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Myelodysplastic syndrom

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https://www.readbyqxmd.com/read/28343141/survival-outcomes-in-iron-chelated-and-non-chelated-patients-with-lower-risk-myelodysplastic-syndromes-review-and-pooled-analysis-of-observational-studies
#1
EDITORIAL
Ivo Abraham, Majed Al Yami, Seongseok Yun, Hee-Ju Kim, Nicole D Vincelette, Ali McBride, Karen MacDonald
No abstract text is available yet for this article.
March 18, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28340281/high-ten-year-remission-rates-following-rituximab-fludarabine-mitoxantrone-and-dexamethasone-r-fnd-with-interferon-maintenance-in-indolent-lymphoma-results-of-a-randomized-study
#2
Loretta J Nastoupil, Peter McLaughlin, Lei Feng, Sattva S Neelapu, Felipe Samaniego, Fredrick B Hagemeister, Ana Ayala, Jorge E Romaguera, Andre H Goy, Eleanor Neal, Michael Wang, Luis Fayad, Michelle A Fanale, Yasuhiro Oki, Jason R Westin, Maria A Rodriguez, Fernando Cabanillas, Nathan H Fowler
We report a single-centre, randomized study evaluating the efficacy and safety of concurrent fludarabine, mitoxantrone, dexamethasone (FND) and rituximab versus sequential FND followed by rituximab in 158 patients with advanced stage, previously untreated indolent lymphoma, enrolled between 1997 and 2002. Patients were randomized to 6-8 cycles of FND followed by 6 monthly doses of rituximab or 6 doses of rituximab given concurrently with FND. All patients who achieved at least a partial response received 12 months of interferon (IFN) maintenance...
March 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28338652/3q26-2-evi1-rearrangement-is-associated-with-poor-prognosis-in-classical-philadelphia-chromosome-negative-myeloproliferative-neoplasms
#3
Zhihong Hu, L Jeffrey Medeiros, Wei Wang, Zi Chen, Guilin Tang, Parsa Hodjat, Su Yang, Lianghua Fang, Yan Li, Srdan Verstovsek, Shimin Hu
Classical Philadelphia chromosome-negative myeloproliferative neoplasms are a group of closely related myeloid disorders with different histologic features and clinical presentations at an early stage, but all later develop into a similar fibrotic stage with variable risk of acute transformation. The significance of 3q26.2/EVI1 rearrangement has been well recognized in acute myeloid leukemia, myelodysplastic syndrome, and chronic myeloid leukemia. However, the clinical importance of 3q26.2/EVI1 rearrangement in classical Philadelphia chromosome-negative myeloproliferative neoplasms is unknown...
March 24, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28338082/a-clinical-trial-for-patients-with-acute-myeloid-leukemia-or-myelodysplastic-syndromes-not-eligible-for-standard-clinical-trials
#4
G Montalban-Bravo, X Huang, K Naqvi, E Jabbour, G Borthakur, C D DiNardo, N Pemmaraju, J Cortes, S Verstovsek, T Kadia, N Daver, W Wierda, Y Alvarado, M Konopleva, F Ravandi, Z Estrov, N Jain, A Alfonso, M Brandt, T Sneed, H C Chen, H Yang, C Bueso-Ramos, S Pierce, E Estey, Z Bohannan, H M Kantarjian, G Garcia-Manero
No abstract text is available yet for this article.
March 24, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28337860/clinical-features-and-long-term-outcomes-of-105-granulomatosis-with-polyangiitis-patients-a-single-center-experience-from-north-india
#5
Aman Sharma, Godasi S R S N K Naidu, Manish Rathi, Roshan Verma, Manish Modi, Benzeeta Pinto, Kusum Sharma, Varun Dhir, Manphool Singhal, Mahesh Prakash, Ritambhra Nada, Naresh K Panda, Ranjana W Minz
AIM: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). METHODS: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28337274/decitabine-treatment-sensitizes-tumor-cells-to-t-cell-mediated-cytotoxicity-in-patients-with-myelodysplastic-syndromes
#6
Zheng Zhang, Qi He, Ying Tao, Juan Guo, Feng Xu, Ling-Yun Wu, You-Shan Zhao, Dong Wu, Li-Yu Zhou, Ji-Ying Su, Lu-Xi Song, Chao Xiao, Xiao Li, Chun-Kang Chang
Decitabine treatment improves immunological recognition that increases expression of cancer-testis antigens (CTAs) against solid tumors. The mechanisms of decitabine enhancement of immunogenicity when used for patients with myelodysplastic syndromes (MDS) remain unclear. In the present study, we found relatively low baseline expression of MAGE-A1, MAGE-A3, and SP17 in MDS-derived cell lines. Decitabine treatment significantly improved MAGE-A1, MAGE-A3, and SP17 expression in these cell lines and in MDS patients...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28336325/allogeneic-hematopoietic-stem-cell-transplantation-is-underutilized-in-older-patients-with-myelodysplastic-syndromes
#7
Bartlomiej M Getta, Ashwin Kishtagari, Patrick Hilden, Martin S Tallman, Molly Maloy, Patrick Gonzales, Hugo Castro-Malaspina, Miguel-Angel Perales, Sergio Giralt, Roni Tamari, Virginia Klimek
Allogeneic hematopoietic stem cell transplantation (HCT) is the only curative treatment for myelodysplastic syndrome (MDS). The proportion of MDS patients referred for transplant evaluation, those undergoing transplantation and the reasons for not undergoing transplant are unknown. In this retrospective analysis, pre-defined HCT eligibility and indications criteria were applied to 362 unselected patients with newly diagnosed MDS seen by Leukemia faculty between 2008 and 2015 at Memorial Sloan Kettering Cancer Center...
March 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28331130/cytopenias-and-clonal-expansion-of-gamma-delta-t-cells-in-a-patient-with-anaplasmosis-a-potential-diagnostic-pitfall
#8
Daniel Marko, Anamarija M Perry, Arjuna Ponnampalam, Michel R Nasr
Human granulocytic anaplasmosis is a rare, tick-borne infectious disease caused by Anaplasma phagocytophilum. Herein, we report a rare case of human granulocytic anaplasmosis associated with cytopenias and clonal expansion of gamma/delta T-cells in the bone marrow. A 77-year old man presented multiple times to the emergency department complaining of muscle weakness. Complete blood count detected cytopenias and peripheral blood smear showed pseudo Pelger-Huet neutrophils. These findings prompted bone marrow evaluation with ancillary studies including flow cytometry, karyotyping and T-cell rearrangement studies...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28330607/association-between-anti-thymocyte-globulin-exposure-and-survival-outcomes-in-adult-unrelated-haemopoietic-cell-transplantation-a-multicentre-retrospective-pharmacodynamic-cohort-analysis
#9
Rick Admiraal, Stefan Nierkens, Moniek A de Witte, Eefke J Petersen, Ger-Jan Fleurke, Luka Verrest, Svetlana V Belitser, Robbert G M Bredius, Reinier A P Raymakers, Catherijne A J Knibbe, Monique C Minnema, Charlotte van Kesteren, Jurgen Kuball, Jaap J Boelens
BACKGROUND: Anti-thymocyte globulin (ATG) is used to prevent graft-versus-host disease (GvHD) after allogeneic haemopoietic cell transplantation (HCT). However, ATG can also cause delayed immune reconstitution of T cells, negatively affecting survival. We studied the relation between exposure to ATG and clinical outcomes in adult patients with acute leukaemia and myelodysplastic syndrome. METHODS: We did a retrospective, pharmacokinetic-pharmacodynamic analysis of data from patients with acute lymphoid leukaemia, acute myeloid leukaemia, or myelodysplastic syndrome receiving their first T-cell repleted allogeneic peripheral blood stem cell HCT with ATG (thymoglobulin) as part of non-myeloablative conditioning from March 1, 2004, to June 1, 2015...
March 16, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28326965/hematogones-detected-by-flow-cytometry-in-a-child-with-vitamin-b12-deficiency
#10
Lisa Sutton, Nkechi Mba
Vitamin B12 deficiency is a known cause of megaloblastic anemia and bone marrow failure. Bone marrow biopsies are not frequently performed as part of the diagnostic workup and can demonstrate morphologic features that overlap with myelodysplastic syndrome (MDS) and acute leukemia. We describe a case of a dysplastic bone marrow with increased bone marrow hematogones detected by flow cytometry in a child with vitamin B12 deficiency. Hematogones are normal B cell precursors, and hyperplasia has been described in a variety of often reactive conditions and also disease...
March 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28324772/new-proposals-of-the-who-working-group-2016-for-the-diagnosis-of-myelodysplastic-syndromes-mds-characteristics-of-refined-mds-types
#11
Corinna Strupp, Kathrin Nachtkamp, Barbara Hildebrandt, Aristoteles Giagounidis, Rainer Haas, Norbert Gattermann, John M Bennett, Carlo Aul, Ulrich Germing
Based on centrally diagnosed 3528 patients in the Düsseldorf registry, we validated the new proposals for the classification of the MDS by the WHO working group: 256 patients were diagnosed as MDSSLD (7,3%), 978 MDSMLD (27,7%), 227 MDS RS SLD (6,4%); 321 MDS RS MLD (9,1%), 159 MDS del(5q) (4,5%), 481 MDSEB 1 (13,6%), 620 MDSEB 2 (17,6%), and 148 MDS-U (4,2%). 352 patients (16,9% of the non blastic types) changed the category, mainly moving from RCMD to MDS RS MLD, RCUD and RCMD to MDS del(5q). Median survival times of the refined groups differed from more than 60 months in the MDSSLD (RS) groups, 37 months in the MDSMLD (RS) groups, 79 months of the MDS del(5q) group and 21 and 11 months in the MDSEB 1 and 2 groups, respectively...
February 27, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28324640/cytogenetics-and-comorbidity-predict-outcomes-in-older-myelodysplastic-syndrome-patients-after-allogenic-stem-cell-transplantation-using-reduced-intensity-conditioning
#12
Orhan Kemal Yucel, Rima M Saliba, Gabriella Rondon, Sairah Ahmed, Amin Alousi, Qaiser Bashir, Stefan O Ciurea, Uday Popat, Isa Khouri, David Marin, Katy Rezvani, Partow Kebriaei, Elizabeth J Shpall, Richard E Champlin, Betül Oran
BACKGROUND: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only treatment with a curative potential for myelodysplastic syndrome (MDS) patients. Allo-HSCT has substantial risks, particularly in the elderly, and its role for older MDS patients has yet to be defined. METHODS: We analyzed 88 MDS patients aged ≥ 60 years with allo-HSCT after reduced intensity conditioning regimens over the last decade. The study cohort had high risk features; 47 of 88 (53...
March 21, 2017: Cancer
https://www.readbyqxmd.com/read/28321924/infectious-complications-in-patients-with-myelodysplastic-syndromes-a-review-of-the-literature-with-emphasis-on-patients-treated-with-5-azacitidine
#13
REVIEW
Markus Radsak, Uwe Platzbecker, Cornelia S Schmidt, Wolf-Karsten Hofmann, Florian Nolte
Myelodysplastic syndromes (MDS) are oligo-clonal diseases of the hematopoietic stem cell compartment resulting in peripheral cytopenias and a tendency of developing acute myeloid leukemia (AML). MDS show an incidence of approximately 4/100.000/year. However, MDS are mainly a disease of the elderly with a sharp increase in incidence in the age decade above 70 years. Due to the demographic change in western countries, it is expected that the MDS will play an increasing role for these health systems. Although anemia is the predominant cytopenia in the majority of patients, about 30-50% of patients suffer from neutropenia of varying severity...
March 21, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28321349/uncovering-clinical-features-of-de-novo-philadelphia-positive-myelodysplasia
#14
Aristides Armas, Chen Chen, Martha Mims, Gustavo Rivero
Myelodysplastic syndrome (MDS) is cytogenetically heterogeneous and retains variable risk for acute myeloid leukemia transformation. Though not yet fully understood, there is an association between genetic abnormalities and defects in gene expression. The functional role for infrequent cytogenetic alteration remains unclear. An uncommon chromosomic abnormality is the presence of the Philadelphia (Ph) chromosome. Here, we report a patient with Ph+ MDS treated with low dose Dasatinib who achieved hematologic response for 7 months...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28321120/decision-analysis-of-allogeneic-hematopoietic-stem-cell-transplantation-for-patients-with-myelodysplastic-syndrome-stratified-according-to-the-revised-international-prognostic-scoring-system-ipss-r
#15
M G Della Porta, C H Jackson, E P Alessandrino, M Rossi, A Bacigalupo, M T van Lint, M Bernardi, B Allione, A Bosi, S Guidi, V Santini, L Malcovati, M Ubezio, C Milanesi, E Todisco, M T Voso, P Musto, F Onida, A P Iori, R Cerretti, G Grillo, A Molteni, P Pioltelli, L Borin, E Angelucci, E Oldani, S Sica, C Pascutto, V Ferretti, A Santoro, F Bonifazi, M Cazzola, A Rambaldi
Allogeneic hematopoietic stem cell transplantation (allo-SCT) represents the only curative treatment for patients with myelodysplastic syndrome (MDS), but involves non-negligible morbidity and mortality. Crucial questions in clinical decision making include the definition of optimal timing of the procedure and the benefit of cytoreduction before transplant in high risk patients. We carried out a decision analysis on 1728 MDS who received supportive care, transplantation or hypomethylating agents (HMAs). Risk assessment was based on the revised International Prognostic Scoring System (IPSS-R)...
March 21, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28321091/azacitidine-therapy-for-low-risk-myelodysplastic-syndrome-developing-after-solid-organ-transplantation
#16
Hiroaki Inoue, Yasuyoshi Morita, Shinya Rai, Hiroaki Kakutani, Yasuyo Ohyama, Yasuhiro Taniguchi, Hirokazu Tanaka, Takahiro Shimada, Yoichi Tatsumi, Takashi Ashida, Itaru Matsumura
Immunosuppressive therapy after solid organ transplantation is known to be a risk factor for the development of myelodysplastic syndromes (MDS). Herein, we report 2 patients, both of whom developed low-risk MDS after solid organ transplantation and were successfully treated with azacitidine (AZA). The 1st case was a 74-year-old man who had received liver transplantation. The initial immunosuppressive therapy consisted of cyclosporine and prednisolone. Nine years after transplantation, he was diagnosed as having MDS (RCMD)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28321087/myelodysplastic-syndrome-diagnosed-on-the-occasion-of-fournier-s-gangrene
#17
Masaaki Adachi, Kimiyoshi Mitsuhashi, Hiroyuki Matsuda, Junko Watanabe, Katsuya Nakanishi
Fournier's gangrene (FG) is a fulminant infective necrotizing fasciitis, which includes the genital, perineal, and perianal regions. A 77-year-old man had previously been diagnosed as having diabetes mellitus (DM) and was treated with pioglitazone (15 mg) and miglitol (150 mg). He developed sudden perineal discomfort, fever with painful penile, and scrotal edema, subsequently leading to urinary retention. According to physical examination and CT scan results for the swollen penis and scrotum, he was diagnosed with FG...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28319072/long-term-follow-up-of-a-retrospective-comparison-of-reduced-intensity-conditioning-and-conventional-high-dose-conditioning-for-allogeneic-transplantation-from-matched-related-donors-in-myelodysplastic-syndromes
#18
R Martino, A Henseler, M van Lint, N Schaap, J Finke, D Beelen, S Vigouroux, E P Alessandrino, G J Mufti, J H Veelken, B Bruno, I Yakoub-Agha, L Volin, J Maertens, R Or, V Leblond, M Rovira, P Kalhs, A F Alvarez, A Vitek, J Sierra, E Wagner, M Robin, T de Witte, N Kröger
This study shows the long-term updated outcomes of a multicenter retrospective study which analyzed 843 patients with myelodysplastic syndrome (MDS) who underwent transplantation with an HLA-identical sibling donor with either reduced-intensity conditioning (RIC) in 213 patients, or standard myeloablative conditioning (MAC) in 630 patients. In multivariate analysis, the 13-year relapse rate was significantly increased after RIC (31% after MAC vs 48% in RIC; HR, 1.5; 95% CI, 1.1-1.9; P=0.04), but with no differences in overall survival (OS) (30% after MAC vs 27% in RIC; P=0...
March 20, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28318095/clinicopathologic-and-molecular-characterization-of-myeloid-neoplasms-with-isolated-t-6-9-p23-q34
#19
V Visconte, S Shetty, B Przychodzen, C Hirsch, J Bodo, J P Maciejewski, E D Hsi, H J Rogers
INTRODUCTION: The t(6;9)(p23;q34);DEK-NUP214 [t(6;9)] abnormality is found in 0.7-1.8% of patients with acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS). FLT3-ITD mutations are detected in t(6;9) patients. The t(6;9) abnormality is associated with poor outcomes. We studied the clinicopathologic and molecular profiles of patients with AML/MDS carrying t(6;9). METHODS: We collected clinical data of nine patients with AML/MDS with isolated t(6;9) (median age = 41 years; male/female = 4/5) and genotyped DNAs using whole exome, Sanger, and targeted sequencing...
March 20, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28314085/targeted-next-generation-sequencing-and-identification-of-risk-factors-in-world-health-organization-defined-atypical-chronic-myeloid-leukemia
#20
Mrinal M Patnaik, Daniela Barraco, Terra L Lasho, Christy M Finke, Kaaren Reichard, Katherine P Hoversten, Rhett P Ketterling, Naseema Gangat, Ayalew Tefferi
Atypical chronic myeloid leukemia (aCML) is an aggressive myeloid neoplasm with overlapping features of myelodysplastic syndromes (prominent granulocytic dysplasia) and myeloproliferative neoplasms (neutrophilic leukocytosis). We studied 25 molecularly-annotated and World Health Organization defined aCML patients; median age 70 years, 84% males. Cytogenetic abnormalities were seen in 36% and gene mutations in 100%. Mutational frequencies were, ASXL1 28%, TET2 16%, NRAS 16%, SETBP1 12%, RUNX1 12%, ETNK1 8% and PTPN11 4%...
March 17, 2017: American Journal of Hematology
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