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Myeloproliferative disease

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https://www.readbyqxmd.com/read/29790256/platelet-characteristics-in-patients-with-essential-thrombocytosis
#1
Oliver Heidmann Pedersen, Mads Lamm Larsen, Erik Lerkevang Grove, Peter Buur van Kooten Niekerk, Søren Bønløkke, Peter H Nissen, Steen Dalby Kristensen, Anne-Mette Hvas
BACKGROUND: Essential thrombocytosis (ET) is a myeloproliferative disorder characterized by an increased platelet count. ET is associated with an increased risk of thrombosis, and procoagulant features of the disease may include an increased number of reactive reticulated platelets and an increased aggregation potential. We aimed to explore the association between platelet count, platelet turnover and platelet aggregation in patients with ET. METHODS: We included 24 ET patients who discontinued antiplatelet therapy prior to blood sampling...
May 23, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29780814/targeting-chronic-myeloid-leukemia-stem-cells-can-transcriptional-program-be-a-druggable-target-for-cancers
#2
REVIEW
Yosuke Masamoto, Mineo Kurokawa
Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm resulting from acquisition of constitutively active BCR-ABL protein tyrosine kinase in a hematopoietic stem cell (HSC). Though tyrosine kinase inhibitors (TKIs) have changed a fatal disease into manageable disease, most patients cannot discontinue TKI treatment due to persistence of TKI-resistant leukemia stem cells (LSCs). Much effort has been made to find out factors or pathways specifically operating in LSCs to selectively target LSCs, with some promising results at least in preclinical models...
2018: Stem Cell Investigation
https://www.readbyqxmd.com/read/29774106/hematopoietic-restricted-ptpn11e76k-reveals-indolent-mpn-progression-in-mice
#3
Stefan P Tarnawsky, Wen-Mei Yu, Cheng-Kui Qu, Rebecca J Chan, Mervin C Yoder
Juvenile Myelomonocytic Leukemia (JMML) is a pediatric myeloproliferative neoplasm (MPN) that has a poor prognosis. Somatic mutations in Ptpn11 are the most frequent cause of JMML and they commonly occur in utero . Animal models of mutant Ptpn11 have probed the signaling pathways that contribute to JMML. However, existing models may inappropriately exacerbate MPN features by relying on non-hematopoietic-restricted Cre-loxP strains or transplantations into irradiated recipients. In this study we generate hematopoietic-restricted models of Ptpn11E76K-mediated disease using Csf1r-MCM and Flt3Cre...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29773831/dnajc17-is-localized-in-nuclear-speckles-and-interacts-with-splicing-machinery-components
#4
A Pascarella, G Ferrandino, S C Credendino, C Moccia, F D'Angelo, B Miranda, C D'Ambrosio, P Bielli, O Spadaro, M Ceccarelli, A Scaloni, C Sette, M De Felice, G De Vita, E Amendola
DNAJC17 is a heat shock protein (HSP40) family member, identified in mouse as susceptibility gene for congenital hypothyroidism. DNAJC17 knockout mouse embryos die prior to implantation. In humans, germline homozygous mutations in DNAJC17 have been found in syndromic retinal dystrophy patients, while heterozygous mutations represent candidate pathogenic events for myeloproliferative disorders. Despite widespread expression and involvement in human diseases, DNAJC17 function is still poorly understood. Herein, we have investigated its function through high-throughput transcriptomic and proteomic approaches...
May 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29761371/tolerability-and-efficacy-of-deferasirox-in-patients-with-transfusional-iron-overload-results-from-a-german-2-year-non-interventional-study
#5
Florian Nolte, Holger Nückel, Burkhard Schmidt, Thomas Geer, Oleg Rubanov, Holger Hebart, Andrea Jarisch, Stefan Albrecht, Christiane Johr, Christiane Schumann, Wolf-Karsten Hofmann
BACKGROUND: Iron overload (IOL) due to repetitive transfusions of packed red blood cells (pRBC) has a major impact on morbidity and mortality in patients with inherited bone marrow failure syndromes and hemoglobinopathies such as thalassemia and sickle cell disease. However, whether IOL influences the outcome of elderly patients with myeloid malignancies is not yet clear. Moreover, clinical trials have reported high drop-out rates during treatment with the oral iron chelator deferasirox (DFX)...
May 14, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29741513/myelofibrosis-clinicopathologic-features-prognosis-and-management
#6
Jennifer M O'Sullivan, Claire N Harrison
Myelofibrosis is one of the BCR-ABL-negative clonal disorders that collectively are known as myeloproliferative neoplasms (MPNs). It is caused by the proliferation of clonal hematopoietic stem cells, which over time leads to characteristic clinical features. The disease presentation is heterogeneous, however, with 30% of patients initially asymptomatic. This variation in clinical phenotype warrants careful risk stratification to guide appropriate management, and prognostic risk scores are continually being refined...
February 2018: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29730367/peripheral-artery-disease-associated-with-myeloproliferative-disorders
#7
Shoko Nakagawa, Osami Kawarada, Takeshi Yagyu, Jiro Matsuo, Yosuke Inoue, Teruo Noguchi, Satoshi Yasuda
No abstract text is available yet for this article.
April 27, 2018: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/29728434/focal-segmental-glomerulosclerosis-in-a-patient-with-prefibrotic-primary-myelofibrosis
#8
Gopal Krishana Bohra, Durga Shankar Meena, Nitin Bajpai, Abhishek Purohit
We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis...
May 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29719285/myeloproliferative-neoplasms-in-danish-twins
#9
Michael Asger Andersen, Ole Weis Bjerrum, Ajenthen Ranjan, Vibe Skov, Torben A Kruse, Mads Thomassen, Axel Skytthe, Hans Carl Hasselbalch, Kaare Christensen
OBJECTIVE: Myeloproliferative neoplasms (MPNs) are a heterogeneous group of diseases characterized by clonal hyperproliferation of immature and mature cells of the myeloid lineage. Genetic differences have been proposed to play a role in the development of MPNs. Monozygotic twin pairs with MPNs have been reported in a few case reports, but the MPN concordance pattern in twins remains unknown. METHOD: All twin pairs born in the period 1900-2010 were identified in the nationwide Danish Twin Registry...
May 2, 2018: Acta Haematologica
https://www.readbyqxmd.com/read/29715157/elevated-neutrophil-to-lymphocyte-ratio-and-platelet-to-lymphocyte-ratio-in-myelofibrosis-inflammatory-biomarkers-or-representatives-of-myeloproliferation-itself
#10
Marko Lucijanic, David Cicic, Tajana Stoos-Veic, Vlatko Pejsa, Jelena Lucijanic, Amina Fazlic Dzankic, Josipa Vlasac Glasnovic, Ena Soric, Marko Skelin, Rajko Kusec
BACKGROUND/AIM: We aimed to investigate clinical associations of inflammatory biomarkers neutrophil-to-lymphocyte-ratio (NLR) and platelet-to-lymphocyte-ratio (PLR) in patients with myelofibrosis, myeloproliferative neoplasm with inflammatory background. PATIENTS AND METHODS: We retrospectively analyzed a cohort of 102 myelofibrosis patients. NLR and PLR were assessed in addition to other disease-specific parameters. RESULTS: NLR and PLR were significantly higher in myelofibrosis than in healthy controls...
May 2018: Anticancer Research
https://www.readbyqxmd.com/read/29713321/-clec9a-mediated-ablation-of-conventional-dendritic-cells-suggests-a-lymphoid-path-to-generating-dendritic-cells-in-vivo
#11
Johanna Salvermoser, Janneke van Blijswijk, Nikos E Papaioannou, Stephan Rambichler, Maria Pasztoi, Dalia Pakalniškytė, Neil C Rogers, Selina J Keppler, Tobias Straub, Caetano Reis E Sousa, Barbara U Schraml
Conventional dendritic cells (cDCs) are versatile activators of immune responses that develop as part of the myeloid lineage downstream of hematopoietic stem cells. We have recently shown that in mice precursors of cDCs, but not of other leukocytes, are marked by expression of DNGR-1/CLEC9A. To genetically deplete DNGR-1-expressing cDC precursors and their progeny, we crossed Clec9a-Cre mice to Rosa-lox-STOP-lox-diphtheria toxin (DTA) mice. These mice develop signs of age-dependent myeloproliferative disease, as has been observed in other DC-deficient mouse models...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29709377/high-energy-injury-is-a-risk-factor-for-preoperative-venous-thromboembolism-in-the-patients-with-hip-fractures-a-prospective-observational-study
#12
Jin Sup Park, Jae Hoon Jang, Ki Young Park, Nam Hoon Moon
BACKGROUND: The purpose of this study was to identify the incidence of preoperative venous thromboembolism (VTE), and determine if high energy hip fracture affects preoperative VTE occurrence. METHODS: Three-hundred nine patients (244 low and 61 high energy injuries) treated between March 2015 and March 2017 were included in this study. Indirect multidetector computed tomographic venography for the detection of preoperative VTE was performed at admission. The incidence of preoperative VTE was compared between high and low energy injury hip fractures...
April 24, 2018: Injury
https://www.readbyqxmd.com/read/29706577/spred1-safeguards-hematopoietic-homeostasis-against-diet-induced-systemic-stress
#13
Yuko Tadokoro, Takayuki Hoshii, Satoshi Yamazaki, Koji Eto, Hideo Ema, Masahiko Kobayashi, Masaya Ueno, Kumiko Ohta, Yuriko Arai, Eiji Hara, Kenichi Harada, Masanobu Oshima, Hiroko Oshima, Fumio Arai, Akihiko Yoshimura, Hiromitsu Nakauchi, Atsushi Hirao
Stem cell self-renewal is critical for tissue homeostasis, and its dysregulation can lead to organ failure or tumorigenesis. While obesity can induce varied abnormalities in bone marrow components, it is unclear how diet might affect hematopoietic stem cell (HSC) self-renewal. Here, we show that Spred1, a negative regulator of RAS-MAPK signaling, safeguards HSC homeostasis in animals fed a high-fat diet (HFD). Under steady-state conditions, Spred1 negatively regulates HSC self-renewal and fitness, in part through Rho kinase activity...
May 3, 2018: Cell Stem Cell
https://www.readbyqxmd.com/read/29703879/progressive-pulmonary-vein-stenosis-in-down-syndrome-infant-a-rare-cause-of-pulmonary-hypertension
#14
Z Norazah, D S Joyce Darshinee
Pulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a Down Syndrome infant with congenital heart disease and transient myeloproliferative leukaemia. This case-report aims to improve awareness among physicians and sonographers of this disease and the importance of pulmonary vein assessment in congenital heart disease with unexplained pulmonary hypertension...
April 2018: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29692658/atypical-chronic-myeloid-leukaemia-a-rare-subtype-of-myelodysplastic-myeloproliferative-neoplasm
#15
REVIEW
Joanna E Drozd-Sokołowska, Anna Waszczuk-Gajda, Krzysztof Mądry, Jadwiga Dwilewicz-Trojaczek
Atypical chronic myeloid leukaemia (aCML) belongs to the group of myelodysplastic/myeloproliferative neoplasms. Changing diagnostic criteria and the rarity of the disease, with incidence approximately 100-times lower than the incidence of BCR-ABL1 -positive chronic myeloid leukaemia, result in limited knowledge on aCML. At present the diagnosis is made based on the presence of granulocytic lineage dysplasia and precisely defined quantitative peripheral blood criteria, after exclusion of other molecularly defined myeloid neoplasms...
2018: Contemporary Oncology Współczesna Onkologia
https://www.readbyqxmd.com/read/29685167/neutropenia-caused-by-hairy-cell-leukemia-in-a-patient-with-myelofibrosis-secondary-to-polycythemia-vera-a-case-report
#16
Andreas Hanssønn Habberstad, Hoa Thi Tuyet Tran, Ulla Randen, Signe Spetalen, Ingunn Dybedal, Geir E Tjønnfjord, Anders Erik Astrup Dahm
BACKGROUND: Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia. CASE PRESENTATION: A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis...
April 24, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29683667/optimization-of-selective-mitogen-activated-protein-kinase-interacting-kinases-1-and-2-mnk1-2-inhibitors-for-the-treatment-of-blast-crisis-leukemia
#17
Hai Yan Yang, Lohitha Rao Chennamaneni, Melvyn Wai Tuck Ho, Shi Hua Ang, Eldwin Sum Wai Tan, Duraiswamy Athisayamani Jeyaraj, Yoon Sheng Yeap, Boping Liu, Esther Hq Ong, Joma Kanikadu Joy, John Liang Kuan Wee, Perlyn Kwek, Priya Retna, Nurul Dinie, Thuy Thi Hanh Nguyen, Shi Jing Tai, Vithya Manoharan, Vishal Pendharkar, Choon Bing Low, Yun Shan Chew, Susmitha Vuddagiri, Kanda Sangthongpitag, Meng Ling Choong, May Ann Lee, Srinivasaraghavan Kannan, Chandra S Verma, Anders Poulsen, Sharon Lim, Charles Chuah, Tiong Sin Ong, Jeffrey Hill, Alex Matter, Kassoum Nacro
Chronic Myeloid Leukemia (CML) is a myeloproliferative disease caused by bcr-abl1, a constitutively active tyrosine kinase fusion gene responsible for an abnormal proliferation of leukemic stem cells (LSCs). Inhibition of BCR-ABL1 kinase activity offers long term relief to CML patients. However, for a proportion of them, BCR-ABL1 inhibition will become ineffective at treating the disease and CML will progress to blast crisis (BC) CML with poor prognosis. BC-CML is often associated with excessive phosphorylated eukaryotic translation initiation factor 4E (eIF4E) which renders LSCs capable of proliferating via self-renewal, oblivious to BCR-ABL1 inhibition...
April 23, 2018: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29683036/assessment-of-endothelial-dysfunction-with-flow-mediated-dilatation-in-myeloproliferative-disorders
#18
Abdulkerim Yildiz, Melike Güryildirim, Mehmet Sezgin Pepeler, Merve Yazol, Suna Özhan Oktar, Kadir Acar
BACKGROUND: Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs. METHODS: Endothelial dysfunction and its associations with other parameters were investigated. A total of 18 patients with polycythemia vera (PV), 24 with essential thrombocytosis (ET), 7 with primary myelofibrosis (PMF), and 30 healthy patients as a control group were included in the study...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29682367/myelodysplastic-syndrome-acute-myeloid-leukemia-arising-in-idiopathic-erythrocytosis
#19
Stephen E Langabeer, Eibhlin Conneally, Catherine M Flynn
The term "idiopathic erythrocytosis (IE)" is applied to those cases where a causal clinical or pathological event cannot be elucidated and likely reflects a spectrum of underlying medical and molecular abnormalities. The clinical course of a patient with IE is described manifesting as a persistent erythrocytosis with a low serum erythropoietin level, mild eosinophilia, and with evidence of a thrombotic event. The patient subsequently developed a myelodysplasic syndrome (MDS) and acute myeloid leukemia (AML), an event not observed in erythrocytosis patients other than those with polycythemia vera (PV)...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29668539/myeloproliferative-neoplasms-in-children-and-adolescents-and-thrombosis-at-unusual-sites-the-role-of-driver-mutations
#20
Laith Tafesh, Kathryn Musgrave, Wing Roberts, Dianne Plews, Peter Carey, Tina Biss
Myeloproliferative neoplasms (MPNs) in childhood and adolescence are rare and seldom complicated by thrombosis. We describe 3 cases of thrombosis at unusual sites in young patients with MPNs. In the pediatric MPN population, unlike in adult MPNs, a clonal mutation is identifiable in only a minority of cases (22% to 26%). All 3 of these individuals had JAK2 mutations driving the disease process. A literature search identified 19 cases of MPN-associated thrombosis in children. Seventeen of the 19 children (89...
April 17, 2018: Journal of Pediatric Hematology/oncology
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