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Myelodysplastic syndromes

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https://www.readbyqxmd.com/read/28718702/a-novel-t-3-9-q21-2-p24-3-associated-with-smarca2-and-znf148-genes-rearrangement-in-myelodysplastic-syndrome
#1
Nicoletta Coccaro, Claudia Brunetti, Giuseppina Tota, Ciro Leo Pierri, Luisa Anelli, Antonella Zagaria, Paola Casieri, Luciana Impera, Crescenzio F Minervini, Angela Minervini, Cosimo Cumbo, Alessandra Ricco, Paola Carluccio, Paola Orsini, Giorgina Specchia, Francesco Albano
No abstract text is available yet for this article.
July 18, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28714944/impact-of-the-presence-of-hla-1-locus-mismatch-and-the-use-of-low-dose-antithymocyte-globulin-in-unrelated-bone-marrow-transplantation
#2
K Kawamura, J Kanda, S Fuji, M Murata, K Ikegame, K Yoshioka, T Fukuda, Y Ozawa, N Uchida, K Iwato, T Sakura, M Hidaka, H Hashimoto, T Ichinohe, Y Atsuta, Y Kanda
HLA 1-locus-mismatched unrelated donors (1MMUD) have been used in allogeneic hematopoietic stem cell transplantation (allo-HCT) for patients who lack an HLA-matched donor. We retrospectively analyzed 3313 patients with acute leukemia or myelodysplastic syndrome who underwent bone marrow transplantation from an HLA allele-matched unrelated donor (MUD) or 1MMUD between 2009 and 2014. We compared the outcomes of MUD (n=2089) and 1MMUD with antithymocyte globulin (ATG) (1MM-ATG(+); n=109) with those of 1MMUD without ATG (1MM-ATG(-); n=1115)...
July 17, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28714470/essential-role-of-fbxl5-mediated-cellular-iron-homeostasis-in-maintenance-of-hematopoietic-stem-cells
#3
Yoshiharu Muto, Masaaki Nishiyama, Akihiro Nita, Toshiro Moroishi, Keiichi I Nakayama
Hematopoietic stem cells (HSCs) are maintained in a hypoxic niche to limit oxidative stress. Although iron elicits oxidative stress, the importance of iron homeostasis in HSCs has been unknown. Here we show that iron regulation by the F-box protein FBXL5 is required for HSC self-renewal. Conditional deletion of Fbxl5 in mouse HSCs results in cellular iron overload and a reduced cell number. Bone marrow transplantation reveals that FBXL5-deficient HSCs are unable to reconstitute the hematopoietic system of irradiated recipients as a result of stem cell exhaustion...
July 17, 2017: Nature Communications
https://www.readbyqxmd.com/read/28714213/smoking-and-alcohol-and-subsequent-risk-of-myelodysplastic-syndromes-in-japan-the-japan-public-health-centre-based-prospective-study
#4
Tomotaka Ugai, Keitaro Matsuo, Norie Sawada, Motoki Iwasaki, Taiki Yamaji, Taichi Shimazu, Shizuka Sasazuki, Manami Inoue, Yoshinobu Kanda, Shoichiro Tsugane
Smoking and alcohol are important modifiable risk factors for human cancers. However, few epidemiological studies have investigated their association with the risk of myelodysplastic syndromes (MDS). Here, we investigated the association of smoking and alcohol consumption and the risk of MDS in a large-scale population-based cohort study in Japan. We included 95 510 Japanese subjects (45 451 men and 50 059 women; age 40-69 years at baseline) and identified 70 MDS cases (50 men and 20 women) during 18·3 years of follow-up...
July 17, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28713749/mucormycosis-of-the-frontal-sinus-a-rare-case-report-and-review
#5
Nanda Kishore Sahoo, Vishal Kulkarni, Amit K Bhandari, Arun Kumar
Mucormycosis of the frontal sinus are rarely seen in day to day clinical practice. Although this fungus is commonly found in the environment, the disease is usually prevented by the immune system and is hence rare. Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscrasias, and immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns. The disease is primarily found in those who are immunocompromised, but it may also manifest in immuno competent persons...
January 2017: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28711727/a-chemotherapy-only-regimen-of-busulfan-melphalan-and-fludarabine-and-rabbit-atg-followed-by-allogeneic-t-cell-depleted-hematopoietic-stem-cell-transplants-for-the-treatment-of-myeloid-malignancies
#6
Barbara Spitzer, Ann A Jakubowski, Esperanza B Papadopoulos, Kirsten Fuller, Patrick D Hilden, James W Young, Juliet Barker, Guenther Koehne, Miguel-Angel Perales, Katharine C Hsu, Marcel R M van den Brink, Nancy A Kernan, Susan E Prockop, Andromachi Scaradavou, Hugo Castro-Malaspina, Richard J O'Reilly, Farid Boulad
We sought to develop a myeloablative chemotherapeutic regimen to secure consistent engraftment of T-cell depleted (TCD) hematopoietic stem cell transplants (HSCT) without the need for total body irradiation, thereby reducing toxicity, while maintaining low rates of GvHD and without increasing relapse. We investigated the myeloablative combination of busulfan and melphalan, with the immunosuppressive agents fludarabine and rabbit anti-thymocyte gloubin (r-ATG) as cytoreduction prior to a T-cell depleted HSCT...
July 12, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28711726/-if-all-you-have-is-a-hammer-%C3%A2-transplants-for-myelodysplastic-syndrome-mds-after-hypomethylating-agents-hma-fail
#7
Alison Wakoff Loren
No abstract text is available yet for this article.
July 12, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28708320/monosomy-7-del-7q-in-inherited-bone-marrow-failure-syndromes-a-systematic-review
#8
REVIEW
Alex Pezeshki, Shreya Podder, Ralph Kamel, Seth J Corey
Inherited bone marrow failure syndromes (IBMFS) are rare cancer predisposition syndromes with an especially high risk of transformation to myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML). We performed a retrospective systematic review of reported MDS/AML arising in the eight most common IBMFS to determine the frequency and outcome of chromosome 7 abnormalities. We identified 738 MDS/AML cases of 4,293 individuals. Monosomy 7 or del (7q) occurred in ∼17%. Greater understanding of the roles played by sequential acquisition of genetic and cytogenetic changes will provide insights into myeloid leukemogenesis and improve the surveillance and hopefully outcomes for individuals with IBMFS...
July 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28706555/advances-in-iron-chelation-therapy-transitioning-to-a-new-oral-formulation
#9
Nirmish R Shah
Iron overload is a concern for patients who require repeated red-blood-cell transfusions due to conditions such as sickle cell disease, thalassemia, or myelodysplastic syndromes. The recommended treatment for removing excess iron in these patients is iron chelation therapy. Currently available iron chelators include deferoxamine, which is administered by injection, and deferasirox and deferiprone, both of which are administered orally. Adherence to iron chelator therapy is an important consideration and may be affected by side effects...
2017: Drugs in Context
https://www.readbyqxmd.com/read/28706392/acquired-aplastic-anemia-associated-with-trisomy-eight-converting-into-acute-myeloid-leukemia
#10
Sumit Grover, Amit Kumar Dhiman, Bhavna Garg, Neena Sood, Vikram Narang
Aplastic anemia (AA) is nowadays considered to be a clonal disorder arising from a defective hematopoietic stem cell developing after a generalized insult to bone marrow. Immunosuppressive treatment (IST) of AA causes suppression of the target dominant population of haematopoietic cells allowing the defective non targeted clones to expand. This may give rise to acute leukemia. Cytogenetic studies for chromosomal aberrations such as trisomy and monosomy may help in detecting such conversions. We present a case of acquired AA in a 60-year-old male presenting with pancytopenia and hypoplastic marrow treated with antithymocyte globulin, converting into myelodysplastic syndrome and later on acute promyelocytic leukemia after being in remission for 4 years...
July 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28705838/leukofiltration-plus-pathogen-reduction-prevents-alloimmune-platelet-refractoriness-in-a-dog-transfusion-model
#11
Sherrill J Slichter, Esther Pellham, S Lawrence Bailey, Todd Christoffel, Irena Gettinger, Lakshmi Gaur, Yvette Latchman, Karen Nelson, Doug Bolgiano
Human Lymphocyte Antigen (HLA) alloimmunization to filter leukoreduced (F-LR) platelets occurs in about 18% of immunosuppressed thrombocytopenic hematology/oncology patients and represents a significant challenge for effective chemotherapy. In a dog platelet transfusion model, we have evaluated other methods of preventing alloimmune platelet refractoriness and demonstrated that successful methods in our dog model are transferable to man. In the present study, donor/recipient pairs were dog lymphocyte antigen (DLA) DR-B incompatible (88% of the pairs), and recipient dogs received up to 8 weekly treated transfusions from a single donor (a highly immunogenic stimulus), or until platelet refractoriness...
July 13, 2017: Blood
https://www.readbyqxmd.com/read/28705782/biologics-in-myelodysplastic-syndrome-related-systemic-inflammatory-and-autoimmune-diseases-french-multicenter-retrospective-study-of-29-patients
#12
REVIEW
Arsene Mekinian, Guillaume Dervin, Nathanael Lapidus, Jean-Emmanuel Kahn, Louis Terriou, Eric Liozon, Eric Grignano, Jean-Charles Piette, Odile Beyne Rauzy, Vincent Grobost, Pascal Godmer, Jerome Gillard, Julien Rossignol, David Launay, Achille Aouba, Thierry Cardon, Laurence Bouillet, Jonathan Broner, Julien Vinit, Lionel Ades, Fabrice Carrat, Clementine Salvado, Eric Toussirot, Mathilde Versini, Nathalie Costedoat-Chalumeau, Jean Baptiste Fraison, Philippe Guilpain, Pierre Fenaux, Olivier Fain
BACKGROUND: Systemic inflammatory and autoimmune diseases (SIADs) associated with myelodysplastic syndromes are often difficult to treat. Corticosteroids are efficient but only usually at high doses. The use of biologics needs to be specified. METHODS: In a French multicenter retrospective study, we analyzed the efficacy and safety of biologics (tumor necrosis factor-α [TNF-α] antagonists, tocilizumab, rituximab and anakinra) for SIADs associated with myelodysplastic syndromes (MDSs)...
July 10, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28705776/short-and-medium-term-biological-variation-estimates-of-leukocytes-extended-to-differential-count-and-morphology-structural-parameters-cell-population-data-in-blood-samples-obtained-from-healthy-people
#13
Sabrina Buoro, Anna Carobene, Michela Seghezzi, Barbara Manenti, Aurelio Pacioni, Ferruccio Ceriotti, Cosimo Ottomano, Giuseppe Lippi
BACKGROUND: Recent studies showed that some cell population data (CPD) parameters of neutrophils may be useful for diagnosing myelodysplastic syndromes and sepsis, for the differential diagnosis of acute promyelocytic leukemia, and some CPD parameters of lymphocytes may be a valuable tool for preliminary screening of B cell lymphoproliferative disease. Notwithstanding the knowledge, no information has been made available about their analytical quality specification. This study was aimed to define short- and medium-term biological variation (BV) estimates and reference change value (RCV) of leukocyte count, extended leukocyte differential and CPD...
July 10, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28705540/three-year-safety-of-radium-223-dichloride-in-patients-with-castration-resistant-prostate-cancer-and-symptomatic-bone-metastases-from-phase-3-randomized-alpharadin-in-symptomatic-prostate-cancer-trial
#14
Christopher C Parker, Robert E Coleman, Oliver Sartor, Nicholas J Vogelzang, David Bottomley, Daniel Heinrich, Svein I Helle, Joe M O'Sullivan, Sophie D Fosså, Aleš Chodacki, Paweł Wiechno, John Logue, Mihalj Seke, Anders Widmark, Dag Clement Johannessen, Peter Hoskin, Nicholas D James, Arne Solberg, Isabel Syndikus, Jan Kliment, Steffen Wedel, Sibylle Boehmer, Marcos Dall'Oglio, Lars Franzén, Øyvind S Bruland, Oana Petrenciuc, Karin Staudacher, Rui Li, Sten Nilsson
BACKGROUND: In Alpharadin in Symptomatic Prostate Cancer (ALSYMPCA) trial, radium-223 versus placebo prolonged overall survival with favorable safety in castration-resistant prostate cancer patients with symptomatic bone metastases. Long-term radium-223 monitoring underlies a comprehensive safety and risk/benefit assessment. OBJECTIVE: To report updated ALSYMPCA safety, including long-term safety up to 3 yr after the first injection. DESIGN, SETTING, AND PARTICIPANTS: Safety analyses from phase 3 randomized ALSYMPCA trial included patients receiving ≥1 study-drug injection (600 radium-223 and 301 placebo)...
July 10, 2017: European Urology
https://www.readbyqxmd.com/read/28698789/a-population-based-study-on-myelodysplastic-syndromes-in-the-lazio-region-italy-medical-miscoding-and-11-year-mortality-follow-up-the-gruppo-romano-laziale-mielodisplasie-experience-of-retrospective-multicentric-registry
#15
Flavia Mayer, Laura Faglioni, Nera Agabiti, Susanna Fenu, Francesco Buccisano, Roberto Latagliata, Roberto Ricci, Maria Antonietta Aloe Spiriti, Caterina Tatarelli, Massimo Breccia, Giuseppe Cimino, Luana Fianchi, Marianna Criscuolo, Svitlana Gumenyuk, Stefano Mancini, Luca Maurillo, Carolina Nobile, Pasquale Niscola, Anna Lina Piccioni, Agostino Tafuri, Giulio Trapè, Alessandro Andriani, Paolo De Fabritiis, Maria Teresa Voso, Marina Davoli, Gina Zini
Data on Myelodysplastic Syndromes (MDS) are difficult to collect by cancer registries because of the lack of reporting and the use of different classifications of the disease. In the Lazio Region, data from patients with a confirmed diagnosis of MDS, treated by a hematology center, have been collected since 2002 by the Gruppo Romano-Laziale Mielodisplasie (GROM-L) registry, the second MDS registry existing in Italy. This study aimed at evaluating MDS medical miscoding during hospitalizations, and patients' survival...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28698788/treatment-of-low-blast-count-aml-using-hypomethylating-agents
#16
REVIEW
Eleonora De Bellis, Luana Fianchi, Francesco Buccisano, Marianna Criscuolo, Luca Maurillo, Laura Cicconi, Mattia Brescini, Maria Ilaria Del Principe, Ambra Di Veroli, Adriano Venditti, Sergio Amadori, William Arcese, Francesco Lo-Coco, Maria Teresa Voso
In 2002, the WHO classification reduced the proportion of blasts in the bone marrow (BM) necessary for the diagnosis of acute myeloid leukemia (AML) from 30% to 20%, eliminating the RAEB-t subtype of myelodysplastic syndromes (MDS). However, this AML subtype, defined as low-blast count AML (LBC-AML, with 20-30% BM-blasts) is characterized by peculiar features, as increased frequency in elderly individuals and after cytotoxic treatment for a different primary disease (therapy-related), poor-risk cytogenetics, lower white blood cell counts, and less frequent mutations of NPM1 and FLT3 genes...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28693140/acute-myeloid-leukemia-with-t-3-21-q26-2-q22-developing-following-low-dose-methotrexate-therapy-for-rheumatoid-arthritis-and-expressing-two-aml1-mds1-evi1-fusion-proteins-a-case-report
#17
Keisuke Tanaka, Gaku Oshikawa, Hiroki Akiyama, Shinya Ishida, Toshikage Nagao, Masahide Yamamoto, Osamu Miura
The t(3;21)(q26.2;q22) translocation is a rare chromosomal abnormality exhibited almost exclusively in therapy-related myelodysplastic syndrome/acute myeloid leukemia (t-MDS/AML) or in the blastic crisis phase of chronic myelogenous leukemia, which results in the fusion of the runt related transcription factor 1 (RUNX1, also called AML1) gene at 21q22 to the myelodysplasia syndrome 1 (MDS1)-ecotropic virus integration site 1 (EVI1) complex locus (MECOM) at 3q26.2, generating various fusion transcripts, including AML1/MDS1/EVI1 (AME)...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28691152/reduced-bucy-2-and-g-csf-primed-bone-marrow-associates-with-low-graft-versus-host-disease-and-transplant-related-mortality-in-allogeneic-hsct
#18
Eucario Leon Rodriguez, Monica M Rivera Franco, Sandra I Perez Alvarez
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the ideal treatment for several diseases. However, the morbidity and mortality associated with the procedure might limit its widespread use; therefore, we implemented reduced BUCY2 as conditioning method along with the use of G-CSF-primed bone marrow (G-BM) in order to reduce complications, including graft-versus-host-disease (GVHD), and to improve survival in these patients. An analysis of transplant characteristics, complications, and survival of patients undergoing an allo-HSCT using this conditioning regimen (busulfan 12 mg/kg and cyclophosphamide 80 mg/kg) plus G-BM was performed...
July 9, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28690314/evolution-of-npm1-negative-therapy-related-myelodysplastic-syndromes-following-curative-treatment-of-npm1-mutant-aml
#19
S Herold, K Sockel, C Sayehli, R Herbst, U Dührsen, U Oelschlägel, A Böttner, H Hindahl, J Kullmer, S Helas, M Sauer, B Mohr, A Mies, M Bornhäuser, G Ehninger, C Röllig, C Thiede, U Platzbecker
Leukemia accepted article preview online, 10 July 2017. doi:10.1038/leu.2017.217.
July 10, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28689016/long-term-follow-up-of-invasive-aspergillosis-in-allogeneic-stem-cell-transplantation-recipients-and-leukemia-patients-differences-in-risk-factors-and-outcomes
#20
S Bonnet, R Duléry, K Regany, M Bouketouche, L Magro, V Coiteux, S Alfandari, C Berthon, B Quesnel, I Yakoub-Agha
Antifungal prophylaxis (AP) has dramatically changed the epidemiology of invasive aspergillosis (IA). To better understand the differences in terms of clinical significance of IA between allogeneic stem cell transplantation (allo-SCT) recipients and patients treated for leukemia, we report a single-center study of 735 unselected consecutive patients treated between 2000 and 2004, before the era of systematic AP. Probable or confirmed IA were observed in 29 patients (2008 EORTC/MSG criteria), including 7/235 undergoing allo-SCT (5...
July 5, 2017: Current Research in Translational Medicine
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