Janus kinase 2 (JAK2) signaling and cytokine independent growth in vitro. Read More

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http://dx.doi.org/10.1097/HS9.0000000000000333DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000472PMC
February 2020

Essential thrombocythemia manifesting as ophthalmic artery occlusion.

Am J Ophthalmol Case Rep 2020 Jun 23;18:100724. Epub 2020 Apr 23.

Department of Ophthalmology, Vajira Hospital, Navamindradhiraj University, 681 Samsen Street, Dusit Bangkok, 10300, Thailand.

Purpose: To report the first case of a patient with essential thrombocythemia (ET) who presented with unilaterally extensive choroidal ischemia coinciding with central retinal artery occlusion (CRAO).Observations: A 64-year-old man presented with a classic sign of short posterior ciliary artery (SPCA) occlusion, Amalric triangular choroidal infarction, coinciding with CRAO in the left eye. He was later diagnosed as having ET due to a massive platelet count of 1,100,000 cells/mm and confirmed genetic testing. Read More

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http://dx.doi.org/10.1016/j.ajoc.2020.100724DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195516PMC

MERGE: A Multinational, Multicenter Observational Registry for Myeloproliferative Neoplasms in Asia, including Middle East, Turkey, and Algeria.

Cancer Med 2020 Apr 30. Epub 2020 Apr 30.

Sir YK Pao Centre for Cancer & Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong, SAR.

Philadelphia chromosome-negative (Ph-) myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal disorders of the bone marrow, and are associated with a high disease burden, reduced quality of life (QOL), and shortened survival. This multinational, multicenter, non-interventional registry "MERGE" was initiated with an objective to collect data on the epidemiological indices of classical Ph-MPNs, existing treatment patterns, and impact of MPNs on health-related QOL in various countries/regions in Asia, including the Middle East, Turkey, and Algeria. Of the 884 eligible patients with MPNs, 169 had myelofibrosis (MF), 301 had polycythemia vera (PV), 373 had essential thrombocythemia (ET), and 41 had unclassified MPNs. Read More

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http://dx.doi.org/10.1002/cam4.3004DOI Listing

Comparison of starting doses of anagrelide as a first-line therapy in patients with cytoreductive therapy-naïve essential thrombocythemia: difference between starting at 0.5 and 1.0 mg/day.

Int J Hematol 2020 Apr 23. Epub 2020 Apr 23.

First Department of Internal Medicine, Kansai Medical University, 2-5-1, Shinmachi, Hirakata-City, Osaka, 573-1010, Japan.

Anagrelide is widely used for cytoreductive therapy in patients with essential thrombocythemia who are at high risk for thrombosis. The recommended starting dose in the package insert of anagrelide varies by country. A high starting dose leads to an early onset of action, but causes a higher incidence of adverse events. Read More

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http://dx.doi.org/10.1007/s12185-020-02876-zDOI Listing

STAT5 is Expressed in CD34/CD38 Stem Cells and Serves as a Potential Molecular Target in Ph-Negative Myeloproliferative Neoplasms.

Cancers (Basel) 2020 Apr 21;12(4). Epub 2020 Apr 21.

Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria.

Janus kinase 2 (JAK2) and signal transducer and activator of transcription-5 (STAT5) play a key role in the pathogenesis of myeloproliferative neoplasms (MPN). In most patients, V617F or mutations are found and lead to activation of various downstream signaling cascades and molecules, including STAT5. We examined the presence and distribution of phosphorylated (p) STAT5 in neoplastic cells in patients with MPN, including polycythemia vera (PV, = 10), essential thrombocythemia (ET, = 15) and primary myelofibrosis (PMF, = 9), and in the V617F-positive cell lines HEL and SET-2. Read More

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http://dx.doi.org/10.3390/cancers12041021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225958PMC

Healthcare resource utilization in myeloproliferative neoplasms: a population-based study from Ontario, Canada.

Leuk Lymphoma 2020 Apr 23:1-12. Epub 2020 Apr 23.

The Elizabeth and Tony Comper MPN Program, Princess Margaret Cancer Centre, Toronto, Canada.

Health resource utilization (HRU) and associated factors of high cost are not well understood in myeloproliferative neoplasms (MPNs). In this population-based, retrospective matched-cohort study, we used administrative health databases of Ontario, Canada to measure treatment costs and HRU for patients with MPN from 2004 to 2016 and compared them to matched controls. In 7130 patients with MPN [essential thrombocythemia (ET) = 3481; polycythemia vera (PV) = 2618; myelofibrosis (MF) = 1031], the mean annualized treatment costs were $16,646 for ET (controls, $7070); $16,360 for PV (controls, $7293); and $25,863 for MF (controls, $7386). Read More

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http://dx.doi.org/10.1080/10428194.2020.1749607DOI Listing

[Analysis of Essential Thrombocythemia to Acute Myeloid Leukemia].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Apr;28(2):411-417

Department of Hematology, The Second Affiliated Hospital of Fujian Medical University, Quanzhou 362000, Fujian Province, China,E-mail:

Objective: To improve the understanding of the transformation of essential thrombocythemia (ET) into acute myeloid leukemia (AML), and to explore the relationship between JAK2 V617F gene mutation and disease transformation.

Methods: The detection of bone marrow morphology,cytogenetics, JAK2 V617F gene were performed before and after transformation, as well as the immunological tests after transformation was performed in 3 patients with ET into AML. The characteristics, clinical features, diagnosis and treatment of the patients before and after transformation were compared. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.02.009DOI Listing

Interferon Alpha Therapy Increases Pro-Thrombotic Biomarkers in Patients with Myeloproliferative Neoplasms.

Cancers (Basel) 2020 Apr 17;12(4). Epub 2020 Apr 17.

Université de Paris, INSERM UMR_S1148, F-75877 Paris CEDEX 18, France.

Myeloproliferative neoplasms (MPN) are associated with an increased risk of arterial and venous thrombosis. Pegylated-interferon alpha (IFN) and hydroxyurea (HU) are commonly used to treat MPN, but their effect on hemostasis has not yet been studied. The aim of our study was to determine whether IFN and HU impact the biological hemostatic profile of MPN patients by studying markers of endothelial, platelet, and coagulation activation. Read More

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http://dx.doi.org/10.3390/cancers12040992DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7226618PMC

Outcome of Pregnancy in the Era of Pegylated Interferon Alpha 2a in Females with Essential Thrombocythemia: An Experience from Qatar.

Case Rep Oncol 2020 Jan-Apr;13(1):336-340. Epub 2020 Mar 26.

National Center for Cancer Care and Research, Department of Oncology - Hematology and BMT Section -, HMC, Doha, Qatar.

Myeloproliferative neoplasms are a diversified group of diseases of the hematopoietic stem cell, such as essential thrombocythemia (ET) and polycythemia vera. They are mainly caused by mutations in the following genes: and . All carry an increased risk to transform into acute leukemia or chronic myelogenous leukemia along with thrombosis and hemorrhagic complications. Read More

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http://dx.doi.org/10.1159/000506447DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7154247PMC

Serum procalcitonin in Philadelphia-negative myeloproliferative neoplasms.

Wien Klin Wochenschr 2020 Apr 16. Epub 2020 Apr 16.

Division of Hematology, Department of Internal Medicine, University Hospital Center Zagreb, Zagreb, Croatia.

Philadelphia-negative myeloproliferative neoplasms (MPNs), essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF), are rare clonal hematopoietic stem cell disorders accompanied by a strong inflammatory milieu, which is directly responsible for constitutional symptoms associated with the disease, such as fever, weight loss or night sweats. Non-hematologists sometimes (and often wrongly) consider the fever in MPN patients to be a symptom of an underlying disease, which may have devastating consequences. Serum procalcitonin (PCT) is a circulating biomarker commonly used to improve the diagnostic accuracy of bacterial infections and to guide antibiotic therapy. Read More

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http://dx.doi.org/10.1007/s00508-020-01651-8DOI Listing

Thrombophilic Risk of Factor V Leiden, Prothrombin G20210A, MTHFR, and Calreticulin Mutations in Essential Thrombocythemia Egyptian Patients.

Adv Hematol 2020 30;2020:7695129. Epub 2020 Mar 30.

Medical Oncology Unit, Oncology Center, Mansoura University, Faculty of Medicine, Mansoura, Egypt.

Objectives: Essential thrombocythemia (ET) is one of the myeloproliferative neoplasms characterized by a sustained elevation of platelet numbers with a tendency for thrombosis and hemorrhage. The aim of this work is to establish the relation between calreticulin, factor V Leiden, prothrombin G20210A, and MTHFR mutations in ET patients and the thrombotic risk of these patients.

Methods: This study was carried out on 120 ET patients and 40 apparently healthy individuals as a control group. Read More

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http://dx.doi.org/10.1155/2020/7695129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7149341PMC

Thrombotic Risk Detection in Patients with Polycythemia Vera: The Predictive Role of Mutations.

Cancers (Basel) 2020 Apr 10;12(4). Epub 2020 Apr 10.

Hematology Department, Hospital Universitario de Gran Canaria Dr. Negrín, 35019 Las Palmas de Gran Canaria, Spain.

The development of thrombotic events is common among patients with polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). We studied the influence of pathogenic mutations frequently associated with myeloid malignancies on thrombotic events using next-generation sequencing (NGS) in an initial cohort of 68 patients with myeloproliferative neoplasms (MPN). As expected, the presence of mutations in and (DTA genes) was positively associated with age for the whole cohort ( = 0. Read More

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http://dx.doi.org/10.3390/cancers12040934DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7226609PMC

The Essential Thrombocythemia, Thrombotic Risk Stratification, and Cardiovascular Risk Factors.

Adv Hematol 2020 27;2020:9124821. Epub 2020 Mar 27.

Hematology Division University Hospital Policlinico "Paolo Giaccone", Via del Vespro 129, 90127 Palermo, Italy.

Essential thrombocythemia is a rare hematological malignancy with good overall survival, but moderate to high risk of developing arterial or venous thrombosis lifelong. Different thrombotic risk scores for patients with essential thrombocythemia have been proposed, but only one of them (the IPSET-t scoring system) takes into account the classical cardiovascular risk factors as one of the scoring items. Currently, in clinical practice, the presence of cardiovascular risk factors in patients with diagnosis of ET rarely determines the decision to initiate cytoreductive therapies. Read More

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http://dx.doi.org/10.1155/2020/9124821DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139859PMC

A randomized, double-blind trial of three aspirin regimens to optimize antiplatelet therapy in essential thrombocythemia.

Blood 2020 Apr 7. Epub 2020 Apr 7.

Catholic University, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.

Essential thrombocythemia (ET) is characterized by abnormal megakaryopoiesis and enhanced thrombotic risk. Once-daily (od), low-dose aspirin is the recommended antithrombotic regimen, but accelerated platelet generation may reduce the duration of platelet cyclooxygenase (COX)-1 inhibition. We performed a multicenter, double-blind trial to investigate the efficacy of three aspirin regimens in optimizing platelet COX-1 inhibition while preserving COX-2-dependent vascular thromboresistance. Read More

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http://dx.doi.org/10.1182/blood.2019004596DOI Listing

The development of T-cell malignancies in patients with pre-existing myeloproliferative neoplasms: a report of three cases.

Ecancermedicalscience 2020 17;14:1011. Epub 2020 Feb 17.

Houston Methodist Cancer Center, 6445 Main Street, Outpatient Center, 24th Floor, Houston, TX 77030, USA.

Secondary acute myeloid leukaemia complicating the natural disease course of pre-existing Philadelphia chromosome-negative myeloproliferative neoplasms (PN-MPN) is well documented and associated with treatment challenges and significant morbidity. The incidence of a T-cell malignancy developing in patients with pre-existing PN-MPN is uncommon, with one case documented in the literature. We present two cases of angioimmunoblastic T-cell lymphoma (AITL) and one case of T-cell acute lymphoblastic leukaemia (T-ALL) that developed in patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF), respectively. Read More

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http://dx.doi.org/10.3332/ecancer.2020.1011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7105335PMC
February 2020

Myeloid neoplasm with isolated del(5q) and the MPLW515L mutation fulfills the WHO diagnostic criteria for ET.

Authors:
Keita Kirito

Int J Hematol 2020 Apr 3. Epub 2020 Apr 3.

Department of Hematology and Oncology, University of Yamanashi, 1110, Shimokato, Chuo, Yamanashi-ken, 409-3898, Japan.

A 70-year-old male was referred to our hospital for marked thrombocytosis without anemia. The patient simultaneously presented with an MPL W515L mutation, one of the major driver mutations in essential thrombocythemia (ET), and deletion of 5q, a characteristic cytogenetic abnormality in myelodysplastic syndrome (MDS). Bone marrow examination showed a combination of both mature hyperlobulated megakaryocytes, as found in ET, and small hypolobulated megakaryocytes, typically found in MDS with del(5q). Read More

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http://dx.doi.org/10.1007/s12185-020-02872-3DOI Listing

Use of rotational thromboelastometry for a global screening of coagulation profile in patients of myeloproliferative neoplasms.

Platelets 2020 Apr 3:1-4. Epub 2020 Apr 3.

Department of Hematology, Eskisehir Osmangazi University Medical School, Eskisehir, Turkey.

Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Read More

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http://dx.doi.org/10.1080/09537104.2020.1742309DOI Listing

DETERMINING THE RISK OF THROMBOSIS AMONG THE PATIENTS WITH ESSENTIAL THROMBOCYTHEMIA.

Georgian Med News 2020 Feb(299):143-146

Institute of Hematology and Transfusiology, Tbilisi, Georgia.

The aim of the studywas to determine the risk of thrombosis among patients with Essential thrombocythemia based on the modern criteria of diagnosis and to reveal the treatment accordingmethods of research in Georgia. We analyzed clinical manifestations of 25 cases of Essential thrombocythemia diagnosed in 2013-2017 y. at the Institute of Hematology and Transfusiology. Read More

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February 2020

Calreticulin haploinsufficiency augments stem cell activity and is required for onset of myeloproliferative neoplasms.

Blood 2020 Mar 27. Epub 2020 Mar 27.

University of Miyazaki, Kiyotake, Japan.

Mutations in JAK2, MPL, or CALR are detected in more than 80% of myeloproliferative neoplasm (MPN) patients and are thought to play a driver role in MPN pathogenesis via autosomal activation of the JAK-STAT signaling cascade. Mutant CALR binds to MPL, activates downstream MPL signaling cascades, and induces essential thrombocythemia in mice. However, embryonic lethality of Calr-deficient mice precludes determination of a role for CALR in hematopoiesis. Read More

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http://dx.doi.org/10.1182/blood.2019003358DOI Listing

Increased von Willebrand factor levels in polycythemia vera and phenotypic differences with essential thrombocythemia.

Res Pract Thromb Haemost 2020 Mar 28;4(3):413-421. Epub 2020 Feb 28.

Servizio Malattie Emorragiche e Trombotiche Fondazione Policlinico Universitario "A. Gemelli" IRCCS Roma Italy.

Background: Acquired von Willebrand factor (VWF) deficiency was described in Philadelphia-negative myeloproliferative neoplasms, especially in essential thrombocythemia (ET). VWF phenotype in contemporary patients with polycythemia vera (PV) remains less explored.

Objectives: To characterize the VWF phenotype in PV and to compare VWF phenotype in PV with matched healthy subjects and ET patients. Read More

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http://dx.doi.org/10.1002/rth2.12315DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086469PMC

Thrombotic, inflammatory, and HIF-regulated genes and thrombosis risk in polycythemia vera and essential thrombocythemia.

Blood Adv 2020 Mar;4(6):1115-1130

Division of Hematology, School of Medicine, University of Utah, Salt Lake City, UT.

Thrombosis is a major cause of morbidity and mortality in polycythemia vera (PV) and essential thrombocythemia (ET). The pathophysiology of thrombosis in these disorders remains unclear, and we hypothesized that upregulation of thrombotic, inflammatory, and hypoxia-inducible factor (HIF)-regulated genes may play a role in it. We performed unbiased RNA sequencing in granulocytes and platelets of PV patients and found differential expression of several thrombotic, inflammatory, and HIF-regulated genes. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019001379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7094018PMC

Comparison of clinical presentation and out-comes of Chikungunya and Dengue virus infections in patients with acute undifferentiated febrile illness from the Sindh region of Pakistan.

PLoS Negl Trop Dis 2020 03 23;14(3):e0008086. Epub 2020 Mar 23.

Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, Pakistan.

Background: Arboviruses are a cause of acute febrile illness and outbreaks worldwide. Recent outbreaks of Chikungunya virus (CHIKV) in dengue endemic areas have alarmed clinicians as unique clinical features differentiating CHIKV from Dengue virus (DENV) are limited. This has complicated diagnostic efforts especially in resource limited countries where lab testing is not easily available. Read More

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http://dx.doi.org/10.1371/journal.pntd.0008086DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141694PMC

Targeted Sequencing Improves DIPSS-Plus Prognostic Scoring in Myelofibrosis Patients Undergoing Allogeneic Transplantation.

Biol Blood Marrow Transplant 2020 Mar 16. Epub 2020 Mar 16.

Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, Washington; Department of Medicine, University of Washington, Seattle, Washington; Seattle Cancer Care Alliance, Seattle, Washington; Department of Pathology, University of Washington, Seattle, Washington. Electronic address:

Primary myelofibrosis (MF) and secondary MF developing after polycythemia vera or essential thrombocythemia are clonal disorders of hematopoiesis. Currently the sole therapy offering the potential of cure is hematopoietic cell transplantation (HCT). Several risk classification systems including clinical, hematologic, and mutational parameters have been proposed. Read More

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http://dx.doi.org/10.1016/j.bbmt.2020.03.007DOI Listing

Essential Thrombocythemia. Reply.

N Engl J Med 2020 03;382(12):e21

Mayo Clinic, Rochester, MN

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http://dx.doi.org/10.1056/NEJMc1917586DOI Listing

Essential Thrombocythemia.

N Engl J Med 2020 03;382(12):e21

Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

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http://dx.doi.org/10.1056/NEJMc1917586DOI Listing

Essential Thrombocythemia.

N Engl J Med 2020 03;382(12):e21

University of Kentucky College of Medicine, Lexington, KY

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http://dx.doi.org/10.1056/NEJMc1917586DOI Listing

Association of mast cells and bone marrow reticulin fibrosis in patients with bcr-abl negative chronic myeloproliferative neoplasms.

Authors:
Hakan Keski

Blood Cells Mol Dis 2020 05 5;82:102420. Epub 2020 Mar 5.

Department of Internal Medicine, Division of Hematology, Kocaeli University, Kocaeli, Turkey. Electronic address:

Background: We aimed to investigate the association of bone marrow mast cell numbers (MCN) and the degree of reticulin fibrosis in patients with chronic myeloproliferative neoplasms (MPN).

Methods: This was a case-control study that recruited 47 patients who were diagnosed with bcr-abl negative MPN. Thirty patients with lymphoma served as controls. Read More

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http://dx.doi.org/10.1016/j.bcmd.2020.102420DOI Listing