7,806 results match your criteria Polycythemia Vera


Properties of FDA-approved small molecule protein kinase inhibitors.

Authors:
Robert Roskoski

Pharmacol Res 2019 Mar 12. Epub 2019 Mar 12.

Blue Ridge Institute for Medical Research, 3754 Brevard Road, Suite 116, Box 19, Horse Shoe, North Carolina 28742-8814, United States. Electronic address:

Because mutations, overexpression, and dysregulation of protein kinases play essential roles in the pathogenesis of many illnesses, this enzyme family has become one of the most important drug targets in the past 20 years. The US FDA has approved 48 small molecule protein kinase inhibitors, nearly all of which are orally effective with the exceptions of netarsudil (which is given as an eye drop) and temsirolimus (which is given intravenously). Of the 48 approved drugs, the majority (25) target receptor protein-tyrosine kinases, ten target non-receptor protein-tyrosine kinases, and 13 target protein-serine/threonine protein kinases. Read More

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http://dx.doi.org/10.1016/j.phrs.2019.03.006DOI Listing

Re-evaluation of hematocrit as a determinant of thrombotic risk in erythrocytosis.

Haematologica 2019 Mar 14. Epub 2019 Mar 14.

Hematology Division, University of Utah

Here we critically evaluate the role of elevated hematocrit as the principal determinant of thrombotic risk in polycythemia and erythrocytosis, defined by an expansion of red cell mass. Since red cell volume determination is no longer readily available, in clinical practice polycythemia and erythrocytosis are defined by elevated hemoglobin and hematocrit. Thrombosis is common in Chuvash erythrocytosis and polycythemia vera. Read More

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http://dx.doi.org/10.3324/haematol.2018.210732DOI Listing

Thromboembolic events in polycythemia vera.

Ann Hematol 2019 Mar 8. Epub 2019 Mar 8.

Hospital del Mar-IMIM, Passeig Marítim 25-29, 08003, Barcelona, Spain.

Thromboembolic events and cardiovascular disease are the most prevalent complications in patients with polycythemia vera (PV) compared with other myeloproliferative disorders and are the major cause of morbidity and mortality in this population. Moreover, a vascular complication such as arterial or venous thrombosis often leads to the diagnosis of PV. The highest rates of thrombosis typically occur shortly before or at diagnosis and decrease over time, probably due to the effects of treatment. Read More

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http://dx.doi.org/10.1007/s00277-019-03625-xDOI Listing
March 2019
2 Reads

Diagnosing or ruling out polycythemia vera in patients with erythrocytosis.

Authors:
Josef T Prchal

Clin Adv Hematol Oncol 2019 Jan;17(1):24-27

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

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January 2019
1 Read

Milestones in the discovery of Budd-Chiari syndrome.

Liver Int 2019 Mar 6. Epub 2019 Mar 6.

Multivisceral Transplant Unit, Gastroenterology, Department of Surgery, Oncology and Gastroenterology, Padua University Hospital, Padua, Italy.

In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty-three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Read More

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http://dx.doi.org/10.1111/liv.14088DOI Listing
March 2019
2 Reads

The role of F-FDG PET in the assessment of a benign hematological disorder: polycythemia.

Hell J Nucl Med 2019 Mar 5. Epub 2019 Mar 5.

Department of Radiology, Perelman School of Medicine, University of Pennsylvania, PA, USA.

Fluorine-18 fluorodeoxyglucose positron emission tomography (F-FDG PET) imaging was conceived in the early 1970 by investigators at the University of Pennsylvania as a research technique to measure brain metabolism and function by employing a non-invasive imaging approach. Soon after the introduction of whole-body PET instruments, F-FDG was utilized in the assessment of a variety of solid tumors and certain hematological malignancies. Yet, the role of F-FDG in assessing benign and uncommon malignant disorders of the bone marrow has not been investigated to a great extent. Read More

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http://dx.doi.org/10.1967/s002449910951DOI Listing
March 2019
1 Read

Irreversible chorea as the initial presentation of polycythemia rubra vera in an elderly woman.

Neurosciences (Riyadh) 2019 Jan;24(1):61-62

Department of Neurology, Royal Cornwall Hospital NHS Trust, Truro, United Kingdom. E-mail:

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http://dx.doi.org/10.17712/nsj.2019.1.20180301DOI Listing
January 2019
1 Read

, a new BMP signaling inhibitor, regulates embryonic hematopoiesis in the zebrafish.

Development 2019 Mar 5. Epub 2019 Mar 5.

University of Geneva, School of medicine, Department of Pathology and Immunology, CMU, University of Geneva, Switzerland

Hematopoiesis maintains the entire blood system and dysregulation of this process can lead to malignancies (leukemia), immunodeficiencies or red blood cell diseases (anemia, polycythemia vera). We took advantage of the zebrafish model that shares most of the genetic program involved in hematopoiesis with mammals to characterize a new gene of unknown function, expressed in the erythroid lineage during primitive, definitive and adult hematopoiesis. This gene, required during primitive and definitive erythropoiesis, encodes a C2H2 zinc finger protein that inhibits BMP signaling. Read More

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http://dx.doi.org/10.1242/dev.164103DOI Listing
March 2019
3 Reads

3023 Mayo Clinic Patients With Myeloproliferative Neoplasms: Risk-Stratified Comparison of Survival and Outcomes Data Among Disease Subgroups.

Mayo Clin Proc 2019 Feb 26. Epub 2019 Feb 26.

Division of Hematology, Department of Internal and Laboratory Medicine, Mayo Clinic, Rochester, MN. Electronic address:

Objective: To document the Mayo Clinic decades-long experience with myeloproliferative neoplasms (MPNs) and provide mature risk-stratified survival data and disease complication estimates.

Patients And Methods: All Mayo Clinic patients with World Health Organization-defined MPNs constituted the core study group and included those with polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).

Results: A total of 3023 consecutive patients (median age, 62 years; range, 18-96 years) were considered: 665 PV, 1076 ET, and 1282 PMF. Read More

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http://dx.doi.org/10.1016/j.mayocp.2018.08.022DOI Listing
February 2019
1 Read

A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms.

BMC Cancer 2019 Feb 28;19(1):184. Epub 2019 Feb 28.

Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok, 10700, Thailand.

Background: Philadelphia (Ph) chromosome-negative myeloproliferative neoplasms (MPNs) are a heterogeneous group of hematopoietic stem cell clonal diseases. Most patients with MPN are asymptomatic at diagnosis although some of them suffer from constitutional symptoms. Thrombosis and bleeding can also be one of the initial manifestations although the reported prevalence varied considerably across the studies. Read More

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http://dx.doi.org/10.1186/s12885-019-5387-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393965PMC
February 2019
2 Reads
3.362 Impact Factor

Polycythemia Vera Presenting as Cardiac Arrest: Novel Management Strategies.

Case Rep Cardiol 2019 22;2019:9656387. Epub 2019 Jan 22.

Division of Cardiology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Canada.

Acute coronary syndromes (ACS) usually occur in patients with multiple cardiac risk factors. In young adults, drug use and hypercoagulable states are common causes for ACS presentations. We report a case of a man in his early 30s who was diagnosed with polycythemia vera (PV) and had a cardiac arrest due to an anterolateral ST elevation myocardial infarction. Read More

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http://dx.doi.org/10.1155/2019/9656387DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362489PMC
January 2019
1 Read

Hepcidin-ferroportin axis in health and disease.

Vitam Horm 2019 8;110:17-45. Epub 2019 Feb 8.

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

Hepcidin is central to regulation of iron metabolism. Its effect on a cellular level involves binding ferroportin, the main iron export protein, resulting in its internalization and degradation and leading to iron sequestration within ferroportin-expressing cells. Aberrantly increased hepcidin leads to systemic iron deficiency and/or iron restricted erythropoiesis. Read More

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http://dx.doi.org/10.1016/bs.vh.2019.01.002DOI Listing
February 2019
2 Reads

Therapeutic erythroapheresis: Experience in patients with polycythemia vera and secondary erythrocytosis.

Med Clin (Barc) 2019 Feb 19. Epub 2019 Feb 19.

Banco de Sangre y Tejidos de Navarra, Pamplona, España; Grupo de Trabajo de la Sociedad Española de Transfusión Sanguínea «Hemoterapia basada en sentido común», España. Electronic address:

Therapeutic erythrocytapheresis (TE) is a more efficient strategy compared to phlebotomy to deplete levels of haematocrit in primary and secondary erythrocytosis.

Objective: To analyse response rate and safety profile of TE in polycythemia vera (PV) and secondary erythrocytosis (SE).

Patients And Method: Retrospective review of all patients with PV or SE treated with TE, due to phlebotomy failure, or comorbidities that prevented changes of blood volumen. Read More

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http://dx.doi.org/10.1016/j.medcli.2018.12.016DOI Listing
February 2019
1 Read

Signal transducer and activator of transcription (STAT)-5: an opportunity for drug development in oncohematology.

Oncogene 2019 Feb 19. Epub 2019 Feb 19.

Instituto Universitario de Investigaciones Biomédicas y Sanitarias (IUIBS), Universidad de Las Palmas de Gran Canaria, Farmacología Molecular y Traslacional - BIOPharm, Las Palmas de G.C., 35016, Las Palmas, Spain.

The signal transducer and activator of transcription (STAT) are transcription factors that work via JAK/STAT pathway regulating the expression of genes involved in cell survival, proliferation, differentiation, development, immune response, and, among other essential biological functions, hematopoiesis. JAK/STAT signaling is strictly regulated under normal physiological conditions. However, a large group of diverse diseases has been associated to an aberrant regulation of STAT factors. Read More

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http://dx.doi.org/10.1038/s41388-019-0752-3DOI Listing
February 2019
1 Read

Spontaneous Subdural Hematoma in a Patient with Polycythemia Vera.

World Neurosurg 2019 Feb 16. Epub 2019 Feb 16.

Department of Neurosurgery, Albany Medical Center, Albany, NY, USA.

Polycythemia vera (PV) is a myeloproliferative disorder usually characterized by an increase tendency towards thromboembolic events. Spontaneous hemorrhage/bleeding in PV patients is seldom reported in Neurosurgical literature. We report the case of a 76 year-old male with PV who developed a spontaneous subdural hematoma requiring surgical evacuation. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.258DOI Listing
February 2019
1 Read

Feasibility of Qualitative Testing of BCR-ABL and JAK2 V617F in Suspected Myeloproliperative Neoplasm (MPN) Using RT-PCR Reversed Dot Blot Hybridization (RT-PCR RDB).

Clin Lymphoma Myeloma Leuk 2019 Jan 19. Epub 2019 Jan 19.

Stem Cell and Cancer Institute, Jakarta, Indonesia; Kalbe Genomics Laboratory, Jakarta, Indonesia.

Background: Defining the presence of BCR-ABL transcript in suspected myeloproliferative neoplasm is essential in establishing chronic myeloid leukemia. In the absence of BCR-ABL, the conventional diagnostic algorithm recommends JAK2 V617F mutation testing to support diagnosis of other MPN diseases such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In certain cases of thrombocythemia, simultaneous upfront testing of both BCR-ABL and JAK2 may be desirable. Read More

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http://dx.doi.org/10.1016/j.clml.2019.01.005DOI Listing
January 2019
2 Reads

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
3 Reads

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
1 Read

[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
3 Reads

[Allele-specific polymerase chain reaction and electrophoretic detection in the detection algorithm clinically significant somatic mutations in the gene of calreticulin (calr).]

Klin Lab Diagn 2018 ;63(9):588-592

Central Research Institute of Epidemiology, 111123, Moscow, Russia.

The detection of somatic mutations in the 9 exon of the calreticulin gene (CALR) is regulated by the clinical recommendations as a diagnostic criterion for chronic Ph-negative myeloproliferative neoplasms (MPN). Some methods of nucleic acids testing are used to identify CALR gene mutations with different requirements for special skills of personnel and expensive equipment. The purpose of this work is to compare the results of the detection of CALR gene mutations in venous blood samples by allele-specific RT-PCR with subsequent electrophoresis, fragment analysis and Sanger- or pyro- sequencing. Read More

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http://dx.doi.org/10.18821/0869-2084-2018-63-8-588-592DOI Listing
January 2018
1 Read

The effect of polycythemia vera on hearing functions: Evaluation of twenty-one patients.

Clin Otolaryngol 2019 Feb 8. Epub 2019 Feb 8.

Department of Hematology, Ege University Medicine Faculty, Izmir, Turkey.

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http://dx.doi.org/10.1111/coa.13303DOI Listing
February 2019
2 Reads

Patient-Derived Induced Pluripotent Stem Cells for Alpha-1 Antitrypsin Deficiency Disease Modeling and Therapeutic Discovery.

Chronic Obstr Pulm Dis 2018 Sep 15;5(4):258-266. Epub 2018 Sep 15.

Center for Regenerative Medicine of Boston University and Boston Medical Center, Boston, Massachusetts.

PIZZ alpha-1 antitrypsin deficiency (AATD) is an autosomal recessive disease affecting approximately 100,000 individuals in the United States and one of the most common hereditary causes of liver disease. The most common form of the disease results from a single base pair mutation (Glu342Lys), known as the "Z" mutation, that encodes a mutant protein (Z alpha-1 antritypsin [AAT]) that is prone to misfolding and is retained in the endoplasmic reticulum (ER) rather than appropriately secreted. Some of the retained mutant protein attains an unusual aggregated or conformation. Read More

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https://journal.copdfoundation.org/jcopdf/id/1204/Patient-De
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http://dx.doi.org/10.15326/jcopdf.5.4.2017.0179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361479PMC
September 2018
8 Reads

The Implications of Liver Biopsy Results in Patients with Myeloproliferative Neoplasms Being Treated with Ruxolitinib.

Case Rep Hematol 2019 6;2019:3294046. Epub 2019 Jan 6.

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

Ruxolitinib is increasingly being utilized for the treatment of myelofibrosis and polycythemia vera, but the potential for hepatic toxicity is poorly understood. We performed a retrospective review of hepatic damage occurring in patients with myeloproliferative neoplasms receiving ruxolitinib. Relevant histologic images of liver biopsies were reviewed by an experienced liver pathologist and reported to a multidisciplinary team including hepatology and hematology. Read More

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http://dx.doi.org/10.1155/2019/3294046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339753PMC
January 2019
2 Reads

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
7 Reads

[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

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
10 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
2 Reads

Janus kinase 2 V617F mutation in an unrelated peripheral blood stem cell donor.

Ci Ji Yi Xue Za Zhi 2019 Jan-Mar;31(1):60-62

Buddhist Tzu Chi Stem Cells Center, Hualien, Taiwan.

Polycythemia vera (PV) is relatively uncommon in early adulthood, and evidence about the prevalence of the Janus kinase 2 (JAK2) V617F mutation in the general population is limited. Here, we report a previously healthy volunteer peripheral blood stem cell (PBSC) donor who developed symptomatic PV with the JAK2 V617F mutation 2 years after PBSC mobilization and harvest. The characteristic mutation was identified retrospectively in the blood sample of the donor at the confirmation typing stage, which was before granulocyte colony-stimulating factor injection. Read More

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http://dx.doi.org/10.4103/tcmj.tcmj_189_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334566PMC
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

Polycythaemia Vera among Sudanese Patients with Special Emphasis on JAK2 Mutations

Asian Pac J Cancer Prev 2019 Jan 25;20(1):41-44. Epub 2019 Jan 25.

Department of Haematology, Faculty of Medical Laboratory Sciences, Al-Neelain University, Khartoum, Sudan.

Background: In recent years, a somatic point mutation in the Janus Kinase 2 (JAK2) gene (1849 G→T, V617F) has been reported to occur in over 90% of patients with polycythemia vera (PV). Another JAK2 mutation in exon 12 had been described and shown capable of activating erythropoietin signaling pathways. Objective: In this study, we aimed to determine the frequency of Jak2 mutations (JAK2V617F and JAK2 exon 12) as well as their relationships with hematological parameters in Sudanese patients with myeloproliferative disorders (MPD). Read More

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http://dx.doi.org/10.31557/APJCP.2019.20.1.41DOI Listing
January 2019
2 Reads

[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
5 Reads

Tuberculosis and atypical mycobacterial infections in ruxolitinib-treated patients with primary or secondary myelofibrosis or polycythemia vera.

Int J Infect Dis 2019 Mar 11;80:134-136. Epub 2019 Jan 11.

Department of Oncology and Haematology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Université de Strasbourg, Inserm UMR-S1113/IRFAC, Strasbourg, France.

Ruxolitinib is a JAK-1/JAK-2 inhibitor indicated for the treatment of polycythemia vera and primary or secondary myelofibrosis. Only one patient (0.2%) was diagnosed with tuberculosis among the 485 patients receiving ruxolitinib in the four pivotal trials. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S12019712193001
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http://dx.doi.org/10.1016/j.ijid.2019.01.002DOI Listing
March 2019
12 Reads

Young versus old age at diagnosis confers distinct genomic profiles in patients with polycythemia vera.

Leukemia 2019 Jan 11. Epub 2019 Jan 11.

Division of Hematology, Washington University School of Medicine, St. Louis, MO, USA.

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http://dx.doi.org/10.1038/s41375-018-0349-9DOI Listing
January 2019
1 Read

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
12 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
4 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
2 Reads

A CBC algorithm combined with immature platelet fraction is able to identify JAK2 V617F mutation-positive polycythaemia vera patients.

Int J Lab Hematol 2019 Jan 2. Epub 2019 Jan 2.

Haematology Laboratory, Medlab Central, Palmerston North Hospital, Palmerston North, New Zealand.

Introduction: The recent lowering of the World Health Organisation (WHO) haemoglobin (Hb) and haematocrit (HCT) thresholds for diagnosis of polycythaemia vera (PV) has markedly increased the proportion of complete blood counts (CBC) that could be referred for haematologist review for comment, including advice on JAK2 V617F mutation analysis. The utility of an algorithm based on CBC indices and immature platelet fraction (IPF) to select cases for haematologist review was investigated.

Methods: IPF analysis was performed on 22 patients with known JAK2 V617F mutation and 41 patients who were negative for this mutation previously tested because of suspicion of PV. Read More

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http://dx.doi.org/10.1111/ijlh.12967DOI Listing
January 2019
6 Reads

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
2 Reads

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
5 Reads

A case report of cerebral infarction caused by polycythemia vera.

Medicine (Baltimore) 2018 Dec;97(52):e13880

Department of Radiology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, China.

Rationale: Polycythemia vera (PV) is a cloned erythrocytotic disease manifested by high proliferation and apoptosis in the bone marrow. The clinical symptoms of PV are occult. In practice, patients with cerebral infarction caused by PV are prone to misdiagnosis and missed diagnosis. Read More

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http://dx.doi.org/10.1097/MD.0000000000013880DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314744PMC
December 2018
2 Reads

Treatment of polycythemia vera.

Authors:
Petr Dulíček

Vnitr Lek 2018 ;64(10):955-960

Polycythemia vera is a chronic myeloproliferative neoplasm characterized by hematopoietic stem cell-derived clonal myeloproliferation resulting in erythrocytosis, leukocytosis and thrombocytosis. Survival is reduced compared with general population. Main reasons of death include thrombohemorrhagic complications, fibrotic progression and leuk-aemic transformation. Read More

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January 2018
8 Reads

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
2 Reads

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
6 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
4 Reads
1.933 Impact Factor

Oxidative and nitrosative stress in myeloproliferative neoplasms: the impact on the AKT / mTOR signaling pathway.

J BUON 2018 Sep-Oct;23(5):1481-1491

Deparment of Molecular Biology, Institute for Medical Research, University of Belgrade, Belgrade, Serbia.

Purpose: A common feature of malignancies is increased reactive oxygen species (ROS) and reactive nitrogen species (RNS). We analyzed the influence of oxidative and nitrosative stress on the activation of AKT/mTOR signaling pathway in myeloproliferative neoplasms (MPN).

Methods: Oxidative stress-induced gene expression in circulatory CD34+ cells of MPN patients was studied by microarray analysis. Read More

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December 2018
14 Reads

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
25 Reads

The Role of Advanced Practitioners in Optimizing Clinical Management and Support of Patients With Polycythemia Vera.

J Adv Pract Oncol 2018 Jan-Feb;9(1):56-66. Epub 2018 Jan 1.

The University of Arizona Cancer Center, Tucson, Arizona; and University of Colorado Anschutz Medical Campus, Aurora, Colorado.

Mr. M, a 65-year-old male, presented to his primary care physician with progressive fatigue, difficulty sleeping, and daily headaches for the past 3 weeks. His headaches were not associated with visual disturbances, cognitive deficits, or nausea/vomiting, and he had no history of migraines. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296415PMC
January 2018
2 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
12 Reads