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myelodysplastic syndrome review

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https://www.readbyqxmd.com/read/29474870/improving-revised-international-prognostic-scoring-system-ipss-r-pre-allogeneic-stem-cell-transplant-does-not-translate-into-better-post-transplant-outcomes-for-patients-with-myelodysplastic-syndromes-a-single-center-experience
#1
Musa Alzahrani, Maryse Power, Yasser Abou Mourad, Michael Barnett, Raewyn Broady, Donna Forrest, Alina Gerrie, Donna Hogge, Stephen Nantel, David Sanford, Kevin Song, Heather Sutherland, Cynthia Toze, Thomas Nevill, Sujaatha Narayanan
BACKGROUND: The natural history of patients with myelodysplastic syndromes (MDS) is variable. The revised international prognostic score (IPSS-R) is commonly used in practice to predict outcome in patients with MDS both at diagnosis and pre-transplant. However the effect of change in IPSS-R pre-allogeneic stem cell transplant with chemotherapy or hypomethylating agents on post-transplant outcomes is currently unknown. AIM: We assessed if improvement in IPSS-R prognostic score pre HSCT would result in improvement in clinical outcomes post-transplant...
February 20, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29467484/we-skip-to-work-alternative-splicing-in-normal-and-malignant-myelopoiesis
#2
REVIEW
Alex C H Wong, John E J Rasko, Justin J-L Wong
Alternative splicing expands the transcriptome thereby promoting protein diversity. It governs critical cellular processes such as differentiation, proliferation and apoptosis in a tissue-specific manner. Aberrant splicing consequent to mutations in splicing factors and disruption of isoform ratios in key regulatory genes provides an important contribution to the pathogenesis of the myelodysplastic syndromes and myeloid leukemia. We review here the central role of alternative splicing in regulating myelopoiesis, and provide clear examples of how global splicing disruption or specific aberrant splicing events might promote leukemogenesis...
January 30, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29458780/immune-dysregulation-in-myelodysplastic-syndrome-clinical-features-pathogenesis-and-therapeutic-strategies
#3
REVIEW
Cong Wang, Yan Yang, Sujun Gao, Jingcheng Chen, Jinyuan Yu, Han Zhang, Mingxi Li, Xingying Zhan, Wei Li
Myelodysplastic syndrome (MDS) is a heterogeneous hematological malignancy, characterized by cytopenia and accompanied by a risk of transformation into acute myeloid leukemia (AML). Epidemiological studies for decades have shown association between autoimmune diseases (AIDs) and MDS. Specifically, patients with antecedent AIDs tends to have an increased risk of developing MDS, and these patients display different clinical characteristics and outcomes. Importantly, immune dysregulation has been the common driving force between MDS and AIDs pathogenesis...
February 2018: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/29445113/a-decade-of-progress-in-myelodysplastic-syndrome-with-chromosome-5q-deletion
#4
REVIEW
Alan List, Benjamin L Ebert, Pierre Fenaux
There are few instances in oncology where reciprocal clinical and laboratory translation studies have accelerated the understanding of disease biology and treatment more so than the decade following the Food and Drug Administration (FDA) approval of lenalidomide (Revlimid TM ; Celgene Corporation, Summit, NJ, USA) for the treatment of patients with myelodysplastic syndrome (MDS) and chromosome 5q deletion (del(5q)). Lenalidomide was approved by the FDA in December 2005 on the merits of a multicenter phase 2 study, which demonstrated sustained and prolonged transfusion independence in the majority of participants...
January 30, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29435332/more-is-less-less-is-more-or-does-it-really-matter-the-curious-case-of-impact-of-azacitidine-administration-schedules-on-outcomes-in-patients-with-myelodysplastic-syndromes
#5
Rory M Shallis, Amer M Zeidan
Myelodysplastic syndromes (MDS) encompass a diverse group of hematologic disorders characterized by ineffective and malignant hematopoiesis, peripheral cytopenias and significantly increased risk of progression to acute myeloid leukemia (AML). The hypomethylating agents (HMA) azacitidine and decitabine induce meaningful clinical responses in a significant subset of patients with MDS. Though never compared directly with decitabine, only azacitidine has improved overall survival (OS) compared to conventional care in a randomized trial in patients with higher-risk MDS...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29435331/systematic-review-of-azacitidine-regimens-in-myelodysplastic-syndrome-and-acute-myeloid-leukemia
#6
Roman M Shapiro, Alejandro Lazo-Langner
Background: 5-Azacitidine administered as a 7-day dosing regimen (7-0-0) is approved in high risk IPSS myelodysplastic syndrome (MDS) patients. Alternative regimens such as a 5-day (5-0-0) or 7-day with a weekend break (5-2-2) are commonly used. No randomized controlled trial has been done directly comparing all three dosing regimens. The objective of this study was to compare the efficacies of the 5-0-0, 5-2-2, and 7-0-0 regimens in MDS and AML. Methods: A systematic review was conducted using MEDLINE, EMBASE and CENTRAL...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29416365/systematic-review-of-health-state-utility-values-for-acute-myeloid-leukemia
#7
Anna Forsythe, Patricia S Brandt, Mike Dolph, Sachin Patel, Adrian Paul J Rabe, Gabriel Tremblay
Background: Cost-utility analyses for acute myeloid leukemia (AML) require health state utility values (HSUVs) in order to calculate quality-adjusted life-years (QALYs) for each health state. Aim: This study reviewed AML-related HSUVs that could be used in economic evaluation studies. Materials and methods: EMBASE, MEDLINE, and Cochrane databases were searched from January 2000 to November 2016 for relevant studies that reported quality of life (QoL) and HSUVs in AML...
2018: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/29414400/primary-cutaneous-histiocytic-sarcoma-a-report-of-five-cases-with-primary-cutaneous-involvement-and-review-of-the-literature
#8
Cynthia M Magro, Najiyah Kazi, Aimee E Sisinger
INTRODUCTION: Histiocytic sarcoma is an extremely rare hematologic malignancy of histiocytic origin. Five cases of primary cutaneous histiocytic sarcoma are presented. MATERIALS AND METHODS: Cases of primary cutaneous histiocytic sarcoma were identified using a natural language search from the dermatopathology data base of Cornell University. RESULTS: There was a male predominance (4 males and 1 female) ranging in age from 33years to 92years (mean age of 73years); all presented with a solitary nodule which involved the head and neck area in four and thigh in one...
February 2018: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29394399/epidemiology-of-invasive-aspergillosis-and-triazole-resistant-aspergillus-fumigatus-in-patients-with-haematological-malignancies-a-single-centre-retrospective-cohort-study
#9
P P Lestrade, W J F M van der Velden, F Bouwman, F J Stoop, N M A Blijlevens, W J G Melchers, P E Verweij, J P Donnelly
Objectives: To investigate the epidemiology and clinical relevance of triazole resistance among patients undergoing treatment for haematological malignancies who are at risk of invasive aspergillosis (IA). Methods: This was a retrospective cohort study for which the records of consecutive patients given chemotherapy for AML or myelodysplastic syndrome (MDS) or who had received an allogeneic HSCT from 2006 to 2012 were reviewed for IA. Triazole resistance was detected by the VIPcheck™ screening method and confirmed by determining the MIC by EUCAST methodology...
January 31, 2018: Journal of Antimicrobial Chemotherapy
https://www.readbyqxmd.com/read/29382003/hemoglobin-hornchurch-%C3%AE-43-cd2-glu-lys-hbb-c-130g-a-in-a-chinese-boy-complicated-with-thrombocytopenia-a-case-report-and-literature-review
#10
Huiping Shi, Zhaoyue Wang
RATIONALE: Hemoglobin Hornchurch is regarded as an asymptomatic hemoglobinopathy with no obvious hematological or clinical abnormalities. Recently, we identified hemoglobin Hornchurch in a 13-year-old Chinese boy complicated with thrombocytopenia, which displayed instability in isopropanol precipitation test. PATIENT CONCERNS: In this case report, we reported a Chinese boy with hemoglobin Hornchurch complicated by thrombocytopenia. The patients have been misdiagnosed as aplastic anemia and myelodysplastic syndrome before...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29372845/myeloid-neoplasm-with-germline-predisposition-a-2016-update-for-pathologists
#11
Juehua Gao, Shunyou Gong, Yi-Hua Chen
CONTEXT: - Myeloid neoplasms with familial occurrence have been rarely reported in the past. With the advance of molecular technology and better understanding of the molecular pathogenesis of myeloid neoplasms, investigating the genetic causes of familial acute myeloid leukemia or myelodysplastic syndrome has become feasible in the clinical setting. Recent studies have identified a rapidly expanding list of germline mutations associated with increased risks of developing myeloid neoplasm in the affected families...
January 26, 2018: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/29368649/secondary-pulmonary-alveolar-proteinosis-a-single-center-retrospective-study-a-case-series-and-literature-review
#12
Dongmei Zhang, Xinlun Tian, Ruie Feng, Xiaobei Guo, Peng Wang, Yusen Situ, Yi Xiao, Kai-Feng Xu
BACKGROUND: Secondary pulmonary alveolar proteinosis (sPAP) is an extremely rare disease. The clinical features of sPAP patients remain to be summarizeds. METHODS: Patients pathologically diagnosed with PAP and with negative results for anti-granulocyte macrophage colony stimulating factor (GM-CSF) autoantibodies from Peking Union Medical College Hospital between January 2000 and July 2016 were retrospectively studied. The PubMed database was also searched for literature to collect published cases...
January 25, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29332607/the-role-of-t2-weighted-gradient-echo-in-the-diagnosis-of-tumefactive-intrahepatic-extramedullary-hematopoiesis-in-myelodysplastic-syndrome-and-diffuse-hepatic-iron-overload-a-case-report-and-review-of-the-literature
#13
Abel A Belay, Andrew M Bellizzi, Alan H Stolpen
BACKGROUND: Extramedullary hematopoiesis is the proliferation of hematopoietic cells outside bone marrow secondary to marrow hematopoiesis failure. Extramedullary hematopoiesis rarely presents as a mass-forming hepatic lesion; in this case, imaging-based differentiation from primary and metastatic hepatic neoplasms is difficult, often leading to biopsy for definitive diagnosis. We report a case of tumefactive hepatic extramedullary hematopoiesis in the setting of myelodysplastic syndrome with concurrent hepatic iron overload, and the role of T2*-weighted gradient-echo magnetic resonance imaging in differentiating extramedullary hematopoiesis from primary and metastatic hepatic lesions...
January 15, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29332326/carcinogenic-potential-of-antitumor-therapies-is-the-risk-predictable
#14
Ivanka Nenova, Janet Grudeva-Popova
The growing number of successfully cured cancer patients has created a new field in oncogenesis. The life expectancy of such patients has increased, however this favorable event may create enough time for epigenetic events to occur which can cause a new carcinognic event, i.e. a secondary malignancy. The terms in use are second primary malignancies as well as therapy-related neoplasms in case the treatment of the first neoplasm is a direct cause. Second primary malignancies can be hematological neoplasms or solid tumors, with solid tumors having higher frequency...
November 2017: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/29331736/dysfunctional-telomeres-and-hematological-disorders
#15
REVIEW
Elena Fiorini, Andrea Santoni, Simona Colla
Telomere biology disorders, which are characterized by telomerase activity haploinsufficiency and accelerated telomere shortening, most commonly manifest as degenerative diseases. Tissues with high rates of cell turnover, such as those in the hematopoietic system, are particularly vulnerable to defects in telomere maintenance genes that eventually culminate in bone marrow (BM) failure syndromes, in which the BM cannot produce sufficient new blood cells. Here, we review how telomere defects induce degenerative phenotypes across multiple organs, with particular focus on how they impact the hematopoietic stem and progenitor compartment and affect hematopoietic stem cell (HSC) self-renewal and differentiation...
January 4, 2018: Differentiation; Research in Biological Diversity
https://www.readbyqxmd.com/read/29311715/the-bone-marrow-niche-in-mds-and-mgus-implications-for-aml-and-mm
#16
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...
January 9, 2018: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/29296910/aging-hematopoiesis-and-the-myelodysplastic-syndromes
#17
REVIEW
Stephen S Chung, Christopher Y Park
The aging hematopoietic system undergoes numerous changes, including reduced production of red blood cells and lymphocytes as well as a relative increase in the production of myeloid cells. Emerging evidence indicates that many of these changes are due to selection pressures from cell-intrinsic and cell-extrinsic factors that result in clonal shifts in the hematopoietic stem cell (HSC) pool, resulting in predominant HSC clones that exhibit the functional characteristics associated with HSC aging. Given the recent descriptions of clonal hematopoiesis in aged populations, the increased risk of developing hematologic malignancies in individuals with clonal hematopoiesis, and the many similarities in hematopoietic aging and acquired bone marrow failure (BMF) syndromes, such as myelodysplastic syndromes (MDS), this raises significant questions regarding the relationship between aging hematopoiesis and MDS, including the factors that regulate HSC aging, whether clonal hematopoiesis is required for the development of MDS, and even whether BMF is an inevitable consequence of aging...
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296795/erythropoiesis-stimulating-agents-in-elderly-patients-with-anemia-response-and-cardiovascular-outcomes
#18
Zachary Gowanlock, Swetha Sriram, Alison Martin, Anargyros Xenocostas, Alejandro Lazo-Langner
A specific cause of anemia cannot be identified in many elderly patients. Erythropoiesis-stimulating agents (ESAs) may play a role in treating these patients with anemia of unknown etiology (AUE). This study examines hemoglobin and cardiovascular outcomes among elderly anemic patients treated with ESAs. We conducted a retrospective cohort study that included all anemic patients older than age 60 years who had erythropoietin (EPO) measured between 2005 and 2013 at a single center. Three independent reviewers used defined criteria to assign each patient's anemia to 1 of 4 groups: chronic kidney disease (CKD), myelodysplastic syndrome, AUE, or other etiology...
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/29273914/myeloid-neoplasms-with-t-12-22-p13-q12-mn1-evt6-a-systematic-review-of-12-cases
#19
Haigang Shao, Jiannong Cen, Suning Chen, Huiying Qiu, Jinlan Pan
t(12;22)(p13;q12) is a rare but recurrent chromosomal abnormality involving the ETS transcription factor ETV6 and meningioma 1 (MN1) genes. In this study, we analyzed the clinical, cytogenetic, and molecular features of five new patients with the t(12;22)/MN1-EVT6 who presented with acute myeloid leukemia or chronic myelomonocytic leukemia. We subsequently reviewed the literature and identified seven additional cases reported with t(12;22)/MN1-EVT6. Our data suggest that neoplasms carrying the t(12;22)/MN1-ETV6, although rare, can commonly present as myeloid neoplasms at the initial diagnosis, including acute myeloid leukemia (n = 8), myelodysplastic syndrome (n = 2), and myelodysplastic/myeloproliferative neoplasms (n = 2)...
December 22, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29243741/impact-of-copy-neutral-loss-of-heterozygosity-and-total-genome-aberrations-on-survival-in-myelodysplastic-syndrome
#20
Cecilia C S Yeung, Scott McElhone, Xue Yan Chen, David Ng, Barry E Storer, H Joachim Deeg, Min Fang
Myelodysplastic syndromes (MDS) are a heterogeneous group of diseases with varying genetic aberrations. Half of MDS patients have normal karyotype, obscuring the underlying condition indicating a need for new markers for improved diagnostics and prognosis. We performed a retrospective review of sequential MDS patients who underwent chromosomal genetic array testing (CGAT) between November 2008 and March 2014. Total Genomic Aberration (TGA) scores, with and without copy-neutral loss of heterozygosity (cnLOH), were compared to pathology and clinical data...
December 15, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
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