4,622 results match your criteria Thrombocytosis Essential


F-FLT PET/MRI for bone marrow failure syndrome-initial experience.

EJNMMI Res 2019 Feb 15;9(1):16. Epub 2019 Feb 15.

Biomedical Imaging Research Center, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji-cho, Fukui, 910-1193, Japan.

Background: Bone marrow failure syndrome (BMFS) is a heterogeneous group of disorders associated with single- or multiple-lineage cytopenia and failure of normal hematopoiesis. We assessed the feasibility of integrated PET/MRI with 3'-deoxy-3'-F-fluorothymidine (F-FLT) to assess the pathophysiology of whole-body bone marrow for the diagnosis and monitoring of BMFS. Twenty-five consecutive patients with BMFS underwent a pre-treatment F-FLT PET/MRI scan. Read More

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http://dx.doi.org/10.1186/s13550-019-0490-0DOI Listing
February 2019

The marrow stem cell niche in normal and malignant hematopoiesis.

Ann N Y Acad Sci 2019 Feb 14. Epub 2019 Feb 14.

Stony Brook University School of Medicine, Stony Brook, New York.

The hematopoietic niche is composed of endothelial cells, mesenchymal stromal cells of several types, and megakaryocytes, and functions to support the survival, proliferation, and differentiation of normal hematopoietic stem cells (HSCs). An abundance of evidence from a range of hematological malignancies supports the concept that the niche also participates in the pathogenesis of malignant hematopoiesis, differentially supporting malignant stem or progenitor cells over that of normal blood cell development. In 2005, patients with myeloproliferative neoplasms were reported to harbor an acquired, activating, missense V617F mutation of the cytokine-signaling Janus kinase (JAK)-2, JAK2 , present in virtually all patients with polycythemia vera and half of patients with essential thrombocythemia and primary myelofibrosis. Read More

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http://dx.doi.org/10.1111/nyas.14028DOI Listing
February 2019

The role of hepcidin, GDF15, and mitoferrin-1 in iron metabolism of polycythemia vera and essential thrombocytosis patients

Turk J Med Sci 2019 Feb 11;49(1):74-80. Epub 2019 Feb 11.

Background/aim: GDF15, hepcidin and mitoferrin-1 (mfrn-1) are proteins involved in systemic iron regulation. There are no studies in the literature demonstrating the serum mfrn-1 levels in polycythemia vera (PV) and essential thrombocythemia (ET) patients. The aim of this study was to investigate GDF15, hepcidin and mfrn-1 levels in PV and ET patients. Read More

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http://dx.doi.org/10.3906/sag-1803-13DOI Listing
February 2019

[Clinical Analysis of 208 Patiets with BCR/ABL Negative Myeloproliferative Neoplasms].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):159-164

Department of Hematology, the Second Affiliated Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.E-mail:

Objective: To analyze the incidence, hemogram, genetics, clinical manifestations, therapeutic efficacy and outcome of patients with myeloproliferative neoplasms(MPN) so as to provide much more therapeutic basis for clinically studying the pathogenesis, diagnosis, and treatment as well as evaluating the prognosis of MPN patients.

Methods: The clinical data and related laboratory test results in 208 cases of BCR/ABL fusion gene regative MPN were collected and analyzed retrospectively.

Results: The MPN could occur at any age, but the highest incidence was observed in patients aged 40-79. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.025DOI Listing
February 2019
2 Reads

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN with RS-T) complicated by hyperleukocytosis and gene analysis in relation to leukocytosis.

J Clin Exp Hematop 2019 Feb 7. Epub 2019 Feb 7.

Myelodysplastic/myeloproliferative neoplasm (MDS/MPN) with ring sideroblasts and thrombocytosis (MDS/MPN with RS-T), which exhibits both an increased number of marrow ring sideroblasts and thrombocytosis, is a rare disorder classified as one of the newly established forms of MDS/MPN in the WHO 2016 classification. A 77-year-old female with marked thrombocytosis of 1,024×10/L was tentatively diagnosed with essential thrombocythemia in 2011, and the thrombocytosis was controlled using hydroxycarbamide and low-dose busulfan. In 2016, the leukocyte count increased to a peak value of 68. Read More

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http://dx.doi.org/10.3960/jslrt.18037DOI Listing
February 2019
1 Read

Discovery and evaluation of ZT55, a novel highly-selective tyrosine kinase inhibitor of JAK2 against myeloproliferative neoplasms.

J Exp Clin Cancer Res 2019 Feb 4;38(1):49. Epub 2019 Feb 4.

State Key Laboratory of Bioactive Substance and Function of Natural Medicines, Institute of Materia Medica, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100050, China.

Background: The JAK2-STAT signaling pathway plays a critical role in myeloproliferative neoplasms (MPN). An activating mutation in JAK2 (V617F) is present in ~ 95% of polycythemia vera, essential thrombocythemia, and primary myelofibrosis cases. This study aims to explore the selective JAK2 inhibitor, evaluate the efficacy and possible mechanism of ZT55 on MPN. Read More

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http://dx.doi.org/10.1186/s13046-019-1062-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360668PMC
February 2019
2 Reads

Radiological features and outcomes of essential thrombocythemia-related stroke.

J Neurol Sci 2019 Jan 22;398:135-137. Epub 2019 Jan 22.

Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Republic of Korea.

Background: Essential thrombocythemia (ET) is known to be associated with an increased vascular event, but the stroke patterns and prognosis have not been studied.

Methods: Between January 2013 and December 2017, acute ischemic stroke patients with ET who were admitted to two tertiary hospital stroke centers in Seoul, Korea were included. We retrospectively reviewed their clinical, laboratory and imaging data. Read More

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http://dx.doi.org/10.1016/j.jns.2019.01.035DOI Listing
January 2019
1 Read

[Risk factors for recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):17-23

Department of Hematology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

To analyze the clinical characteristics of recurrent thrombosis in patients with polycythemia vera (PV) and essential thrombocythemia (ET) to probe the risk factors for recurrent thrombosis in patients with ET and PV. The clinical data of 104 ET and PV patients with thrombosis in Beijing Anzhen Hospital from February 2001 to November 2016 were retrospectively analyzed. Thrombosis reoccurred in 38 patients. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.004DOI Listing
January 2019
2 Reads

Acute ischemic stroke in the setting of essential thrombocytemia (clinical cases).

Ter Arkh 2018 Aug;90(7):102-104

National Research Center of Hematology, Moscow, Russia.

This article describes several clinical cases of acute ischemic stroke among patients suffering from essential thrombocytemia. Ambiguity of etiological factors of stroke is demonstrated among patients with this pathology. Thrombocytosis and high allele load in the Jak2 gene play an important role (even with normal platelet count) in progression of cerebrovascular disease. Read More

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http://dx.doi.org/10.26442/terarkh2018907102-104DOI Listing
August 2018
1 Read

Cepeginterferon alfa-2b in the treatment of chronic myeloproliferative diseases.

Ter Arkh 2018 Aug;90(7):23-29

National Research Center for hematology, Moscow, Russia.

Aim: A comparative evaluation of the effectiveness of different therapeutic strategies in patients with polycythemia vera (PV) and essential thrombocythemia (ET).

Materials And Methods: Patients with PV or ET, diagnosed according to the criteria WHO 2016 were included in the study. The primary endpoint - 6 months of therapy (clinical-hematological and molecular responses). Read More

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http://dx.doi.org/10.26442/terarkh201890723-29DOI Listing
August 2018
7 Reads

Relationship between symptom burden and disability leave among patients with myeloproliferative neoplasms (MPNs): findings from the Living with MPN patient survey.

Ann Hematol 2019 Jan 29. Epub 2019 Jan 29.

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

Patients with myeloproliferative neoplasms (MPNs) experience burdensome symptoms that negatively affect their quality of life. How MPN symptoms relate with medical disability leave (MDL) among patients with the disease has not been previously examined. Using data collected from the Living with MPNs patient survey, symptom burden and functional status were compared in patients who reported taking MDL due to their MPN versus patients who reported no changes in employment status. Read More

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http://dx.doi.org/10.1007/s00277-019-03610-4DOI Listing
January 2019
1 Read

Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age, a systematic review.

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Department of Hematology, Guy and St Thomas NHS Trust, London, United Kingdom.

Although it is well known that myeloproliferative neoplasms occur in younger patients, few large cohorts of such patients have been reported. Thus, our knowledge is limited especially for children and young adults about circumstances of diagnosis, outcome and treatment. We performed a systematic review of cases published since 2005, concerning patients aged below 20-years at the time of diagnosis of essential thrombocythemia or polycythemia vera. Read More

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http://dx.doi.org/10.3324/haematol.2018.200832DOI Listing
January 2019
1 Read

[Differential Diagnosis of Erythrocytosis - Background and Clinical Relevance].

Dtsch Med Wochenschr 2019 Jan 23;144(2):128-135. Epub 2019 Jan 23.

Due to its rare incidence, erythrocytosis frequently represents a challenge for the treating doctors. The erythropoiesis (= production of erythrocytes) is located in the bone marrow, and the hormone erythropoietin (EPO) takes control in its regulation. Therefore, measurement of EPO in serum is one of the main diagnostic steps. Read More

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http://dx.doi.org/10.1055/a-0739-8340DOI Listing
January 2019
4 Reads

Detection of Mutations Using High Resolution Melting Curve Analysis (HRM-A); Application on a Large Cohort of Greek ET and MF Patients.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019009. Epub 2019 Jan 1.

Haematology Research Laboratory, Biomedical Research Foundation, Academy of Athens, Athens, Greece.

Background And Objectives: Somatic mutations in the calreticulin gene () are detected in approximately 70% of patients with essential thrombocythemia (ET) and primary or secondary myelofibrosis (MF), lacking the and mutations. To determine the prevalence of frameshift mutations in a population of MPN patients of Greek origin, we developed a rapid low-budget PCR-based assay and screened samples from 5 tertiary Haematology units. This is a first of its kind report of the Greek patient population that also disclosed novel mutants. Read More

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http://dx.doi.org/10.4084/MJHID.2019.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328041PMC
January 2019
1 Read

A Rare Case of Triple-Negative Essential Thrombocythemia in a Young Postsplenectomy Patient: A Diagnostic Challenge.

Case Rep Hematol 2018 16;2018:9079462. Epub 2018 Dec 16.

Division of Hematology, University of Texas Health Science Center at Houston, Houston, TX, USA.

The distinction between primary and reactive thrombocytosis by bone marrow histology is very important. Reactive thrombocytosis, the most common cause of thrombocytosis, can be expected in postsplenectomy states; however, close hematological evaluation of prolonged thrombocytosis is essential to identify patients who may have an underlying myeloproliferative neoplasm. We report a 37-year-old woman who was found to have portal, mesenteric, and splenic vein thrombosis with thrombocytosis, two months after she had a splenectomy for spontaneous splenic rupture. Read More

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http://dx.doi.org/10.1155/2018/9079462DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311807PMC
December 2018
2 Reads

The S505A thrombopoietin receptor mutation in childhood hereditary thrombocytosis and essential thrombocythemia is S505N: single letter amino acid code matters.

Leukemia 2019 Feb 11;33(2):563-564. Epub 2019 Jan 11.

Université catholique de Louvain, de Duve Institute and WELBIO, Brussels, Belgium.

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http://dx.doi.org/10.1038/s41375-018-0356-xDOI Listing
February 2019
1 Read

Endovascular Treatment of Cerebral Venous Sinus Thrombosis and Insights into Intracranial Coagulopathy.

J Stroke Cerebrovasc Dis 2019 Jan 8. Epub 2019 Jan 8.

Columbia University, Vagelos College of Physicians & Surgeons, New York, New York.

Cerebral venous sinus thrombosis (CVST) requires anticoagulation to promote vessel recanalization. Current anticoagulation paradigms utilize plasma tests from peripheral venous/arterial samples for therapeutic monitoring. We describe a medically-refractory case of CVST in a 35-year-old woman later found to have JAK2 mutation and essential thrombocytosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10523057183071
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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2018.12.015DOI Listing
January 2019
9 Reads

Plastic-based acoustofluidic devices for high-throughput, biocompatible platelet separation.

Lab Chip 2019 Jan;19(3):394-402

Department of Mechanical Engineering and Material Science, Duke University, Durham, NC 27707, USA.

Platelet separation is a crucial step for both blood donation and treatment of essential thrombocytosis. Here we present an acoustofluidic device that is capable of performing high-throughput, biocompatible platelet separation using sound waves. The device is entirely made of plastic material, which renders the device disposable and more suitable for clinical use. Read More

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http://xlink.rsc.org/?DOI=C8LC00527C
Publisher Site
http://dx.doi.org/10.1039/c8lc00527cDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366625PMC
January 2019
5 Reads

Recurrent ischaemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms.

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

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

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

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

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

In-stent Massive Thrombi Formation During Primary Percutaneous Coronary Intervention in a Patient with Acute Myocardial Infarction Complicated with Essential Thrombocythemia.

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Cardiology, Japanese Red Cross Kyoto Daiichi Hospital, Japan.

A 61-year-old man with essential thrombocythemia (ET) presented with acute myocardial infarction (AMI) and underwent primary percutaneous coronary intervention. After stent deployment from the left main (LM) to the left anterior descending artery, intravascular ultrasound revealed thrombi formation in the whole stent. Two days later, optical frequency domain imaging confirmed stent malapposition and thrombi remaining in only the LM. Read More

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http://dx.doi.org/10.2169/internalmedicine.2083-18DOI Listing
January 2019
1 Read

A Bone Marrow Biopsy is Mandatory for a Diagnosis of Essential Thrombocythemia.

Authors:
Zen Kobayashi

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology, JA Toride Medical Center, Japan.

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http://dx.doi.org/10.2169/internalmedicine.1252-18DOI Listing
January 2019

The authors' reply to "Bone marrow biopsy is mandatory for a diagnosis of essential thrombocythemia".

Intern Med 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology and Cerebrovascular Medicine, Saitama Medical University International Medical Center, Japan.

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http://dx.doi.org/10.2169/internalmedicine.1406-18DOI Listing
January 2019

[Takotsubo cardiomyopathy developing during anagrelide therapy in a patient with essential thrombocythemia].

Rinsho Ketsueki 2018 ;59(12):2606-2608

Department of Internal Medicine and General Medicine, Japanese Red Cross Kitami Hospital.

A 76-year-old woman taking anagrelide (ANA) for essential thrombocythemia (ET) was rushed to the hospital by ambulance because of severe chest pain. Echocardiography revealed apical akinesis with basal and mid-hyperkinesis, and a coronary angiography revealed no significant stenosis in the dominant coronary artery. The patient was diagnosed with takotsubo cardiomyopathy (Tako). Read More

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http://dx.doi.org/10.11406/rinketsu.59.2606DOI Listing
January 2018
1 Read

Therapeutic thrombocytapheresis for extreme thrombocytosis after chemotherapy in essential thrombocytosis.

J Clin Apher 2019 Jan 9. Epub 2019 Jan 9.

Apheresis Unit, Department of Hemotherapy and Hemostasis, Hematology and Oncology Institute, IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.

Essential thrombocytosis (ET) is a chronic myeloproliferative neoplasm characterized by the presence of thrombocytosis and it can be complicated by thrombotic and/or hemorrhagic events. Treatment options include low-dose aspirin and cytoreductive agents such as hydroxyurea. In cases of extreme thrombocytosis, therapeutic thrombocytapheresis can be a useful procedure. Read More

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http://dx.doi.org/10.1002/jca.21683DOI Listing
January 2019
5 Reads

Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis: "2019 Update on Diagnosis, Risk-stratification, and Management".

Am J Hematol 2019 Jan 7. Epub 2019 Jan 7.

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thrombocytosis (RARS-T); now called myelodysplastic/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T).

Diagnosis: MDS-RS is a lower-risk MDS, with single or multilineage dysplasia (SLD/MLD), <5% bone marrow (BM) blasts and ≥ 15% BM RS (≥5% in the presence of SF3B1 mutations). Read More

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http://doi.wiley.com/10.1002/ajh.25397
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http://dx.doi.org/10.1002/ajh.25397DOI Listing
January 2019
19 Reads

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

Blood Cells Mol Dis 2019 Mar 20;75:35-40. Epub 2018 Dec 20.

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

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

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http://dx.doi.org/10.1016/j.bcmd.2018.12.004DOI Listing
March 2019
3 Reads

Calreticulin mutation specific CAL2 immunohistochemistry accurately identifies rare calreticulin mutations in myeloproliferative neoplasms.

Pathology 2018 Dec 31. Epub 2018 Dec 31.

MTA-SE Momentum Molecular Oncohematology Research Group, Semmelweis University, 1st Department of Pathology and Experimental Cancer Research, Budapest, Hungary. Electronic address:

Mutations of the multifunctional protein calreticulin (CALR) are recognised as one of the main driver alterations involved in the pathogenesis of Philadelphia negative myeloproliferative neoplasms (Ph MPN) and also represent a major diagnostic criterion in the most recent World Health Organization classification of myeloid neoplasms. Nowadays, quantitative assessment of the driver mutations is gaining importance, as recent studies demonstrated the clinical relevance of the mutation load reflecting the size of the mutant clone. Here, we performed for the first time a manual and automated quantitative assessment of the CALR mutation load at protein level using CAL2, a recently developed CALR mutation specific monoclonal antibody, on a cohort of 117 patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF) and compared the CALR protein mutation loads with the CALR mutation load values established by a molecular assay. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00313025183038
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http://dx.doi.org/10.1016/j.pathol.2018.11.007DOI Listing
December 2018
3 Reads

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

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

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

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

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http://dx.doi.org/10.12659/AJCR.911854DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325661PMC
January 2019
1 Read

Mutation patterns in essential thrombocythemia, polycythemia vera and secondary myelofibrosis.

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

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

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http://dx.doi.org/10.1080/10428194.2018.1522437DOI Listing
January 2019
1 Read

Clinicopathological and molecular features of SF3B1-mutated myeloproliferative neoplasms.

Hum Pathol 2018 Dec 27. Epub 2018 Dec 27.

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

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

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

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

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

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

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

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

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

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

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

Ultrastruct Pathol 2018 Nov-Dec;42(6):498-507. Epub 2018 Dec 24.

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

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

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http://dx.doi.org/10.1080/01913123.2018.1558323DOI Listing
December 2018
4 Reads
1.133 Impact Factor

Genetic studies in the evaluation of myeloproliferative neoplasms.

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

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

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

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

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

Cell Biol Int 2019 Feb 7;43(2):192-206. Epub 2019 Jan 7.

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

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

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http://dx.doi.org/10.1002/cbin.11084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347554PMC
February 2019
3 Reads
1.933 Impact Factor

The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia.

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

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

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

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

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

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

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

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

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

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http://dx.doi.org/10.1182/bloodadvances.2018024018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306879PMC
December 2018
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Safety and efficacy of combined ruxolitinib and decitabine in accelerated and blast-phase myeloproliferative neoplasms.

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

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

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

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

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

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

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

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

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http://dx.doi.org/10.1186/s12967-018-1729-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296062PMC
December 2018
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Do All Patients With Polycythemia Vera or Essential Thrombocythemia Need Cytoreduction?

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

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

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

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

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

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

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

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

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.013DOI Listing
October 2018
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Somatic Mutations in Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.

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

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

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

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http://dx.doi.org/10.1053/j.seminhematol.2018.04.005DOI Listing
October 2018
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Does hydroxycarbamide therapy really induce leukemic transformation in patients with essential thrombocythemia?

Leuk Res 2019 Jan 22;76:94-95. Epub 2018 Nov 22.

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

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http://dx.doi.org/10.1016/j.leukres.2018.11.011DOI Listing
January 2019

Increased risk of heparin induced thrombocytopenia and thrombosis in patients with essential thrombocythemia carrying the homozygous JAK2 V617F mutation.

J Thromb Thrombolysis 2019 Jan;47(1):155-156

Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.

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http://dx.doi.org/10.1007/s11239-018-1773-4DOI Listing
January 2019
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Singular case of osteolytic lesions revealing transformation of myeloproliferative syndrome to acute leukemia.

Joint Bone Spine 2018 Nov 22. Epub 2018 Nov 22.

Service de rhumatologie, CHU d'Angers, 4, rue Larrey, 49933 Angers cedex 9, France; Groupe d'Eudes Remodelage Osseux et bioMatériaux LHEA UPRES EA 4658, CHU d'Angers, 4, rue Larrey, 49933 Angers cedex 9, France. Electronic address:

Hematological malignancies can cause bone lesions, of which the most common are the punched-out foci of osteolysis seen in multiple myeloma. However, osteosclerotic lesions are more common in myeloproliferative disorders. We report the unusual case of a patient with myeloproliferative syndrome in whom the development of osteolytic lesions revealed transformation to acute leukemia. Read More

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http://dx.doi.org/10.1016/j.jbspin.2018.11.004DOI Listing
November 2018
13 Reads

MPL-mutated essential thrombocythemia: a morphologic reappraisal.

Blood Cancer J 2018 Nov 20;8(12):121. Epub 2018 Nov 20.

Division of Hematology, Departments of Internal and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.

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http://dx.doi.org/10.1038/s41408-018-0159-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246562PMC
November 2018
2 Reads

Splenic mass in a case of -mutated essential thrombocythemia.

Clin Case Rep 2018 Nov 9;6(11):2291-2292. Epub 2018 Oct 9.

Division of Pathology Uji-Tokushukai Medical Center Uji Kyoto Japan.

We report here an intrasplenic large mass in an elderly case of essential thrombocythemia (ET)-myelofibrosis. Laparoscopic splenectomy revealed extramedullary hematopoiesis (EMH) and a type 1 gene mutation (CALR-c.1092_1143del52) in the splenic mass. Read More

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http://dx.doi.org/10.1002/ccr3.1857DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230599PMC
November 2018
1 Read

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

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

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

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

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http://dx.doi.org/10.3233/CBM-171145DOI Listing
January 2018
3 Reads

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

Eur J Haematol 2019 Mar 10;102(3):227-234. Epub 2019 Jan 10.

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

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

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

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http://doi.wiley.com/10.1111/ejh.13197
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http://dx.doi.org/10.1111/ejh.13197DOI Listing
March 2019
11 Reads