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https://www.readbyqxmd.com/read/29754885/langerhans-cell-histiocytosis-in-children-history-classification-pathobiology-clinical-manifestations-and-prognosis
#1
REVIEW
Jolie Krooks, Milen Minkov, Angela G Weatherall
Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Clinical manifestations range from isolated disease with spontaneous resolution to life-threatening multisystem disease...
June 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29728108/decitabine-demonstrates-antileukemic-activity-in-b-cell-precursor-acute-lymphoblastic-leukemia-with-mll-rearrangements
#2
C Roolf, A Richter, C Konkolefski, G Knuebel, A Sekora, S Krohn, J Stenzel, B J Krause, B Vollmar, H Murua Escobar, C Junghanss
BACKGROUND: Promotor hypermethylation of CpG islands is common in B cell precursor acute lymphoblastic leukemia (BCP-ALL) with mixed lineage leukemia (MLL) gene rearrangements. Hypomethylating agents (HMA) such as azacitidine (AZA) and decitabine (DEC) reduce DNA hypermethylation by incorporation into DNA and were successfully introduced into the clinic for the treatment of myeloid neoplasias. METHODS: Here, we investigated whether HMA induce comparable biological effects in MLL-positive BCP-ALL...
May 4, 2018: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/29726055/dogs-with-acute-myeloid-leukemia-or-lymphoid-neoplasms-large-cell-lymphoma-or-acute-lymphoblastic-leukemia-may-have-indistinguishable-mediastinal-masses-on-radiographs
#3
Erin Epperly, Kelly R Hume, Steven Moirano, Tracy Stokol, Joanne Intile, Hollis N Erb, Peter V Scrivani
Acute myeloid leukemia is an uncommon hematopoietic neoplasm of dogs that should be differentiated from lymphoid neoplasms, such as lymphoma, because of different treatment protocols and a worse prognosis. Thoracic radiography is performed frequently in dogs with suspected hematopoietic neoplasia, and detecting a mediastinal mass often prioritizes lymphoma as the most likely diagnosis. However, we have observed a mediastinal mass in several dogs with acute myeloid leukemia and hypothesized that (1) the frequency of a mediastinal mass was higher and (2) the size of the mass was larger in dogs with acute myeloid leukemia compared to dogs with lymphoid neoplasms...
May 3, 2018: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/29703819/new-insights-from-studies-of-clonal-hematopoiesis
#4
Christopher J Gibson, David P Steensma
Clonal hematopoiesis (CH) describes an asymptomatic expansion of blood cells descended from a single hematopoietic stem cell. Recent studies have shown that CH increases in frequency with aging, and is often driven by somatic mutations in genes that are recurrently mutated in hematologic malignancies. When CH is associated with a mutation in a leukemia-associated gene at a variant allele frequency of 0.02 or greater, it is termed "clonal hematopoiesis of indeterminate potential" (CHIP).  CHIP has a 0...
April 27, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29700805/pathogenetic-implication-of-fusion-genes-in-acute-promyelocytic-leukemia-apl-and-their-diagnostic-utility
#5
REVIEW
S M Baba, A A Pandith, Z A Shah, R A Baba
Acute promyelocytic leukemia (APL) is a class of myeloid neoplasias characterized by immature leukemic promyelocytes in the bone marrow and peripheral blood. It was described in 1957 by Leif Hillestad and the main pathogenetic event of the disease is the emergence of hybrid fusion gene PML-RARα, which results from translocation between chromosomes 15 and 171 .
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29699374/inhibitory-effects-of-arsenic-trioxide-and-thalidomide-on-angiogenesis-and-vascular-endothelial-growth-factor-expression-in-leukemia-cells
#6
Mahnaz Mohammadi Kian, Saeed Mohammadi, Mahmoud Tavallaei, Bahram Chahardouli, Saharbano Rostami, Mahdi Zahedpanah, Ardeshir Ghavamzadeh, Mohsen Nikbakht
Acute myeloid leukemia (AML) is a blood disorder characterized by uncontrolled proliferation of myeloid progenitors and decrease in the apoptosis rate. The vascular endothelial growth factor (VEGF) promotes blood vessel regeneration which might play important roles in development and progression of neoplasia. Our previous studies focused on cytotoxicity and anticancer effects of arsenic trioxide (ATO) and thalidomide (THAL) as an anti-VEGF compound in the AML cell model. ATO also affects regulatory genes involved in cell proliferation and apoptosis...
April 27, 2018: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/29560286/-pan3-psma2-fusion-resulting-from-a-novel-t-7-13-p14-q12-chromosome-translocation-in-a-myelodysplastic-syndrome-that-evolved-into-acute-myeloid-leukemia
#7
Ioannis Panagopoulos, Ludmila Gorunova, Hege Kilen Andersen, Astrid Bergrem, Anders Dahm, Kristin Andersen, Francesca Micci, Sverre Heim
Background: Acquired primary chromosomal changes in cancer are sometimes found as sole karyotypic abnormalities. They are specifically associated with particular types of neoplasia, essential in establishing the neoplasm, and they often lead to the generation of chimeric genes of pathogenetic, diagnostic, and prognostic importance. Thus, the report of new primary cancer-specific chromosomal aberrations is not only of scientific but also potentially of clinical interest, as is the detection of their gene-level consequences...
2018: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/29527921/a-blood-pact-the-significance-and-implications-of-eif4e-on-lymphocytic-leukemia
#8
V Venturi, T Masek, M Pospisek
Elevated levels of eukaryotic initiation factor 4E (eIF4E) are implicated in neoplasia, with cumulative evidence pointing to its role in the etiopathogenesis of hematological diseases. As a node of convergence for several oncogenic signaling pathways, eIF4E has attracted a great deal of interest from biologists and clinicians whose efforts have been targeting this translation factor and its biological circuits in the battle against leukemia. The role of eIF4E in myeloid leukemia has been ascertained and drugs targeting its functions have found their place in clinical trials...
March 12, 2018: Physiological Research
https://www.readbyqxmd.com/read/29518763/medullary-thyroid-cancer-leukemia-mesothelioma-and-meningioma-associated-with-germline-apc-and-rasal1-variants-a-new-syndrome
#9
Anna Angelousi, Nikolaos Settas, Fabio R Faucz, Charalampos Lyssikatos, Martha Quezado, Narjes Nasiri-Ansari, Constantine A Stratakis, Eva Kassi
Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor hereditary in 35% of cases. The most common syndromic form is in the context of the multiple endocrine neoplasia type 2 (MEN 2) syndromes in association with other tumors and due to germline RET mutations. We describe a 57-year-old female patient diagnosed with sporadic MTC. The patient had a history of other neoplasias, such as acute myeloid leukemia, for which she had received chemotherapy, and two other solid tumors, peritoneal mesothelioma and meningioma...
October 2017: Hormones: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29487200/interleukin-30-il27p28-shapes-prostate-cancer-stem-like-cell-behavior-and-is-critical-for-tumor-onset-and-metastasization
#10
Carlo Sorrentino, Stefania L Ciummo, Giuseppe Cipollone, Sara Caputo, Matteo Bellone, Emma Di Carlo
Prostate cancer stem-like cells (PCSLC) are believed to be responsible for prostate cancer onset and metastasis. Autocrine and microenvironmental signals dictate PCSLC behavior and patient outcome. In prostate cancer patients, IL30/IL27p28 has been linked with tumor progression, but the mechanisms underlying this link remain mostly elusive. Here, we asked whether IL30 may favor prostate cancer progression by conditioning PCSLCs and assessed the value of blocking IL30 to suppress tumor growth. IL30 was produced by PCSLCs in human and murine prostatic intraepithelial neoplasia and displayed significant autocrine and paracrine effects...
February 27, 2018: Cancer Research
https://www.readbyqxmd.com/read/29455318/expression-of-terminal-deoxynucleotidyl-transferase-tdt-in-classical-seminoma-a-potential-diagnostic-pitfall
#11
Alexander Brobeil, Florian Wagenlehner, Stefan Gattenlöhner
Seminomas are the most frequent testicular tumors and in spite of specific markers some histological subtypes can be diagnostically challenging due to the potential overlap of morphologic features and a variant antigen expression. Terminal deoxynucleotidyl transferase (TdT) is a DNA polymerase present in hematogones, thymic T cells, lymphoblastic lymphoma/leukemia (LBL), and in some cases of acute myeloid leukemia but so far has not been described to be expressed in seminomas. After observing a reactivity of TdT in one case of seminoma, we analyzed ten additional tumors by immunohistochemistry to determine their spectrum of reactivity for TdT...
March 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29355432/an-update-on-the-treatment-of-pediatric-onset-langerhans-cell-histiocytosis-through-pharmacotherapy
#12
REVIEW
Milen Minkov
Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasia driven by activation mutations alongside the MAPK pathway. Its broad spectrum of clinical manifestations and diverse course and clinical outcome, make interpretation of trial data difficult. Use of different stratification systems further complicates comparison among trials. Areas covered: This review summarizes the published data derived from prospective clinical trials from Phase II onwards. PubMed was searched using combinations of the keywords 'Langerhans cell histiocytosis', 'histiocytosis X', 'pediatric', 'children', 'treatment', and 'therapy'...
February 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29311715/the-bone-marrow-niche-in-mds-and-mgus-implications-for-aml-and-mm
#13
REVIEW
Irene M Ghobrial, Alexandre Detappe, Kenneth C Anderson, David P Steensma
Several haematological malignancies, including multiple myeloma (MM) and acute myeloid leukaemia (AML), have well-defined precursor states that precede the development of overt cancer. MM is almost always preceded by monoclonal gammopathy of undetermined significance (MGUS), and at least a quarter of all patients with myelodysplastic syndromes (MDS) have disease that evolves into AML. In turn, MDS are frequently anteceded by clonal haematopoiesis of indeterminate potential (CHIP). The acquisition of additional genetic and epigenetic alterations over time clearly influences the increasingly unstable and aggressive behaviour of neoplastic haematopoietic clones; however, perturbations in the bone-marrow microenvironment are increasingly recognized to have key roles in initiating and supporting oncogenesis...
April 2018: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/29132164/mutational-spectrum-of-fanconi-anemia-associated-myeloid-neoplasms
#14
Mwe Mwe Chao, Kathrin Thomay, Gudrun Goehring, Marcin Wlodarski, Victor Pastor, Brigitte Schlegelberger, Detlev Schindler, Christian Peter Kratz, Charlotte Niemeyer
Individuals with Fanconi anemia (FA) have a high risk of developing myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML), yet the secondary somatic mutations lending to these malignancies remain to be further elucidated. We employed a next-generation sequencing myeloid neoplasia gene panel to determine the mutational spectrum of FA-related MDS/AML. Ten of 16 patients showed missense, nonsense, insertion or duplication mutations in 13 genes. In contrast to findings in MDS in the general population, mutations in genes involved in RNA splicing were rarely affected...
November 2017: Klinische Pädiatrie
https://www.readbyqxmd.com/read/29123956/mutations-in-jak2-and-calreticulin-genes-are-associated-with-specific-alterations-of-the-immune-system-in-myelofibrosis
#15
Marco Romano, Daria Sollazzo, Sara Trabanelli, Martina Barone, Nicola Polverelli, Margherita Perricone, Dorian Forte, Simona Luatti, Michele Cavo, Nicola Vianelli, Camilla Jandus, Francesca Palandri, Lucia Catani
Myelofibrosis (MF) is a clonal neoplasia associated with chronic inflammation due to aberrant cytokine production. Mutations in Janus Kinase-2 (JAK2), calreticulin (CALR) and myeloproliferative leukemia protein (MPL) genes have been recently associated to MF and they all activate the JAK/STAT signaling pathway. Since this pathway is essential in shaping the immune response, we investigated the role of circulating immune subsets and cytokines in 38 patients (20 carrying JAK2(V617F) ,13 exon-9 CALR mutation and 5 triple negative)...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/29108292/azacitidine-or-intensive-chemotherapy-for-older-patients-with-secondary-or-therapy-related-acute-myeloid-leukemia
#16
Pierre-Yves Dumas, Sarah Bertoli, Emilie Bérard, Clémence Médiavilla, Edwige Yon, Suzanne Tavitian, Thibaut Leguay, Françoise Huguet, Edouard Forcade, Noël Milpied, Audrey Sarry, Mathieu Sauvezie, Pierre Bories, Arnaud Pigneux, Christian Récher
The treatment of older patients with acute myeloid leukemia that is secondary to previous myelodysplastic syndrome, myeloproliferative neoplasm, or prior cytotoxic exposure remains unsatisfactory. We compared 92 and 107 patients treated, respectively, with intensive chemotherapy or azacitidine within two centres. Diagnoses were 37.5% post-myelodysplastic syndrome, 17.4% post-myeloproliferative neoplasia, and 45.1% therapy-related acute myeloid leukemia. Patients treated by chemotherapy had less adverse cytogenetics, higher white blood-cell counts, and were younger: the latter two being independent factors entered into the multivariate analyses...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29107340/langerhans-cell-histiocytosis-a-neoplastic-disorder-driven-by-ras-erk-pathway-mutations
#17
REVIEW
Gary Tran, Thy N Huynh, Amy S Paller
Langerhans cell histiocytosis (LCH) is a disorder of myeloid neoplasia of dendritic cells that affects 1 in 200,000 children <15 years of age and even fewer adults. LCH presents with a spectrum of clinical manifestations. High-risk stratification is reserved for infiltration of blood, spleen, liver, and lungs. After decades of debate on the disease pathogenesis, a neoplastic mechanism is now favored on the basis of LCH cell clonality, rare cases of familial clustering, and recent evidence of mutations involving the Ras/Raf/MEK (mitogen-activated protein kinase kinase)/ERK (extracellular signal-regulated kinase) pathway in lesional biopsy specimens...
March 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29064023/diagnostic-and-prognostic-utility-of-fluorescence-in-situ-hybridization-fish-analysis-in-acute-myeloid-leukemia
#18
REVIEW
Patrick R Gonzales, Fady M Mikhail
PURPOSE OF REVIEW: Acute myeloid leukemia (AML) is a hematologic neoplasia consisting of incompletely differentiated hematopoietic cells of the myeloid lineage that proliferate in the bone marrow, blood, and/or other tissues. Clinical implementation of fluorescence in situ hybridization (FISH) in cytogenetic laboratories allows for high-resolution analysis of recurrent structural chromosomal rearrangements specific to AML, especially in AML with normal karyotypes, which comprises approximately 33-50% of AML-positive specimens...
December 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28985529/multiplex-crispr-cas9-based-genome-editing-in-human-hematopoietic-stem-cells-models-clonal-hematopoiesis-and-myeloid-neoplasia
#19
Zuzana Tothova, John M Krill-Burger, Katerina D Popova, Catherine C Landers, Quinlan L Sievers, David Yudovich, Roger Belizaire, Jon C Aster, Elizabeth A Morgan, Aviad Tsherniak, Benjamin L Ebert
Hematologic malignancies are driven by combinations of genetic lesions that have been difficult to model in human cells. We used CRISPR/Cas9 genome engineering of primary adult and umbilical cord blood CD34+ human hematopoietic stem and progenitor cells (HSPCs), the cells of origin for myeloid pre-malignant and malignant diseases, followed by transplantation into immunodeficient mice to generate genetic models of clonal hematopoiesis and neoplasia. Human hematopoietic cells bearing mutations in combinations of genes, including cohesin complex genes, observed in myeloid malignancies generated immunophenotypically defined neoplastic clones capable of long-term, multi-lineage reconstitution and serial transplantation...
October 5, 2017: Cell Stem Cell
https://www.readbyqxmd.com/read/28958974/myeloid-neoplasia-and-lymphoblastic-lymphoma-with-eosinophilia-after-rai-a-case-report
#20
Mahmut Büyükşimşek, Semra Paydaş, Ali Oğul, Emine Bağır, Melek Ergin
BACKGROUND: Thyroid cancer is the most common endocrine cancers with an increasing incidence around the world in the last three decades. The increased risk of secondary cancer is associated with genetic predisposition or radioactive iodine (RAI) used in the treatment. CASE REPORT: 65-year old male patient was operated for thyroid papillary cancer. Total thyroidectomy was performed. He received RAI two times. After six years he presented with eosinophilic myeloproliferative neoplasia and T-cell lymphoblastic leukemia/lymphoma, together...
September 29, 2017: Balkan Medical Journal
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