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leukemia myelomonocytic

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https://www.readbyqxmd.com/read/28102729/chronic-myelomonocytic-leukemia-with-double-mutations-in-dnmt3a-and-flt3-itd-treated-with-decitabine-and-sorafenib
#1
Jia Gu, Zhiqiong Wang, Min Xiao, Xia Mao, Li Zhu, Ying Wang, Wei Huang
Chronic myelomonocytic leukemia (CMML) is a heterogeneous neoplastic hematologic disorder with worse overall survival. Half of CMML have mutations, but case with concomitant mutations of DNA methyltransferase 3A (DNMT3A) and Internal tandem duplications of the juxtamembrane domain of FLT3 (FLT3-ITD) in CMML was not reported before. We reported a 51-year-old man who had CMML with concomitant mutations in DNMT3A and FLT3-ITD.The patient received decitabine and sorafenib combined treatment. In this report, we reviewed DNMT3A mutation and FLT3 mutation, and we reviewed treatment of decitabine and sorafenib...
January 19, 2017: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/28098151/juvenile-myelomonocytic-leukemia-associated-variants-are-associated-with-neo-natal-lethal-noonan-syndrome
#2
Heather Mason-Suares, Diana Toledo, Jean Gekas, Katherine A Lafferty, Naomi Meeks, M Cristina Pacheco, David Sharpe, Thomas E Mullen, Matthew S Lebo
Gain-of-function variants in some RAS-MAPK pathway genes, including PTPN11 and NRAS, are associated with RASopathies and/or acquired hematological malignancies, most notably juvenile myelomonocytic leukemia (JMML). With rare exceptions, the spectrum of germline variants causing RASopathies does not overlap with the somatic variants identified in isolated JMML. Studies comparing these variants suggest a stronger gain-of-function activity in the JMML variants. As JMML variants have not been identified as germline defects and have a greater impact on protein function, it has been speculated that they would be embryonic lethal...
January 18, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28096091/use-of-hematopoietic-cell-transplantation-for-patients-with-myelodysplastic-syndrome-and-chronic-myelomonocytic-leukemia
#3
Theo de Witte, David Bowen, Marie Robin, Luca Malcovati, Dietger Niederwieser, Ibrahim Yakoub-Agha, Ghulam J Mufti, Pierre Fenaux, Guillermo Sanz, Rodrigo Martino, Emilio Paolo Alessandrino, Francesco Onida, Argiris Symeonidis, Jakob Passweg, Guido Kobbe, Arnold Ganser, Uwe Platzbecker, Jürgen Finke, Michel van Gelder, Arjan A van de Loosdrecht, Per Ljungman, Reinhard Stauder, Liisa Volin, H Joachim Deeg, Corey Cutler, Wael Saber, Richard Champlin, Sergio Giralt, Claudio Anasetti, Nicolaus Kröger
No abstract text is available yet for this article.
January 17, 2017: Blood
https://www.readbyqxmd.com/read/28092999/second-malignancies-in-survivors-of-chronic-myelomonocytic-leukemia-a-u-s-population-based-study
#4
Mohamed Abd El-Fattah
No abstract text is available yet for this article.
January 16, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28092882/blastic-plasmacytoid-dendritic-cell-neoplasm-associated-with-chronic-myelomonocytic-leukemia
#5
Zhihong Hu, Tsieh Sun
No abstract text is available yet for this article.
September 22, 2016: Blood
https://www.readbyqxmd.com/read/28090486/therapy-related-myeloid-neoplasms-in-children-and-adolescents
#6
Hee Won Cho, Young Bae Choi, Eun Sang Yi, Ji Won Lee, Ki Woong Sung, Hong Hoe Koo, Keon Hee Yoo
BACKGROUND: This retrospective study aimed to characterize and analyze the outcome of therapy-related myeloid neoplasms (t-MNs) in children and adolescents. METHODS: The medical records of 16 patients under 21 years of age at the time of t-MN diagnosis were reviewed. RESULTS: The median patient age was 11.5 years (range, 1.6-20.4 yr). Twelve patients had therapy-related acute myeloid leukemia, 3 patients had myelodysplastic syndrome, and 1 patient had chronic myelomonocytic leukemia...
December 2016: Blood Research
https://www.readbyqxmd.com/read/28027687/asxl1-mutations-in-myeloid-neoplasms-pathogenetic-considerations-impact-on-clinical-outcomes-and-survival
#7
Juliana Alvarez Argote, Constantin Dasanu
BACKGROUND: ASXL1 gene mutations include nonsense, missense, and frameshift mutations. Although their clinical significance is still debated, they may play an important role in the pathogenesis of several hematologic malignancies. METHODS: Herein, we offer a comprehensive review on ASXL1 mutations, and link them with survival and clinical outcomes in patients with various myeloid neoplasms. Most relevant publications were identified through searching the PubMed/Medline database for articles published from inception to February 2016...
December 27, 2016: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28001311/extreme-lymphocytosis-with-myelomonocytic-morphology-in-a-horse-with-diffuse-large-b-cell-lymphoma
#8
Kristina Meichner, Blaire H Kraszeski, Jessica R Durrant, Carol B Grindem, Babetta A Breuhaus, Peter F Moore, Jennifer A Neel, Keith E Linder, Luke B Borst, Jonathan E Fogle, Jaime L Tarigo
An 11-year-old, 443-kg Haflinger mare was presented to the North Carolina State University Veterinary Teaching Hospital with a 2-week history of lethargy and a 3-day duration of anorexia, pyrexia, tachycardia, and ventral edema. Severe pitting edema, peripheral lymphadenopathy, and a caudal abdominal mass were noted on physical examination. An extreme leukocytosis (154.3 × 10(3) /μL) and microscopic hematologic findings suggestive of myelomonocytic leukemia were observed. Serum protein electrophoresis revealed a monoclonal gammopathy and urine protein electrophoresis revealed a monoclonal light chain proteinuria...
December 21, 2016: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/27988409/cerebral-rhizomucor-infection-treated-by-posaconazole-delayed-release-tablets-in-an-allogeneic-stem-cell-transplant-recipient
#9
Diego O Andrey, Laurent Kaiser, Stéphane Emonet, Veronique Erard, Yves Chalandon, Christian van Delden
Mucormycosis (zygomycosis) is an emerging fungal disease in allogeneic hematopoietic stem cell transplant (allo-HSCT) recipients. A 30-year-old woman diagnosed with acute myelomonocytic leukemia and needing allo-HSCT presented pulmonary and cerebral infection due to Rhizomucor pusillus. This fungal infection was treated with surgical treatment and posaconazole delayed-release tablets. This strategy allowed reaching high drug levels that could not be obtained with the posaconazole solution.
December 14, 2016: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/27981789/comparison-of-abnormal-cell-flagging-of-the-hematology-analyzers-sysmex-xn-and-sysmex-xe-5000-in-oncohematologic-patients
#10
J R Furundarena, M Sainz, A Uranga, L Cuevas, I Lopez, J Zubicaray, A Bizjak, N Robado, M Araiz
INTRODUCTION: Hematology analyzers should optimize flagging while minimizing false-negative results and unnecessary microscopic reviews. METHODS: We compared flagging performance of Sysmex XE-5000 and XN analyzers in oncohematologic patients. Differential counts were performed by Cellavision digital system (100 cells) and a hematologist (another 100 cells). RESULTS: First, we included 292 samples (86 with blasts): 28 acute lymphoblastic leukemia, 88 acute myeloid leukemia, 91 myelodysplastic syndromes, 45 chronic myeloproliferative neoplasms, and 40 chronic myelomonocytic leukemia...
December 16, 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27967252/hyperleukocytosis-and-leukostasis-management-of-a-medical-emergency
#11
Sabrina Giammarco, Patrizia Chiusolo, Nicola Piccirillo, Alessia Di Giovanni, Elisabetta Metafuni, Luca Laurenti, Simona Sica, Livio Pagano
Hyperleukocytosis is defined as a white blood cell count greater than 100,000/mL in patients affected by acute leukemia and often it is associated with increased morbidity and mortality, that can be up to 40% if unrecognized. Areas covered: Risk factors include younger age, myelomonocytic or monocytic/monoblastic morphology, microgranular variant of acute promyelocitic leukemia and T-cell ALL, and some cytogenetic abnormalities. Poor prognosis due to high early death rate secondary to leukostasis. The mechanisms at the origin of leukostasis are still poorly understood...
December 26, 2016: Expert Review of Hematology
https://www.readbyqxmd.com/read/27958379/a-systems-biology-approach-to-investigate-the-mechanism-of-action-of-trabectedin-in-a-model-of-myelomonocytic-leukemia
#12
L Mannarino, L Paracchini, I Craparotta, M Romano, S Marchini, R Gatta, E Erba, L Clivio, C Romualdi, M D'Incalci, L Beltrame, L Pattini
This study was designed to investigate the mode of action of trabectedin in myelomonocytic leukemia cells by applying systems biology approaches to mine gene expression profiling data and pharmacological assessment of the cellular effects. Significant enrichment was found in regulons of target genes inferred for specific transcription factors, among which MAFB was the most upregulated after treatment and was central in the transcriptional network likely to be relevant for the specific therapeutic effects of trabectedin against myelomonocytic cells...
December 13, 2016: Pharmacogenomics Journal
https://www.readbyqxmd.com/read/27913534/myelodysplastic-and-myeloproliferative-disorders-of-childhood
#13
Henrik Hasle
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27910026/recurrent-cytogenetic-abnormalities-in-myelodysplastic-syndromes
#14
Meaghan Wall
Cytogenetic analysis has an essential role in diagnosis, classification, and prognosis of myelodysplastic syndromes (MDS). Some cytogenetic abnormalities are sufficiently characteristic of MDS to be considered MDS defining in the appropriate clinical context. MDS with isolated del(5q) is the only molecularly defined MDS subtype. The genes responsible for many aspects of 5q- syndrome, the distinct clinical phenotype associated with this condition, have now been identified. Cytogenetics forms the cornerstone of the most widely adopted prognostic scoring systems in MDS, the international prognostic scoring system (IPSS) and the revised international prognostic scoring system (IPPS-R)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27895769/unusual-lower-back-pain-with-monocytosis-a-case-report
#15
Sheng-Cheng Wu, Tzu-Chuan Huang, Wen-Ya Yu, Yi-Ying Wu
There are numerous causes of lower back pain. In the oncological setting, spine metastasis from a solid tumor is the most common. However, hematological disorders should also be taken into consideration. The current study presents a case of chronic myelomonocytic leukemia with the initial presentation of chronic lower back pain, followed by symptoms that included urinary retention, stool incontinence and left gum swelling, in a patient who was eventually diagnosed with granulocytic sarcoma (GS) over the sacral region...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27881041/incidence-and-survival-outcomes-of-chronic-myelomonocytic-leukemia-in-the-united-states
#16
Guru Subramanian Guru Murthy, Ishwori Dhakal, Paulette Mehta
Chronic myelomonocytic leukemia (CMML) is an aggressive neoplasm with sparse data on outcomes at a population level. Using Surveillance Epidemiology and End Results (SEER) database, we identified 2238 patients with CMML diagnosed in the period 2003-2013. We found that the disease incidence was significantly higher with advancing age and lower in females, Blacks, and Asian/pacific islanders. Median OS declined significantly with increasing age (age 20-39 - 25 months, age 40-59 - 20 months, age 60-79 - 18 months, and age ≥80 - 11 months, p < ...
November 23, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27863755/identification-of-the-myst3-crebbp-fusion-gene-in-infants-with-acute-myeloid-leukemia-and-hemophagocytosis
#17
Francianne Gomes Andrade, Elda Pereira Noronha, Rosania Maria Baseggio, Teresa Cristina Cardoso Fonseca, Bruno Marcelo Rocha Freire, Isis M Quezado Magalhaes, Ilana R Zalcberg, Maria S Pombo-de-Oliveira
BACKGROUND: Acute myeloid leukemia presenting the MYST3-CREBBP fusion gene is a rare subgroup associated with hemophagocytosis in early infancy and monocytic differentiation. The aim of this study was to define the relevant molecular cytogenetic characteristics of a unique series of early infancy acute myeloid leukemia cases (≤24months old), based on the presence of hemophagocytosis by blast cells at diagnosis. METHODS: A series of 266 infant cases of acute myeloid leukemia was the reference cohort for the present analysis...
October 2016: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/27859216/cooperation-of-mll-af10-om-lz-with-ptpn11-activating-mutation-induced-monocytic-leukemia-with-a-shorter-latency-in-a-mouse-bone-marrow-transplantation-model
#18
Jen-Fen Fu, Sung-Tzu Liang, Ying-Jung Huang, Kung-Hao Liang, Tzung-Hai Yen, Der-Cherng Liang, Lee-Yung Shih
PTPN11 mutation, a RAS signaling pathway mutation, is associated with MLL translocations in acute leukemia. A girl with MLL/AF10 AML was found to carry PTPN11(G503A) . To study the impact of PTPN11(G503A) cooperating with MLL/AF10 on leukemogenesis, we established a retroviral transduction/transplantation mouse model. Compared to the MLL/AF10(OM-LZ) leukemia cells harboring PTPN11(wt) , the cells harboring PTPN11(G503A) were hypersensitive to GM-CSF and IL3, and more resistant to death upon treatment with daunorubicin but sensitive to cytarabine...
March 1, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/27852053/therapy-related-acute-myeloid-leukemia-following-treatment-of-lymphoid-malignancies
#19
Sarah Bertoli, Arthur Sterin, Suzanne Tavitian, Lucie Oberic, Loïc Ysebaert, Reda Bouabdallah, François Vergez, Audrey Sarry, Emilie Bérard, Françoise Huguet, Guy Laurent, Thomas Prébet, Norbert Vey, Christian Récher
Therapy-related acute myeloid leukemia (t-AML) is a heterogeneous entity most frequently related to breast cancer or lymphoproliferative diseases (LD). Population-based studies have reported an increased risk of t-AML after treatment of lymphomas. The aim of this study was to describe the characteristics and outcome of 80 consecutive cases of t-AML following treatment of LD. t-AML accounted for 2.3% of all AML cases, occurred 60 months after LD diagnosis, and were characterized by a high frequency of FAB M6 AML and poor-risk cytogenetic abnormalities...
November 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27849645/current-management-of-patients-with-chronic-myelomonocytic-leukemia
#20
Ana Alfonso, Guillermo Montalban-Bravo, Guillermo Garcia-Manero
PURPOSE OF REVIEW: The present review will focus on the current management of patients with chronic myelomonocytic leukemia (CMML) as well as in future therapeutic perspectives. RECENT FINDINGS: CMML is a clonal hematopoietic stem cell disorder characterized by peripheral blood monocytosis and myelodysplastic and myeloproliferative alterations in the bone marrow. Clinical behavior of the disease can be heterogeneous, with some patients having an indolent form of the disease, whereas others experience an aggressive course with decreased survival and eventual transformation to leukemia...
January 2017: Current Opinion in Oncology
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