4,654 results match your criteria Thrombocytosis Essential


Acute ST-segment Elevation Myocardial Infarction as the First Manifestation of Essential Thrombocytosis.

Cureus 2019 Feb 7;11(2):e4032. Epub 2019 Feb 7.

Oncology, Florida Cancer Specialists, Atlantis, USA.

A 36-year-old female with no significant past medical history presented with sudden onset of crushing substernal chest pain. When the emergency medical services (EMS) arrived, she had a cardiac arrest requiring defibrillation two times in the field prior to arriving at the hospital. In the emergency department (ED), the electrocardiogram (ECG) was significant for ST-elevation that suggested acute anterolateral infarct. Read More

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http://dx.doi.org/10.7759/cureus.4032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6456283PMC
February 2019

[Relationship between Thrombotic Events and Peripheral Neutrophil-to-lymphocyte Ratio in Patients with Newly Diagnosed Essential Thrombocythemia].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Apr;27(2):534-538

Institute of Hematology, Xuzhou Medical University The Affiliated Hospital of Xuzhou Medical University, Xuzhou 221002, Jiangsu Province, China.Department of Hematology.The Affiliated Hospital of Xuzhou Medical University, Xuzhou 221002, Jiangsu Province, China.E-mail:

Objective: To explore the role of neutrophil-to-lymphocyte ratio(NLR) in patients with newly diagnosed essential thrombocythemia(ET) and its relationship with thrombotic events.

Methods: The clinical and follow-up data of 150 ET patients were retrospectively analyzed. The risk factors of thrombotic events and role of NLR by statistical methods. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2019.02.036DOI Listing
April 2019
3 Reads

[Symptomatic Profiles of 173 Chinese Patients with Essential Thrombocythemia].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Apr;27(2):520-526

State Key Laboratory of Experimental Hematology, Key Laboratory of Gene Therapy for Blood Disease, Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, 300020, China,E-mail:

Objective: To explore the symptomatic burden of patients with essential thrombocythemia (ET) and its relation with clinical characteristics including the mutation status, therapeutic protocols and sex.

Methods: Total of 173 Chinese ET patients were selected and grouped on the basis of disease characteristics (mutation status, therapeutic pro to- cols, and sex).

Results: All the groups showed low-to-high symptom burden, with the highest in the Hu (hydroxyurea)-group (total symptom score [TSS], 14. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2019.02.034DOI Listing
April 2019
4 Reads

and mutations in essential thrombocythemia: Case series and review of literature.

Hematol Rep 2019 Feb 12;11(1):7868. Epub 2019 Mar 12.

Hematology/Oncology Department, Joan C. Edwards School of Medicine, Marshall University, WV, USA.

Essential thrombocythemia (ET) is a clonal bone marrow disease, characterized by increased production of platelets along with other clinical and bone marrow findings. Most patients with ET will have a somatic mutation in one of the known gene locations of , or that can upregulate the JAK-STAT pathway. mutation is present in 5% of cases with the most common mutations being and In this report we describe 2 cases of patients with clinical and laboratory picture of ET. Read More

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https://www.pagepress.org/journals/index.php/hr/article/view
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http://dx.doi.org/10.4081/hr.2019.7868DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434327PMC
February 2019
1 Read

High Level of Circulating Microparticles in Patients with BCR/ABL Negative Myeloproliferative Neoplasm - a Pilot Study.

Klin Onkol 2019 ;32(2):109-116

Background: Microparticles (MPs) are small (0.1-1 μm) cell-derived vesicles released during activation or apoptosis, with a surface-exposed phosphatidylserine along with antigens indicating the cellular origin. The level of MPs is known to be elevated in thromboembolic diseases and malignancies; it is believed that MPs are not only amplifying but can also initiate the thrombogenesis processes. Read More

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http://dx.doi.org/10.14735/amko2019109DOI Listing
January 2019
1 Read

Clinical and molecular features of patients with prefibrotic primary myelofibrosis previously diagnosed as having essential thrombocythemia in Japan.

Eur J Haematol 2019 Apr 12. Epub 2019 Apr 12.

Department of Hematology and Stem Cell Regulation, Juntendo University Graduate School of Medicine, Tokyo.

Objective: Prefibrotic/early primary myelofibrosis (pre-PMF) and essential thrombocythemia (ET) exhibited different features of bone marrow, however, this is not always easy to judge objectively, making pathologists' distinction often suboptimal. In the WHO 2008 criteria, pre-PMF was not defined as a subgroup of PMF; therefore, affected patients were at a higher risk of misdiagnosis with ET. In this study, we examined the prevalence of pre-PMF patients among those previously diagnosed with ET in Japan. Read More

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http://dx.doi.org/10.1111/ejh.13236DOI Listing
April 2019
1 Read

Mean Platelet Volume and Platelet Distribution Width Indicate that Platelets Remain Small for Most of Their Lifespans in Patients with Essential Thrombocythemia.

Clin Lab 2019 Apr;65(4)

Background: Platelets are large when young and immature and shrink as they age. The mean platelet volume (MPV) and platelet distribution width (PDW) reflect the volume and distribution of platelets, respectively. We compared the MPVs and PDWs of patients with immune thrombocytopenic purpura (ITP) or essential thrombocythemia (ET) to those of healthy individuals to test whether these values can reflect the pathomechanisms of these diseases. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180938DOI Listing
April 2019
1 Read

Interferon Alpha 2a and 2b in Ophthalmology: A Review.

J Interferon Cytokine Res 2019 Apr 5. Epub 2019 Apr 5.

1 Department and Clinic of Ophthalmology, University of Wrocław Medical College, Wrocław, Poland.

Interferon alpha (IFN-α) is a glycoprotein with antitumor, antiviral, and immunomodulatory activity, used widely in the treatment of viral infections (hepatitis B and C, condylomata acuminata, herpes zoster, etc.), hematological disorders (leukemia, multiple myeloma, T cell lymphoma, and essential thrombocythemia), and solid tumors (clear cell carcinoma in the metastatic stage, melanoma, hepatocellular carcinoma, and cervical neoplasia). Studies have proven the effectiveness of IFN-α in the treatment of ophthalmic disorders involving the anterior segment of the eye (conjunctival papilloma, squamous neoplasia, conjunctival mucosa-associated lymphoid tissue, Mooren's ulcer, and vernal keratoconjunctivitis) and the posterior segment of the eye (serpiginous choroidopathy, posterior uveitis, pseudophakic and diabetic cystoid macular edema, and proliferative diabetic retinopathy). Read More

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https://www.liebertpub.com/doi/10.1089/jir.2018.0125
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http://dx.doi.org/10.1089/jir.2018.0125DOI Listing
April 2019
4 Reads

Distinguishing essential thrombocythemia JAK2V617F from polycythemia vera: limitations of erythrocyte values.

Haematologica 2019 Apr 4. Epub 2019 Apr 4.

Weill Cornell Medicine.

Distinguishing essential thrombocythemia JAK2V617F from polycythemia vera is difficult because of shared mutation and phenotypic characteristics. The World Health Organization suggested hemoglobin and hematocrit values to diagnose polycythemia vera, but their sensitivity and specificity were not tested. Moreover, red cell values do not accurately predict red cell mass, which we use to discriminate essential thrombocythemia JAK2V617F from polycythemia vera. Read More

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http://dx.doi.org/10.3324/haematol.2018.213108DOI Listing
April 2019
2 Reads

Characteristics and Outcomes of Young Adults with Philadelphia negative Myeloproliferative Neoplasms.

Eur J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Rappaport faculty of medicine, Technion, Haifa, Israel.

Objective: Approximately 10% of Philadelphia (Ph) negative myeloproliferative neoplasms (NPM) are diagnosed at young adulthood. We aim to define the features of this group.

Methods: A multicenter retrospective study, including patients 18-45 years of age, diagnosed with Ph negative MPN between 1985-2017. Read More

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http://dx.doi.org/10.1111/ejh.13232DOI Listing
April 2019
6 Reads

Coexistence of Hermansky-Pudlak syndrome and JAK2-positive essential thrombocythemia.

Ultrastruct Pathol 2019 Apr 1:1-5. Epub 2019 Apr 1.

b Department of Histology, Cerrahpasa Faculty of Medicine , Istanbul University-Cerrahpasa , Istanbul , Turkey.

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder consisting of oculocutaneous albinism, platelet storage pool deficiency, and lysosomal accumulation of ceroid lipofuscin. The storage pool deficiency of HPS is associated with the lack of dense bodies in the platelets, resulting in impaired response in the secondary phase of aggregation. Patients with HPS have normal coagulation tests; however, their bleeding time is usually prolonged despite normal or increased platelet counts. Read More

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http://dx.doi.org/10.1080/01913123.2019.1593269DOI Listing
April 2019
6 Reads
1.133 Impact Factor

Comparison of DIPSS and MYSEC-PM for prediction of outcome in post-PV and ET myelofibrosis after allogeneic stem-cell transplantation.

Biol Blood Marrow Transplant 2019 Mar 28. Epub 2019 Mar 28.

University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address:

We aimed to validate the MYelofibrosis SECondary to PV and ET prognostic model (MYSEC- PM) in 159 patients with myelofibrosis secondary to polycythemia vera (PV) and essential thrombocythemia (ET) from the European Society for Blood and Marrow Transplantation registry undergoing transplantation from matched siblings or unrelated donors. Furthermore, we aimed to test its prognostic performance in comparison with the Dynamic International Prognostic Scoring System (DIPSS). Score performance was analyzed using the concordance index (C): the probability that a patient who experienced an event had a higher risk score than a patient who did not (C >0. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.03.024DOI Listing
March 2019
3 Reads

Mice with Calr mutations homologous to human CALR mutations only exhibit mild thrombocytosis.

Blood Cancer J 2019 Mar 29;9(4):42. Epub 2019 Mar 29.

Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

Calreticulin (CALR) exon 9 frameshift mutations, commonly detected in essential thrombocythemia (ET) and primary myelofibrosis patients, activate signal transducer and activator of transcription (STAT) proteins in the presence of Myeloproliferative Leukemia Virus (MPL) and induce ET in vivo. Loss of the KDEL motif, an endoplasmic reticulum retention signal, and generation of many positively charged amino acids (AAs) in the mutated C-terminus are thought to be important for disease induction. To test this hypothesis, we generated mice harboring a Calr frameshift mutation using the CRISPR/Cas9 system. Read More

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http://dx.doi.org/10.1038/s41408-019-0202-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440999PMC
March 2019
2 Reads

Contemporary management of essential thrombocythemia in children.

Expert Rev Hematol 2019 Mar 29. Epub 2019 Mar 29.

b Department of Women's and Children's Health, Pediatric Hemato-Oncology , University of Padova , Padova , Italy.

Introduction: Essential thrombocythemia (ET) is a disease which is extremely rare in children. Only recently, data on pediatric ET have become available Areas covered: In children with sustained platelet count over 450 x 10/L, secondary thrombocytosis must be ruled out. ET workup comprehends research of JAK2V617F, CALR and MPL mutations and bone marrow biopsy (BM). Read More

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https://www.tandfonline.com/doi/full/10.1080/17474086.2019.1
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http://dx.doi.org/10.1080/17474086.2019.1602034DOI Listing
March 2019
18 Reads

Fulminant Essential Thrombocythemia Associated with Acquired Von Willebrand Syndrome and Bleeding Episodes in a 14-year-old Girl.

Hamostaseologie 2019 Mar 20. Epub 2019 Mar 20.

Division of Pediatric Hematology/Oncology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Background:  Essential thrombocythemia is a chronic myeloproliferative neoplasm. It is extremely rare in children below 15 years of age with an estimated annual incidence of only 0.09 per million. Read More

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http://dx.doi.org/10.1055/s-0039-1679929DOI Listing
March 2019
1 Read

Evaluation of platelet function in essential thrombocythemia under different analytical conditions.

Platelets 2019 Mar 20:1-8. Epub 2019 Mar 20.

a Medicina II, ASST Santi Paolo e Carlo - Dipartimento di Scienze della Salute , Università degli Studi di Milano , Milano Italy.

Background: Studies of platelet aggregation (PA) in essential thrombocythemia (ET) reported contrasting results, likely due to differences in analytical conditions.

Objective: We investigated platelet aggregation using different techniques and analytical conditions.

Patients And Methods: PA was studied by light-transmission aggregometry (LTA) in platelet-rich plasma (PRP) and impedance aggregometry in PRP and whole blood (WB). Read More

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http://dx.doi.org/10.1080/09537104.2019.1584668DOI Listing
March 2019
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Impact of Hydroxyurea on Survival and Risk of Thrombosis Among Older Patients With Essential Thrombocythemia.

J Natl Compr Canc Netw 2019 Mar;17(3):211-219

bDepartment of Chronic Disease Epidemiology, Yale School of Public Health.

ABSTRACTBackground: Current guidelines recommend hydroxyurea (HU) as frontline therapy for patients with high-risk essential thrombocythemia (ET) to prevent thrombosis. However, little is known about the impact of HU on thrombosis or survival among these patients in the real-world setting.

Patients And Methods: A retrospective cohort study was conducted of older adults (aged ≥66 years) diagnosed with ET from 2007 through 2013 using the linked SEER-Medicare database. Read More

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http://dx.doi.org/10.6004/jnccn.2018.7095DOI Listing
March 2019
1 Read
4.178 Impact Factor

Rare type 1-like and type 2-like calreticulin mutants induce similar myeloproliferative neoplasms as prevalent type 1 and 2 mutants in mice.

Oncogene 2019 03 19;38(10):1651-1660. Epub 2018 Oct 19.

INSERM, UMR 1170, Gustave Roussy, Villejuif, France.

Frameshift mutations in the calreticulin (CALR) gene are present in 30% of essential thrombocythemia and myelofibrosis patients. The two most frequent mutations are CALR del52 (type 1, approximately 60%) and CALR ins5 (type 2, around 30%), but many other rarer mutations exist accounting each for less than 2% of all CALR mutations. Most of them are structurally classified as type 1-like and type 2-like CALR mutations according to the absence or presence of a residual wild-type calcium-binding motif and the modification of the alpha-helix structure. Read More

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http://dx.doi.org/10.1038/s41388-018-0538-zDOI Listing
March 2019
6 Reads

[Essential thrombocythemia accompanied by adrenal infarction].

Rinsho Ketsueki 2019;60(2):106-111

Department of Hematology, Kyoto City Hospital.

A 64-year-old man presented to the emergency department with sudden-onset upper abdominal pain and pain in the left chest area. His platelet count was 121.7×10/µl. Read More

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http://dx.doi.org/10.11406/rinketsu.60.106DOI Listing
January 2019
7 Reads

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

Mayo Clin Proc 2019 Apr 26;94(4):599-610. 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
April 2019
6 Reads

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
3 Reads
3.362 Impact Factor

Homozygous CALR Mutation in Primary Myelofibrosis and Its Effect on Disease Phenotype: A Case Report and Review of the Literature.

Case Rep Hematol 2019 20;2019:1430170. Epub 2019 Jan 20.

Department of Clinical Haematology, National Institute of Blood Diseases & Bone Marrow Transplantation, P.O. Box-75300, Karachi, Pakistan.

Somatic mutations in gene have been reported in 60%-88% of patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF) who are negative for and mutations. Most of the mutations analyzed to date are heterozygous mutations in exon 9 of the gene. Homozygosity in gene is rarely reported, and its association with clinical behavior of disease and impact on outcome of patients is not studied so far. Read More

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http://dx.doi.org/10.1155/2019/1430170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360597PMC
January 2019
3 Reads

Developments in diagnosis and treatment of essential thrombocythemia.

Expert Rev Hematol 2019 Mar 13;12(3):159-171. Epub 2019 Mar 13.

a Ospedale di Circolo , ASST Sette Laghi, Hematology , Varese , Italy.

Introduction: Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterized by thrombocytosis, increased risk of thrombotic/hemorrhagic events and clonal evolution into blast phase or myelofibrosis. Areas covered: The authors will discuss biology, diagnosis, prognosis, therapy, and outcome of ET. An accurate molecular-morphologic assessment is necessary in order to properly establish diagnosis and prognosis of ET. Read More

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http://dx.doi.org/10.1080/17474086.2019.1585239DOI Listing
March 2019
1 Read

Activation of TF-Dependent Blood Coagulation Pathway and VEGF-A in Patients with Essential Thrombocythemia.

Medicina (Kaunas) 2019 Feb 16;55(2). Epub 2019 Feb 16.

Department of Pathophysiology, Faculty of Pharmacy, Nicolaus Copernicus University in Toruń, Collegium Medicum in Bydgoszcz, 85-094 Bydgoszcz, Poland.

Recent studies suggest that a vascular endothelial growth factor (VEGF-A) may be involved in the thrombotic process by stimulating the expression of tissue factor in vascular endothelial cells. Tissue factor (TF) can also stimulate the transcription of the gene encoding VEGF-A. The relationship between coagulation and angiogenesis in myeloproliferative neoplasms is not fully understood. Read More

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http://dx.doi.org/10.3390/medicina55020054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409549PMC
February 2019
2 Reads

Prevalence of MPL (W515K/L) Mutations in Patients with Negative-JAK2 (V617F) Myeloproliferative Neoplasm in North-East of Iran.

Iran J Pathol 2018 25;13(4):397-402. Epub 2018 Sep 25.

Cellular and Molcular Research Center, Birjand University of Medical Sciences, Birjand, Iran.

Background And Objective: Janus kinase 2 (JAK2) and Myeloproliferative Leukemia (MPL) mutations are confirmatory indicators for Myeloproliferative Neoplasm (MPN). The current study was performed to determine the frequency of MPL mutation in MPN patients without JAK2 mutation, in order to assign MPL mutation frequency in North-East of Iran.

Methods: Total of 105 negative JAK2 cases including 5 Myeloproliferative Disorders (MPD), 15 Polycytemia Vera (PV) and 15 Essential Thrombocytosis (ET) who referred to Qaem Medical Center were assigned to this study. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358559PMC
September 2018
2 Reads

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

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377687PMC
February 2019
3 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
4 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
4 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 Mar 7;59(1):29-33. 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
March 2019
3 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
12 Reads

Radiological features and outcomes of essential thrombocythemia-related stroke.

J Neurol Sci 2019 Mar 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
March 2019
2 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
4 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
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
13 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 May 29;98(5):1119-1125. 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
May 2019
6 Reads

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
2 Reads

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

Dtsch Med Wochenschr 2019 01 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
6 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
4 Reads

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
3 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
3 Reads

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

J Stroke Cerebrovasc Dis 2019 Apr 8;28(4):e7-e9. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6441363PMC
April 2019
16 Reads

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

Lab Chip 2019 01;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
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http://dx.doi.org/10.1039/c8lc00527cDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366625PMC
January 2019
13 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
17 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
2 Reads

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

Authors:
Zen Kobayashi

Intern Med 2019 Apr 10;58(8):1193. 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
April 2019
2 Reads