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Chronic myeloproliferative neoplasm

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https://www.readbyqxmd.com/read/29622863/summary-and-review-of-the-abstracts-on-philadelphia-negative-myeloproliferative-neoplasms-presented-at-haematocon-2017
#1
Tathagata Chatterjee, Ankur Ahuja
There are lot of grey zones in Philadelphia negative chronic myeloproliferative neoplasms (CMPNs) and that's the reason they are in hit list of researchers. Having a spectrum of disorders their diagnosis is very important and especially to differentiate from each other since they overlap with each other in many ways. Diagnosis doesn't start from lab but with clinical phenotype. Clinical phenotype not only able to provide us the diagnosis but also helps in management of the disease per se. When diagnosis comes, the old timer but an evergreen morphology plays an important role which along with the newer generation tool "molecular" helps in differentiating these disorders...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29622860/laboratory-monitoring-of-chronic-myeloid-leukemia-in-patients-on-tyrosine-kinase-inhibitors
#2
REVIEW
Richa Chauhan, Sudha Sazawal, H P Pati
Chronic Myeloid Leukemia (CML) is a myeloproliferative neoplasm characterized by translocation of genetic material from chromosome 9 to chromosome 22 to form a fusion gene (BCR-ABL1) that is responsible for abnormal tyrosine kinase activity and alteration of various downstream signaling pathways. In addition to morphological diagnosis of CML phase, it is essential to detect BCR-ABL1 fusion by either metaphase cytogenetics or reverse transcriptase polymerase chain reaction that also determines type of mRNA transcript...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29616837/outcome-of-ph-negative-myeloproliferative-neoplasms-transforming-to-accelerated-or-leukemic-phase
#3
Lise-Marie Mollard, Aurélie Chauveau, Françoise Boyer-Perrard, Nathalie Douet-Guilbert, Roch Houot, Isabelle Quintin-Roué, Marie-Anne Couturier, Anaig Dagorne, Mohamed Malou, Ronan Le Calloch, Odile Luycx, Sylvain Thepot, Mathilde Hunault, Gaelle Guillerm, Christian Berthou, Valérie Ugo, Éric Lippert, Jean-Christophe Ianotto
Myeloproliferative neoplasms (MPN) are chronic disorders that can sometimes evolve into accelerated or leukemic phases. We retrospectively identified 122 patients with such blastic phases. The overall median survival was four months: 10.2 months for patients treated with intensive treatments compared to three months for best supportive care (p = .005). Azacytidine, intensive chemotherapies, or allogeneic stem cell transplantation gave the highest median survivals with 9, 10.2, and 19.4 months, respectively...
April 4, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29599206/impact-of-hydroxycarbamide-and-interferon-%C3%AE-on-red-cell-adhesion-and-membrane-protein-expression-in-polycythemia-vera
#4
Mégane Brusson, Maria De Grandis, Sylvie Cochet, Sylvain Bigot, Mickaël Marin, Marjorie Leduc, François Guillonneau, Patrick Mayeux, Thierry Peyrard, Christine Chomienne, Caroline Le Van Kim, Bruno Cassinat, Jean-Jacques Kiladjian, Wassim El Nemer
Polycythemia vera is a chronic myeloproliferative neoplasm characterized by the JAK2V617F mutation, elevated blood cell counts and a high risk of thrombosis. Although the red cell lineage is primarily affected by JAK2V617F, the impact of mutated JAK2 on circulating red blood cells is poorly documented. Recently, we showed that in polycythemia vera erythrocytes had abnormal expression of several proteins including Lu/BCAM adhesion molecule and proteins from the endoplasmic reticulum, mainly calreticulin and calnexin...
March 29, 2018: Haematologica
https://www.readbyqxmd.com/read/29587671/coexisting-of-bone-marrow-fibrosis-dysplasia-and-an-x-chromosomal-abnormality-in-chronic-neutrophilic-leukemia-with-csf3r-mutation-a-case-report-and-literature-review
#5
Xue Bin Wu, Wei Wei Wu, Yue Zhou, Xuan Wang, Jia Li, Yang Yu
BACKGROUND: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN) with less than 40 cases of patients being reported or clinically suspected meeting with 2008 World Health Organization ("WHO") diagnostic criteria. The current diagnosis of CNL remains to exclude other diseases. Recently, a new biomarker of CSF3R mutations that is almost invariably present in CNL has been identified. There is no effective treatment for CNL, therefore prognosis of the disease is poor, but it may be attributed to the presence of both SETBP1 and CSF3R gene mutations...
March 27, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29562644/understanding-splenomegaly-in-myelofibrosis-association-with-molecular-pathogenesis
#6
REVIEW
Moo-Kon Song, Byeong-Bae Park, Ji-Eun Uhm
Myelofibrosis (MF) is a clinical manifestation of chronic BCR-ABL1-negative chronic myeloproliferative neoplasms. Splenomegaly is one of the major clinical manifestations of MF and is directly linked to splenic extramedullary hematopoiesis (EMH). EMH is associated with abnormal trafficking patterns of clonal hematopoietic cells due to the dysregulated bone marrow (BM) microenvironment leading to progressive splenomegaly. Several recent data have emphasized the role of several cytokines for splenic EMH. Alteration of CXCL12/CXCR4 pathway could also lead to splenic EMH by migrated clonal hematopoietic cells from BM to the spleen...
March 18, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29542026/the-role-of-caspase-genes-polymorphisms-in-genetic-susceptibility-to-philadelphia-negative-myeloproliferative-neoplasms-in-a-portuguese-population
#7
Ana P Azevedo, Susana N Silva, Alice Reichert, Fernando Lima, Esmeraldina Júnior, José Rueff
Our main aim was to evaluate the role of caspases' genes SNPs in Philadelphia-chromosome negative chronic myeloproliferative neoplasms (PN-MPNs) susceptibility. A case-control study in 133 Caucasian Portuguese PN-MPNs patients and 281 matched controls was carried out, studying SNPs in apoptosis related caspases: rs1045485 and rs1035142 (CASP8), rs1052576, rs2308950, rs1132312 and rs1052571 (CASP9), rs2227309 and rs2227310 (CASP7) and rs13006529 (CASP10). After stratification by pathology diagnosis for essential thrombocythemia (ET), female gender or JAK2 positive, there is a significant increased risk for those carrying at least one variant allele for CASP9 (C653T) polymorphism (OR 2...
March 14, 2018: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/29534592/effect-of-calr-and-jak2-mutations-on-the-clinical-and-hematological-phenotypes-of-the-disease-in-patients-with-myelofibrosis-long-term-experience-from-a-single-center
#8
M Palova, T Szotkowski, A Hlusi, K Indrak, J Navratilova, M Divoka, T Papajik
Primary myelofibrosis (PMF) is a chronic clonal myeloid disorder. Together with essential thrombocythemia (ET) and polycythemia vera (PV), it belongs to a group of Philadelphia chromosome-negative myeloproliferative neoplasms. An integral part of laboratory tests carried out in this disease group is detecting the presence of mutations in the Janus kinase 2 gene at position 617 (JAK2 V617F) and in the gene encoding for the receptor for thrombopoietin (myeloproliferative leukemia virus oncogene, MPL) found in approximately 60% of PMF patients...
2018: Neoplasma
https://www.readbyqxmd.com/read/29520559/life-for-patients-with-myelofibrosis-the-physical-emotional-and-financial-impact-collected-using-narrative-medicine-results-from-the-italian-back-to-life-project
#9
Francesca Palandri, Giulia Benevolo, Alessandra Iurlo, Elisabetta Abruzzese, Angelo M Carella, Chiara Paoli, Giuseppe A Palumbo, Massimiliano Bonifacio, Daniela Cilloni, Alessandro Andriani, Attilio Guarini, Diamante Turri, Elena Maria Elli, Antonietta Falcone, Barbara Anaclerico, Pellegrino Musto, Nicola Di Renzo, Mario Tiribelli, Renato Zambello, Caterina Spinosa, Alessandra Ricco, Letizia Raucci, Bruno Martino, Mario Annunziata, Silvia Pascale, Anna Marina Liberati, Giorgio La Nasa, Margherita Maffioli, Massimo Breccia, Novella Pugliese, Silvia Betti, Gianfranco Giglio, Antonietta Cappuccio, Luigi Reale
PURPOSE: Myelofibrosis (MF) is a chronic myeloproliferative neoplasm characterised by an aggressive clinical course, with disabling symptoms and reduced survival. Patients experience a severely impaired quality of life and their families face the upheaval of daily routines and high disease-related financial costs. The aim of this study was to investigate the perceptions of Italian patients and their caregivers about living with MF and the burden of illness associated with MF. METHODS: A quali-quantitative questionnaire and a prompted written narrative survey were administered to patients affected by primary or post-essential thrombocythemia/post-polycythaemia vera MF and their primary caregiver in 35 Italian haematological centres...
March 8, 2018: Quality of Life Research
https://www.readbyqxmd.com/read/29512199/chronic-neutrophilic-leukemia-2018-update-on-diagnosis-molecular-genetics-and-management
#10
Michelle A Elliott, Ayalew Tefferi
DISEASE OVERVIEW AND DIAGNOSIS: Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥ 25 x 109 /L of which ≥ 80% are neutrophils, with < 10% circulating neutrophil precursors with blasts rarely observed. In addition, there is no dysplasia, nor clinical or molecular criteria for other myeloproliferative neoplasms. UPDATE ON DIAGNOSIS: Previously the diagnosis of CNL was often as one of exclusion based on no identifiable cause for physiologic neutrophilia in patients fulfilling the aforementioned criteria...
August 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29512182/atypical-chronic-myeloid-leukaemia-a-case-of-an-orphan-disease-a-multicenter-report-by-the-polish-adult-leukemia-group
#11
Joanna Drozd-Sokołowska, Krzysztof Mądry, Anna Waszczuk-Gajda, Przemysław Biecek, Paweł Szwedyk, Katarzyna Budziszewska, Magdalena Raźny, Magdalena Dutka, Agata Obara, Ewa Wasilewska, Krzysztof Lewandowski, Agnieszka Piekarska, Grażyna Bober, Helena Krzemień, Beata Stella-Hołowiecka, Katarzyna Kapelko-Słowik, Waldemar Sawicki, Małgorzata Paszkowska-Kowalewska, Rafał Machowicz, Jadwiga Dwilewicz-Trojaczek
Atypical chronic myeloid leukaemia (aCML) belongs to myelodysplastic/myeloproliferative neoplasms. Because of its rarity and changing diagnostic criteria throughout subsequent classifications, data on aCML are very scarce. Therefore, we at the Polish Adult Leukemia Group performed a nationwide survey on aCML. Eleven biggest Polish centres participated in the study. Altogether, 45 patients were reported, among whom only 18 patients (40%) fulfilled diagnostic criteria. Among misdiagnosed patients, myelodysplastic/myeloproliferative syndrome unclassifiable and chronic myelomonocytic leukaemia were the most frequent diagnoses...
March 7, 2018: Hematological Oncology
https://www.readbyqxmd.com/read/29508552/coexistence-of-jak2-or-calr-mutation-is-a-rare-but-clinically-important-event-in-chronic-myeloid-leukemia-patients-treated-with-tyrosine-kinase-inhibitors
#12
K Lewandowski, M Gniot, M Wojtaszewska, Z Kanduła, R Becht, E Paczkowska, E Mędraś, E Wasilewska, M Iwoła
INTRODUCTION: There are 7 designated conditions under the category of myeloproliferative neoplasms (MPN), including chronic myelogenous leukemia (CML) and classical MPN, that is, polycythemia vera (PV), essential thrombocythaemia (ET), and primary myelofibrosis (PMF). Recently, reports about Philadelphia and JAK2 V617F-positive MPN cases have been described in literature. The coexistence of different molecular defects may change the clinical and laboratory manifestation of MPN and may result in an inappropriate interpretation of the response to treatment with tyrosine kinase inhibitors in CML patients...
March 6, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29483845/-mll2-kmt2d-and-mll3-kmt2c-expression-correlates-with-disease-progression-and-response-to-imatinib-mesylate-in-chronic-myeloid-leukemia
#13
Doralina do Amaral Rabello, Vivian D'Afonseca da Silva Ferreira, Maria Gabriela Berzoti-Coelho, Sandra Mara Burin, Cíntia Leticia Magro, Maira da Costa Cacemiro, Belinda Pinto Simões, Felipe Saldanha-Araujo, Fabíola Attié de Castro, Fabio Pittella-Silva
Background: Chronic myeloid leukemia (CML) is a clonal myeloproliferative neoplasm whose pathogenesis is linked to the Philadelphia chromosome presence that generates the BCR - ABL 1 fusion oncogene. Tyrosine kinase inhibitors (TKI) such as imatinib mesylate (IM) dramatically improved the treatment efficiency and survival of CML patients by targeting BCR-ABL tyrosine kinase. The disease shows three distinct clinical-laboratory stages: chronic phase, accelerated phase and blast crisis...
2018: Cancer Cell International
https://www.readbyqxmd.com/read/29480036/-emerging-drugs-for-the-treatment-of-myelofibrosis
#14
Aditya Shreenivas, John Mascarenhas
INTRODUCTION: Myelofibrosis (MF) is a Philadelphia chromosome-negative myeloproliferative neoplasm (MPN). It can be sub-categorized into primary myelofibrosis, post polycythemia vera myelofibrosis and post essential thrombocythemia myelofibrosis. MF is a life-threatening hematologic malignancy characterized by dysregulation of the Janus associated kinase (JAK)/signal transducer and activator of transcription (STAT) signaling network and a heightened inflammatory state. AREAS COVERED: We cover the pathogenesis, clinical features, new prognostic models, current treatment of MF and discuss agents in development...
February 26, 2018: Expert Opinion on Emerging Drugs
https://www.readbyqxmd.com/read/29468276/identification-and-assessment-of-frailty-in-older-patients-with-chronic-myeloid-leukemia-and-myelofibrosis-and-indications-for-tyrosine-kinase-inhibitor-treatment
#15
REVIEW
Massimo Breccia, Francesca Palandri, Luigiana Luciano, Giulia Benevolo, Massimiliano Bonifacio, Giovanni Caocci, Fausto Castagnetti, Giuseppe A Palumbo, Alessandra Iurlo, Francesco Landi
The incidence of cancer, including myeloproliferative neoplasms (MPNs), is projected to increase significantly due to the growing proportion of people aged > 65 years. These older individuals are a heterogeneous population in terms of fitness, comorbidity, and psychological reserve. Therefore, age per se does not always provide an accurate indication of condition in patients with cancer. Frailty has been proposed as an alternative measure of vulnerability that might better indicate which patients can tolerate standard cancer treatment and those who may benefit from treatment adjustment...
February 22, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29467191/targeted-next-generation-sequencing-in-blast-phase-myeloproliferative-neoplasms
#16
Terra L Lasho, Mythri Mudireddy, Christy M Finke, Curtis A Hanson, Rhett P Ketterling, Natasha Szuber, Kebede H Begna, Mrinal M Patnaik, Naseema Gangat, Animesh Pardanani, Ayalew Tefferi
Among 248 consecutive patients with blast phase myeloproliferative neoplasm (MPN-BP), DNA collected at the time of blast transformation was available in 75 patients (median age, 66 years; 64% men). MPN-BP followed primary myelofibrosis in 39 patients, essential thrombocythemia in 20 patients, and polycythemia vera in 16 patients. A myeloid neoplasm-relevant 33-gene panel was used for next-generation sequencing. Driver mutation distribution was JAK2 57%, CALR 20%, MPL 9%, and triple-negative 13%. Sixty-four patients (85%) harbored other mutations/variants, including 37% with ≥3 mutations; most frequent were ASXL1 47%, TET2 19%, RUNX1 17%, TP53 16%, EZH2 15%, and SRSF2 13%; relative mutual exclusivity was expressed by TP53 , EZH2 , LNK , RUNX1 , SRSF2 , and NRAS/KRAS mutations...
February 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29466766/recent-advances-in-the-genomics-and-therapy-of-bcr-abl1-positive-and-negative-chronic-myeloproliferative-neoplasms
#17
REVIEW
Tariq I Mughal, Jason Gotlib, Ruben Mesa, Steffen Koschmieder, H Jean Khoury, Jorge E Cortes, Tiziano Barbui, Rüdiger Hehlmann, Michael Mauro, Susanne Saussele, Jerald P Radich, Richard A Van Etten, Giuseppe Saglio, Srdnan Verstovek, Robert Peter Gale, Omar Abdel-Wahab
This review is based on the presentations and deliberations at the 7th John Goldman Chronic Myeloid Leukemia (CML) and Myeloproliferative Neoplasms (MPN) Colloquium which took place in Estoril, Portugal on the 15th October 2017, and the 11th post-ASH International Workshop on CML and MPN which took place on the 6th-7th December 2016, immediately after the 58th American Society of Hematology Annual Meeting. Rather than present a resume of the proceedings, we have elected to address some of the topical translational research and clinically relevant topics in greater detail...
February 14, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29463446/how-to-treat-chronic-myeloid-leukemia-cml-in-older-adults
#18
Marlise R Luskin, Daniel J DeAngelo
Chronic myeloid leukemia (CML), a myeloproliferative neoplasm defined by the t(9;22)(q34;q11) chromosomal translocation, primarily affects older adults. Historically, effective treatment options were not available for older CML patients ineligible for curative allogeneic stem cell transplant, and the disease was therefore usually fatal within several years of diagnosis. The development of tyrosine kinase inhibitors (TKIs) that effectively target the constitutively active mutant tyrosine kinase in CML has dramatically improved outcomes for all patients with CML, including older patients...
February 18, 2018: Journal of Geriatric Oncology
https://www.readbyqxmd.com/read/29462765/incidence-of-haematological-malignancies-eastern-cape-province-south-africa-2004-2013
#19
Diana Oelofse, Ilse Truter
BACKGROUND: The incidence of haematological malignancies in Africa's rapidly urbanising populations is insufficiently explored. Reliable population-based cancer statistics, however, continues to be a scarce resource in Africa and tends to be urban biased with limited rural coverage. In addition, many haematological malignancies are regarded as rare cancers, a sub-group that often affects the young disproportionately and require advanced diagnostic services and facilities able to deliver costly sophisticated treatments...
February 17, 2018: Cancer Epidemiology
https://www.readbyqxmd.com/read/29455651/targeting-few-to-help-hundreds-jak-mapk-and-rock-pathways-as-druggable-targets-in-atypical-chronic-myeloid-leukemia
#20
REVIEW
Stefania Rocca, Giovanna Carrà, Pietro Poggio, Alessandro Morotti, Mara Brancaccio
Atypical Chronic Myeloid Leukemia (aCML) is a myeloproliferative neoplasm characterized by neutrophilic leukocytosis and dysgranulopoiesis. From a genetic point of view, aCML shows a heterogeneous mutational landscape with mutations affecting signal transduction proteins but also broad genetic modifiers and chromatin remodelers, making difficult to understand the molecular mechanisms causing the onset of the disease. The JAK-STAT, MAPK and ROCK pathways are known to be responsible for myeloproliferation in physiological conditions and to be aberrantly activated in myeloproliferative diseases...
February 19, 2018: Molecular Cancer
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