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https://www.readbyqxmd.com/read/28521633/cd30-expression-in-pediatric-neoplasms-study-of-585-cases
#1
Jinjun Cheng, Haiqing Zhu, John Kim Choi
CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8), and its normal expression is restricted to activated T and B cells. In tumor cells, CD30 expression is most commonly associated with lymphoid malignancies (Hodgkin and non-Hodgkin lymphomas) and is a therapeutic target using anti-CD30 antibody. CD30 expression has been reported also in mostly adult non-lymphoid malignancies, raising the possibility of CD30-targeted therapy for additional tumors. In this study, we examined the incidence of CD30 expression in 251 hematopoietic and 334 non-hematopoietic cases of pediatric tumors...
June 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28512190/hematopoietic-origin-of-langerhans-cell-histiocytosis-and-erdheim-chester-disease-in-adults
#2
Paul Milne, Venetia Bigley, Chris M Bacon, Antoine Néel, Naomi McGovern, Simon Bomken, Muzlifah Haniffa, Eli L Diamond, Benjamin H Durham, Johannes Visser, David Hunt, Harsha Gunawardena, Mac Macheta, Kenneth L McClain, Carl Allen, Omar Abdel-Wahab, Matthew Collin
Langerhans cell histiocytosis (LCH) and Erdheim Chester Disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAP kinase pathway genes. BRAF(V600E) mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH or ECD arise from hematopoietic stem cells (HSC) nor which potential blood borne precursors lead to the formation of histiocytic lesions. In this study, BRAF V600E allele-specific PCR was used to map the neoplastic clone in 20 adults with LCH ECD and HCL...
May 16, 2017: Blood
https://www.readbyqxmd.com/read/28502479/megakaryocytes-in-myeloproliferative-neoplasms-have-unique-somatic-mutations
#3
Belinda B Guo, Richard J Nigel Allcock, Bob Mirzai, Jacques A Jacobus Malherbe, Fizzah A Choudry, Mattia Frontini, Hun Chuah, James Liang, Simon E Kavanagh, Rebecca Howman, Willem H Ouwehand, Kathryn A Fuller, Wendy N Erber
Myeloproliferative neoplasms (MPNs) are a group of related clonal hemopoietic stem cell disorders associated with hyperproliferation of myeloid cells. They are driven by mutations in the hemopoietic stem cell, most notably JAK2(V617F), CALR, and MPL. Clinically, they have the propensity to progress to myelofibrosis and transform to acute myeloid leukemia. Megakaryocytic hyperplasia with abnormal features are characteristic, and it is thought that these cells stimulate and drive fibrotic progression. The biological defects underpinning this remain to be explained...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28501635/determining-the-role-of-inflammation-in-the-selection-of-jak2-mutant-cells-in-myeloproliferative-neoplasms
#4
Jie Zhang, Angela G Fleischman, Dominik Wodarz, Natalia L Komarova
Myeloproliferative neoplasm (MPN) is a hematologic malignancy characterized by the clonal outgrowth of hematopoietic cells with a somatically acquired mutation most commonly in JAK2 (JAK2(V617F)). This mutation endows upon myeloid progenitors cytokine independent growth and consequently leads to excessive production of myeloid lineage cells. It has been previously suggested that inflammation may play a role in the clonal evolution of JAK2(V617F) mutants. In particular, it is possible that one or more cellular kinetic parameters of hematopoietic stem cells (HSCs) are affected by inflammation, such as division or death rates of cells, and the probability of HSC differentiation...
May 10, 2017: Journal of Theoretical Biology
https://www.readbyqxmd.com/read/28499938/allogeneic-stem-cell-transplantation-in-myelofibrosis
#5
REVIEW
Tania Jain, Ruben A Mesa, Jeanne M Palmer
Myeloproliferative neoplasm is a category in the World Health Organization classification of myeloid tumors. BCR-ABL1-negative MPN is a subcategory that includes primary myelofibrosis (MF), post-essential thrombocythemia MF, and post-polycythemia vera MF. These disorders are characterized by stem cell-derived clonal myeloproliferation. Clinically, these diseases present with anemia and splenomegaly, as well as significant constitutional symptoms, such as severe fatigue, symptoms associated with an enlarged spleen and liver, pruritus, fevers, night sweats, and bone pain...
May 9, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28497563/frequency-of-polycythemia-in-individuals-with-normal-complete-blood-cell-counts-according-to-the-new-2016-who-classification-of-myeloid-neoplasms
#6
A F Sandes, M V Gonçalves, M de L Chauffaille
INTRODUCTION: Polycythemia vera (PV) is a disorder characterized by clonal proliferation of myeloid cells and increased red blood cell mass. Recently, the revised 2016 WHO classification of myeloid neoplasms decreased the threshold levels of hemoglobin and hematocrit for the diagnosis of PV. However, the new proposed cutoffs have remarkable overlap with the normal reference values reported and the clinical impact of these new cutoffs has not been widely assessed in the general population...
May 12, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28495918/bone-marrow-morphology-is-a-strong-discriminator-between-chronic-eosinophilic-leukemia-not-otherwise-specified-from-reactive-idiopathic-hypereosinophilic-syndrome
#7
Sa A Wang, Robert P Hasserjian, Wayne Tam, Albert G Tsai, Julia T Geyer, Tracy I George, Kathryn Foucar, Heesun J Rogers, Eric D Hsi, Bryan A Rea, Adam Bagg, Carlos Bueso-Ramos C, Daniel A Arber, Srdan Verstovsek, Attilio Orazi
Chronic eosinophilic leukemia, not otherwise specified can be difficult to distinguish from idiopathic hypereosinophilic syndrome according to the current World Health Organization guideline. To examine if BM morphological features might aid in the differential diagnosis of these two entities, we studied a total of 139 patients with a diagnosis of chronic eosinophilic leukemia, not otherwise specified (n=17) or idiopathic hypereosinophilic syndrome (n=122). As a group, abnormal BM morphological features, resembling myelodysplastic syndromes, myeloproliferative neoplasm or myelodysplastic/myeloproliferative neoplasm were identified in 40/139 (27%) patients, 16(94%) chronic eosinophilic leukemia and 24 (20%) hypereosinophilic syndrome...
May 11, 2017: Haematologica
https://www.readbyqxmd.com/read/28491265/experience-with-ruxolitinib-in-the-treatment-of-polycythaemia-vera
#8
REVIEW
Samah Alimam, Claire Harrison
Polycythaemia vera (PV) is a myeloproliferative neoplasm classically characterized by an erythrocytosis and is associated with a high risk of thromboembolic events, constitutional symptoms burden and risk of transformation to myelofibrosis and acute myeloid leukaemia. Therapy is directed at the haematocrit (HCT) to reduce the risk of thrombotic events and usually comprises low-dose aspirin and phlebotomy to maintain HCT at >45%. Frequently in addition, cytoreductive therapy is indicated in high-risk patients for normalizing haematological parameters to mitigate the occurrence of thromboembolic events...
April 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28484168/current-diagnosis-and-treatment-for-acute-myeloid-leukemia
#9
Yukio Kobayashi
Genomic studies of acute myeloid leukemia have been extensively in progress. In patients with therapy-related myeloid neoplasms, peripheral blood before chemotherapy was found to have a minute clone with mutated oncogenes and tumor suppressor genes. Patients who were in complete remission showed a fraction of mutated genes. This status involves clonal hematopoiesis of indeterminate potential and demonstrates that a fraction of cells with mutated genes cannot result in leukemia. Regarding treatment, the results of two phase 3 studies have shown efficacy; midostaurin is effective in combination with the standard 7+3 therapy, and another mixed compound comprising micellized cytarabine and daunorubicin at a mixing molar ratio of 5 : 1 is more effective than the standard 7+3 therapy...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28484156/bcr-abl1-positive-chronic-myeloid-leukemia-emerging-in-a-patient-with-secondary-myelofibrosis-harboring-the-jak2-v617f-mutation
#10
Ayae Amemiya, Yoshikazu Ito, Yasunori Ishibashi, Yuu Saito, Seiichiro Katagiri, Tamiko Suguro, Michiyo Asano, Seiichiro Yoshizawa, Daigo Akahane, Yuko Tanaka, Hiroaki Fujimoto, Seiichi Okabe, Moritaka Gotoh, Tetsuzo Tauchi, Kazuma Ohyashiki
A 53-year-old woman with a 27-year history of myeloproliferative neoplasms came to our hospital because of a marked white blood cell count increase and progressive anemia. Clinical examination demonstrated positivity for BCR-ABL1 and JAK2-V617F mutations. She was given a diagnosis of chronic myeloid leukemia. Using the international scale, a molecular response (MR) (4.5) was achieved after treatment with dasatinib, despite the persistence of marked splenomegaly. The pathological findings of myelofibrosis were demonstrated by bone marrow biopsy...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28482851/checkpoint-inhibitors-in-hematological-malignancies
#11
REVIEW
Chi Young Ok, Ken H Young
Inhibitory molecules such as PD-1, CTLA-4, LAG-3, or TIM-3 play a role to keep a balance in immune function. However, many cancers exploit such molecules to escape immune surveillance. Accumulating data support that their functions are dysregulated in lymphoid neoplasms, including plasma cell myeloma, myelodysplastic syndrome, and acute myeloid leukemia. In lymphoid neoplasms, aberrations in 9p24.1 (PD-L1, PD-L2, and JAK2 locus), latent Epstein-Barr virus infection, PD-L1 3'-untranslated region disruption, and constitutive JAK-STAT pathway are known mechanisms to induce PD-L1 expression in lymphoma cells...
May 8, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28476038/prognostic-role-of-tet2-deficiency-in-myelodysplastic-syndromes-a-meta-analysis
#12
Yun Lin, Zhijuan Lin, Kun Cheng, Zhihong Fang, Zhifeng Li, Yiming Luo, Bing Xu
Tet methylcytosine dioxygenase2 gene (TET2) is one of the most frequently mutated gene in myeloid neoplasm, but the prognostic role of TET2 aberrations in myelodysplastic syndromes (MDS) remains unclear. Therefore, we performed a meta-analysis. Fourteen eligible studies with 1983 patients were included in this meta-analysis. Among these, 2 studies evaluated the impact that the TET2 expression level had on the prognosis. The combined hazard ratios (HR) estimated for overall survival (OS) was 1.00 (95%CI: 0.74 to 1...
April 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28472771/setbp1-dysregulation-in-congenital-disorders-and-myeloid-neoplasms
#13
REVIEW
Nicoletta Coccaro, Giuseppina Tota, Antonella Zagaria, Luisa Anelli, Giorgina Specchia, Francesco Albano
Myeloid malignancies are characterized by an extreme molecular heterogeneity, and many efforts have been made in the past decades to clarify the mechanisms underlying their pathogenesis.In this scenario SET binding protein 1 (SETBP1) has attracted a lot of interest as a new oncogene and potential marker, in addition to its involvement in the Schinzel-Giedon syndrome (SGS). Our review starts with the analysis of the structural characteristics of SETBP1, and extends to its corresponding physiological and pathological functions...
April 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28470464/expanding-diversity-and-common-goal-of-regulatory-t-and-b-cells-ii-in-allergy-malignancy-and-transplantation
#14
REVIEW
Grażyna Korczak-Kowalska, Anna Stelmaszczyk-Emmel, Katarzyna Bocian, Ewelina Kiernozek, Nadzieja Drela, Joanna Domagała-Kulawik
Regulation of immune response was found to play an important role in the course of many diseases such as autoimmune diseases, allergy, malignancy, organ transplantation. The studies on immune regulation focus on the role of regulatory cells (Tregs, Bregs, regulatory myeloid cells) in these disorders. The number and function of Tregs may serve as a marker of disease activity. As in allergy, the depletion of Tregs is observed and the results of allergen-specific immunotherapy could be measured by an increase in the population of IL-10(+) regulatory cells...
May 3, 2017: Archivum Immunologiae et Therapiae Experimentalis
https://www.readbyqxmd.com/read/28459613/impact-of-expert-pathologic-review-of-lymphoma-diagnosis-study-of-patients-from-the-french-lymphopath-network
#15
Camille Laurent, Marine Baron, Nadia Amara, Corinne Haioun, Mylène Dandoit, Marc Maynadié, Marie Parrens, Beatrice Vergier, Christiane Copie-Bergman, Bettina Fabiani, Alexandra Traverse-Glehen, Nicole Brousse, Marie-Christine Copin, Patrick Tas, Tony Petrella, Marie-Christine Rousselet, Josette Brière, Fréderic Charlotte, Catherine Chassagne-Clement, Thérèse Rousset, Luc Xerri, Anne Moreau, Antoine Martin, Diane Damotte, Peggy Dartigues, Isabelle Soubeyran, Michel Peoch, Pierre Dechelotte, Jean-François Michiels, Antoine de Mascarel, Françoise Berger, Céline Bossard, Flavie Arbion, Isabelle Quintin-Roué, Jean-Michel Picquenot, Martine Patey, Blandine Fabre, Henri Sevestre, Cécile Le Naoures, Marie-Pierre Chenard-Neu, Claire Bastien, Sylvie Thiebault, Laurent Martin, Manuela Delage, Thomas Filleron, Gilles Salles, Thierry Jo Molina, Georges Delsol, Pierre Brousset, Philippe Gaulard
Purpose To prospectively assess the clinical impact of expert review of lymphoma diagnosis in France. Materials and Methods From January 2010 to December 2013, 42,145 samples from patients with newly diagnosed or suspected lymphomas were reviewed, according to the 2008 WHO classification, in real time by experts through the Lymphopath Network. Changes in diagnosis between referral and expert review were classified as major or minor according to their potential impact on patient care. Results The 42,145 reviewed samples comprised 36,920 newly diagnosed mature lymphomas, 321 precursor lymphoid neoplasms, 314 myeloid disorders, and 200 nonhematopoietic neoplasms, with 4,390 benign lesions...
May 1, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28455647/cmml-clinical-and-molecular-aspects
#16
REVIEW
Raphael Itzykson, Matthieu Duchmann, Nolwenn Lucas, Eric Solary
Chronic Myelomonocytic Leukemia is a chronic myeloid neoplasm occurring mostly in the elderly with overlapping features of myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) characterized by chronic monocytosis. Recent progresses in the molecular and cellular pathogenesis of CMML have stirred a renewed interest in this clinically heterogeneous disorder. Here, we review the recent progresses in the biology of CMML and how it affects its current and future clinical management.
June 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28450183/hematopoietic-stem-cell-transplant-activity-in-pediatric-cancer-between-2008-and-2014-in-the-united-states-a-cibmtr-report
#17
Pooja Khandelwal, Heather R Millard, Elizabeth Thiel, Hisham Abdel-Azim, Allistair A Abraham, Jeffery J Auletta, Farid Boulad, Valerie I Brown, Bruce M Camitta, Ka Wah Chan, Sonali Chaudhury, Morton J Cowan, Miguel Angel-Diaz, Shahinaz M Gadalla, Robert Peter Gale, Gregory Hale, Kimberly A Kasow, Amy K Keating, Carrie L Kitko, Margaret L MacMillan, Richard F Olsson, Kristin M Page, Adriana Seber, Angela R Smith, Anne B Warwick, Baldeep Wirk, Parinda A Mehta
This CIBMTR report describes the use of hematopoietic stem cell transplantation (HSCT) in 4408 pediatric patients with cancer undergoing allogeneic (allo) and 3076 undergoing autologous (auto) HSCT in the United States between 2008 and 2014. In both settings, there was a greater proportion of males (n=4327; 57%), children<10 years of age (n=4412; 59%), Caucasians (n=5787; 77%) and children with a performance score ≥ 90% at HSCT (n=6187; 83%). Leukemia was the most common indication for an allo-transplant (n=4170; 94%), and among these, acute lymphoblastic leukemia (ALL) in second complete remission (n=829; 20%) and acute myeloid leukemia in first complete remission (n=800; 19%) were the most common...
April 24, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28449810/novel-t-5-11-q32-q13-4-with-numa1-pdgfrb-fusion-in-a-myeloid-neoplasm-with-eosinophilia-with-response-to-imatinib-mesylate
#18
Ying S Zou, Nicole L Hoppman, Zeba N Singh, Sameer Sawhney, Sandy D Kotiah, Maria R Baer
We report a NUMA1-PDGFRB fusion in a myeloproliferative neoplasm with eosinophilia in a 61-year old man, with response to imatinib mesylate therapy. A t(5;11) chromosome translocation involving bands 5q32 and 11q13.4 was identified by metaphase chromosome analysis, and rearrangement of the platelet-derived growth factor receptor beta (PDGFRB) gene on 5q32 was demonstrated by FISH using a PDGFRB break-apart probe set. Bacterial artificial chromosome (BAC) FISH mapping of the PDGFRB fusion partner gene narrowed the breakpoint at 11q13...
April 2017: Cancer Genetics
https://www.readbyqxmd.com/read/28447248/somatic-setbp1-mutations-in-myeloid-neoplasms
#19
REVIEW
Hideki Makishima
SETBP1 is a SET-binding protein regulating self-renewal potential through HOXA-protein activation. Somatic SETBP1 mutations were identified by whole exome sequencing in several phenotypes of myelodysplastic/myeloproliferative neoplasms (MDS/MPN), including atypical chronic myeloid leukemia, chronic myelomonocytic leukemia, and juvenile myelomonocytic leukemia as well as in secondary acute myeloid leukemia (sAML). Surprisingly, its recurrent somatic activated mutations are located at the identical positions of germline mutations reported in congenital Schinzel-Giedion syndrome...
June 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28445830/overexpression-of-heme-oxygenase-1-in-bone-marrow-stromal-cells-promotes-microenvironment-mediated-imatinib-resistance-in-chronic-myeloid-leukemia
#20
Ping Liu, Dan Ma, Zhengyu Yu, Nana Zhe, Mei Ren, Ping Wang, Meisheng Yu, Jun Huang, Qin Fang, Jishi Wang
Neoplasm cells from patients with chronic myeloid leukemia (CML) interact with stromal cells of the surrounding microenvironment. Bone marrow stromal cells (BMSCs) represent the main population in CML marrow stroma, which may play a key role in disease support and progression. Heme oxygenase-1 (HO-1) is a key enzyme of antioxidative metabolism that is associated with cell proliferation and resistance to apoptosis. We herein up-regulated HO-1 expression of BMSCs and evaluated whether BMSCs influenced K562 cells survival...
April 23, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
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