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Chronic myeloproliferative neoplasm

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https://www.readbyqxmd.com/read/29040257/current-challenges-in-the-management-of-essential-thrombocythemia
#1
Ariel Kleman, Arun K Singavi, Laura C Michaelis
Essential thrombocythemia (ET), an uncommon blood cancer, is one of the classic myeloproliferative neoplasms, a category that also includes polycythemia vera and primary myelofibrosis. All 3 diseases are clonal hematopoietic stem cell disorders. Since 2005, when scientists discovered a molecular aberration driving clonal hematopoiesis in polycythemia vera, our understanding of the genomic underpinnings of these conditions has increased rapidly. Over the last decades, primary prevention of thrombotic and hemorrhagic complications has improved the lives of patients with ET, and the ability to characterize the disease by the presence or absence of molecular mutations has lent precision to our prognostic models...
October 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29027642/familial-mpn-predisposition
#2
REVIEW
Tsewang Tashi, Sabina Swierczek, Josef T Prchal
Chronic myeloproliferative neoplasms (MPN) characteristically arise from a somatic mutation in the pluripotent hematopoietic stem cell, and most common recurring mutations are in the JAK2, CALR, and cMPL genes. However, these mutations are not founder mutations, but mainly drive the disease phenotype and a pre-existing germline predisposition has been long speculated, but has not been clearly defined to date. Genome-wide association studies in family clusters of MPN have identified a number of genetic variants that are associated with increased germline risk for developing clonal MPN...
October 13, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/29027146/correction-to-jsh-guideline-for-tumors-of-hematopoietic-and-lymphoid-tissues-lukemia-4-chronic-myelogenous-leukemia-cml-myeloproliferative-neoplasms-mpn
#3
Noriko Usui
In the original publication of this article, the "Acknowledgements" was not included and it is given below for your reading.
October 12, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29025582/a-novel-trip11-flt3-fusion-in-a-patient-with-a-myeloid-lymphoid-neoplasm-with-eosinophilia
#4
Alfred Chung, Yanli Hou, Robert S Ohgami, Ann Von Gehr, Dianna G Fisk, Krishna M Roskin, Xu Li, Linda Gojenola, Charles D Bangs, Daniel A Arber, Andrew Z Fire, Athena M Cherry, James L Zehnder, Jason Gotlib, Jason D Merker
FLT3 fusions are associated with myeloid and lymphoid neoplasms with eosinophilia. We describe a patient presenting with clinicopathologic features of both chronic eosinophilic leukemia, not otherwise specified (CEL, NOS) and systemic mastocytosis (SM). The bone marrow demonstrated a myeloproliferative neoplasm with eosinophilia and aggregates of atypical mast cells. Cytogenetic analysis revealed a t(13;14)(q12;q32), which was subsequently molecularly characterized as a novel TRIP11-FLT3 rearrangement. A KIT D816V mutation was also identified...
October 2017: Cancer Genetics
https://www.readbyqxmd.com/read/28991281/trombosis-venosa-espl%C3%A3-cnica-como-manifestaci%C3%A3-n-inicial-de-mielofibrosis-primaria
#5
Gregorio Campos-Cabrera, Virginia Campos-Cabrera, Salvador Campos-Cabrera, José-Luis Campos-Villagómez, Alejandra Romero-González
other: Las neoplasias mieloproliferativas (NMP) son alteraciones crónicas de las células madre hematopoyéticas clonales caracterizadas por una mayor producción de granulocitos, glóbulos rojos o plaquetas. Una de las principales complicaciones de las NMP es la aparición de problemas trombóticos venosos y arteriales causados por un aumento en la agregación plaquetaria y la generación de trombina. Se evaluaron 11 casos de mielofibrosis primaria (MP), de los cuales dos debutaron con trombosis venosa esplácnica (TVE)...
2017: Revista de Investigación Clínica; Organo del Hospital de Enfermedades de la Nutrición
https://www.readbyqxmd.com/read/28988389/treatment-free-remission-a-new-therapeutic-goal-in-chronic-myelogenous-leukemia
#6
REVIEW
Ahmed Gamal Elsayed, Roma Srivastava, Muhammad Omer Jamil
PURPOSE OF REVIEW: Chronic myelogenous leukemia (CML) is a chronic myeloproliferative neoplasm characterized by the presence of Philadelphia chromosome [t(9:22)] leading to the presence of pathognomonic fusion gene product, BCR-ABL1. This leads to constitutive activation of ABL1 kinase. CML was a difficult-to-treat illness until the advent of small molecule tyrosine kinase inhibitor (TKI), imatinib which revolutionized therapy of CML. Since then, multiple second- and third-generation TKIs have been formulated which have proven effective and has led to marked improvement in survival...
October 7, 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28983777/therapy-for-chronic-myelomonocytic-leukemia-in-a-new-era
#7
REVIEW
Tamara K Moyo, Michael R Savona
Chronic myelomonocytic leukemia (CMML) is a myeloid malignancy which shares clinical and morphologic features of myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPNs) and is classified by the WHO as an MDS/MPN. The defining feature of CMML is clonal hematopoiesis that results in peripheral monocytosis. The benefit of early treatment is currently unclear, and treatment may be held until the disease exhibits accelerated blast counts or the patient becomes symptomatic. Optimal treatments for CMML are not well defined...
October 6, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28949941/polycythemia-vera-from-new-modified-diagnostic-criteria-to-new-therapeutic-approaches
#8
Margherita Maffioli, Barbara Mora, Francesco Passamonti
Polycythemia vera (PV) is a Philadelphia chromosome-negative chronic myeloproliferative neoplasm that is associated with a Janus kinase 2 (JAK2) mutation in most cases. The most recent update to the World Health Organization diagnostic criteria for PV was published in 2016. These were the modifications with the greatest effect: (1) lowering the hemoglobin threshold, allowing a diagnosis of PV at 16.5 g/dL in males and at 16.0 g/dL in females and (2) introducing a hematocrit cutoff (49% in males and 48% in females)...
September 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/28942516/approach-to-mpn-symptom-assessment
#9
REVIEW
Holly Geyer, Ruben A Mesa
PURPOSE OF REVIEW: Myeloproliferative neoplasms (MPNs) are recognized for their debilitating symptom burdens. The purpose of this review is to understand the complexity of the MPN symptom burden and identify how validated MPN Patient Reported Outcome (PRO) tools may be integrated into clinical practice to assess the MPN symptom burden. RECENT FINDINGS: Significant heterogeneity exists both within and between MPN subtypes. Surrogates of disease burden such as risk scores and MPN chronicity often fail to correlate with symptomatic burden...
September 23, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28936634/jsh-guideline-for-tumors-of-hematopoietic-and-lymphoid-tissues-lukemia-4-chronic-myelogenous-leukemia-cml-myeloproliferative-neoplasms-mpn
#10
Noriko Usui
No abstract text is available yet for this article.
September 21, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28935846/a-t-1-9-translocation-involving-csf3r-as-a-novel-mechanism-in-unclassifiable-chronic-myeloproliferative-neoplasm
#11
Jesús Gutiérrez-Abril, Iñigo Santamaría, Ana S Pitiot, Ana Gutiérrez-Fernández, Ángel Alvarez-Eguiluz, José M Vicente, Carmen Sanzo, Soledad González-Muñiz, Milagros Balbín, Xose S Puente
No abstract text is available yet for this article.
September 21, 2017: Haematologica
https://www.readbyqxmd.com/read/28927137/atypical-chronic-myeloid-leukemia-with-isochromosome-x-p10-a-case-report
#12
Masahide Yamamoto, Sayaka Suzuki, Jun-Ichi Mukae, Keisuke Tanaka, Ken Watanabe, Gaku Oshikawa, Tetsuya Fukuda, Naomi Murakami, Osamu Miura
Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/myeloproliferative neoplasm (MDS/MPN). Although recurrent chromosomal and genetic abnormalities are frequently observed in aCML, none are specific to this type of leukemia. The present study reported a case of aCML associated with i(X)(p10), a rare recurrent chromosomal abnormality of hematological malignancy. A 40-year-old female was referred to the Tokyo Medical and Dental University Hospital (Tokyo, Japan) due to slight leukocytosis and anemia...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28885734/chronic-myelomonocytic-leukemia-masquerading-as-cutaneous-indeterminate-dendritic-cell-tumor-expanding-the-spectrum-of-skin-lesions-in-chronic-myelomonocytic-leukemia
#13
Sanam Loghavi, Jonathan L Curry, Guillermo Garcia-Manero, Keyur P Patel, Jie Xu, Joseph D Khoury, Carlos A Torres-Cabala, Priyadharsini Nagarajan, Phyu P Aung, Bernard R Gibson, Brandon P Goodwin, Brent C Kelly, Brinda R Korivi, L Jeffrey Medeiros, Victor G Prieto, Hagop M Kantarjian, Carlos E Bueso-Ramos, Michael T Tetzlaff
Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions...
September 8, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28885361/angioimmunoblastic-t-cell-lymphoma-and-hypereosinophilic-syndrome-with-fip1l1-pdgfra-fusion-gene-effectively-treated-with-imatinib-a-case-report
#14
Masayo Yamamoto, Katsuya Ikuta, Yasumichi Toki, Mayumi Hatayama, Motohiro Shindo, Yoshihiro Torimoto, Toshikatsu Okumura
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28881484/primary-cells-in-bcr-fgfr1-positive-8p11-myeloproliferative-syndrome-are-sensitive-to-dovitinib-ponatinib-and-dasatinib
#15
Niklas Landberg, Arta Dreimane, Marianne Rissler, Rolf Billström, Helena Ågerstam
OBJECTIVES: Translocations involving the fibroblast growth factor receptor 1 (FGFR1) gene are associated with the 8p11 myeloproliferative syndrome (EMS), a rare neoplasm that following a usually short chronic phase progresses into acute myeloid or lymphoid leukemia. The treatment commonly involves chemotherapy and, if possible, allogeneic stem cell transplantation which is the only therapeutic option for long-term survival. Given the aggressive course of EMS, we here evaluated tyrosine kinase inhibitors as treatment options to delay disease progression...
September 7, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28869184/the-treatment-landscape-of-myelofibrosis-before-and-after-ruxolitinib-approval
#16
Andrew T Kuykendall, Chetasi Talati, Najla Al Ali, Kendra Sweet, Eric Padron, David A Sallman, Jeffrey E Lancet, Alan F List, Kenneth S Zuckerman, Rami S Komrokji
INTRODUCTION/BACKGROUND: Myelofibrosis (MF) is a chronic myeloproliferative neoplasm that presents with a heterogeneous clinical phenotype and prognosis. Before the US Food and Drug Administration approval of ruxolitinib, treatment options were varied and had limited effect. The increased use of ruxolitinib has drastically altered the MF treatment landscape. In this study, we aimed to clarify the clinical situations in which ruxolitinib is being used and analyze its effect on this landscape...
August 5, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28859112/mathematical-modelling-as-a-proof-of-concept-for-mpns-as-a-human-inflammation-model-for-cancer-development
#17
Morten Andersen, Zamra Sajid, Rasmus K Pedersen, Johanne Gudmand-Hoeyer, Christina Ellervik, Vibe Skov, Lasse Kjær, Niels Pallisgaard, Torben A Kruse, Mads Thomassen, Jesper Troelsen, Hans Carl Hasselbalch, Johnny T Ottesen
The chronic Philadelphia-negative myeloproliferative neoplasms (MPNs) are acquired stem cell neoplasms which ultimately may transform to acute myelogenous leukemia. Most recently, chronic inflammation has been described as an important factor for the development and progression of MPNs in the biological continuum from early cancer stage to the advanced myelofibrosis stage, the MPNs being described as "A Human Inflammation Model for Cancer Development". This novel concept has been built upon clinical, experimental, genomic, immunological and not least epidemiological studies...
2017: PloS One
https://www.readbyqxmd.com/read/28804122/shp2-is-required-for-bcr-abl1-induced-hematologic-neoplasia
#18
S Gu, A Sayad, G Chan, W Yang, Z Lu, C Virtanen, R A Van Etten, B G Neel
BCR-ABL1-targeting tyrosine kinase inhibitors (TKIs) have revolutionized treatment of Philadelphia chromosome-positive (Ph(+)) hematologic neoplasms. Nevertheless, acquired TKI resistance remains a major problem in chronic myeloid leukemia (CML), and TKIs are less effective against Ph(+) B-cell acute lymphoblastic leukemia (B-ALL). GAB2, a scaffolding adaptor that binds and activates SHP2, is essential for leukemogenesis by BCR-ABL1, and a GAB2 mutant lacking SHP2 binding cannot mediate leukemogenesis. Using a genetic loss-of-function approach and bone marrow transplantation models for CML and BCR-ABL1(+) B-ALL, we show that SHP2 is required for BCR-ABL1-evoked myeloid and lymphoid neoplasia...
August 14, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28774880/randomized-phase-2-study-of-low-dose-decitabine-vs-low-dose-azacitidine-in-lower-risk-mds-and-mds-mpn
#19
Elias Jabbour, Nicholas J Short, Guillermo Montalban-Bravo, Xuelin Huang, Carlos Bueso-Ramos, Wei Qiao, Hui Yang, Chong Zhao, Tapan Kadia, Gautam Borthakur, Naveen Pemmaraju, Koji Sasaki, Zeev Estrov, Jorge Cortes, Farhad Ravandi, Yesid Alvarado, Rami Komrokji, Mikkael A Sekeres, David P Steensma, Amy DeZern, Gail Roboz, Hagop Kantarjian, Guillermo Garcia-Manero
Hypomethylating agents (HMAs) improve survival in patients with higher-risk myelodysplastic syndromes (MDS) but are less well-studied in lower-risk disease. We compared the safety and efficacy of low-dose decitabine vs low-dose azacitidine in this group of patients. Adults with low- or intermediate 1-risk MDS or MDS/myeloproliferative neoplasm (MPN), including chronic myelomonocytic leukemia, according to the International Prognostic Scoring System, were randomly assigned using a Bayesian adaptive design to receive either azacitidine 75 mg/m(2) intravenously/subcutaneously daily or decitabine 20 mg/m(2) intravenously daily for 3 consecutive days on a 28-day cycle...
September 28, 2017: Blood
https://www.readbyqxmd.com/read/28770949/treatment-of-patients-with-primary-myelofibrosis-using-dasatinib
#20
Q-L Song, B Zhang, Y Xu, R-X Xia, X-H Lu, Z-X Pei, Q-W Xu, W-Y Li, Z-D Li
OBJECTIVE: Primary myelofibrosis (PMF) is a chronic clonal myeloproliferative neoplasm. It is associated with a poor prognosis, with a median survival time of approximately five years. Thus far, there are no specific targeted drugs for PMF. In this study, we evaluated the efficacy and safety of dasatinib, a second-generation tyrosine kinase inhibitor, in six PMF patients. PATIENTS AND METHODS: From June 1, 2015 to February 29, 2016, six patients with PMF in our department were enrolled into this trial...
July 2017: European Review for Medical and Pharmacological Sciences
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