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European Journal of Haematology

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https://www.readbyqxmd.com/read/28543758/deregulated-expression-of-long-non-coding-rna-uca1-in-multiple-myeloma
#1
L Sedlarikova, B Gromesova, V Kubaczkova, L Radova, J Filipova, J Jarkovsky, L Brozova, R Velichova, M Almasi, M Penka, R Bezdekova, M Stork, Z Adam, L Pour, M Krejci, P Kuglík, R Hajek, S Sevcikova
OBJECTIVES: Long non-coding RNAs (lncRNAs) are RNA transcripts longer than 200 nucleotides that are not translated into proteins. They are involved in pathogenesis of many diseases including cancer and have a potential to serve as diagnostic and prognostic markers. We aimed to investigate lncRNA expression profiles in bone marrow plasma cells of newly diagnosed multiple myeloma (MM) patients in comparison to normal bone marrow plasma cells of healthy donors (HD) in a 3-phase biomarker study...
May 25, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28544116/incidence-characteristics-and-outcome-of-solitary-plasmacytoma-and-plasma-cell-leukemia-population-based-data-from-the-swedish-myeloma-register
#2
Hareth Nahi, Anna Genell, Göran Wålinder, Katarina Uttervall, Gunnar Juliusson, Karin Forsberg, Markus Hansson, Ronald Svensson, Olle Linder, Kristina Carlson, Bo Björkstrand, Sigurdur Y Kristinsson, Ulf Henrik Mellqvist, Cecilie Blimark, Ingemar Turesson
Solitary plasmacytoma (SP) and Plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP), (n=124) and extramedullary plasmacytoma (EMP), (n=67) have better overall survival (OS) than MM (n=3549)...
May 22, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28543682/residual-thymic-tissue-and-lymph-node-involvement-by-acute-myeloid-leukaemia-presenting-as-mediastinal-strongly-18-fdg-pet-positive-masses
#3
Georg Maschmeyer, Ingo Brink, Doris Jähne, Renate Arnold, Olaf Schega
We report on a multidisciplinary management of a 68-year old AML patient presenting with a PET-positive mediastinal tumour typical for lymph node metastasis. It was removed via minimally invasive thoracoscopic intervention and was identified as a thymus residual infiltrated by AML. Follow-up PET-CT scan after resection and remission induction chemotherapy was completely normal. To our knowledge, this is the first documented case report of AML presenting with PET-positive infiltrates of thymic and lymph node tissue along the aortic bow mimicking a second intrathoracic malignancy...
May 22, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28523822/development-of-a-novel-flow-cytometric-immunobead-array-to-quantify-vwf-ag-and-vwf-gpibr-and-its-application-in-acute-myocardial-infarction
#4
Bin Yan, Mengqiao Xu, Yunxiao Zhao, Haijun Guo, Lijun Xia, Changgeng Ruan, Yiming Zhao
OBJECTIVES: Both von Willebrand disease (VWD) and acute myocardial infarction (AMI) involve quantitative and qualitative changes in von Willebrand factor (VWF). Our objective was to develop a rapid and precise flow cytometric immunobead array (FCIA) to quantify VWF antigen (VWF:Ag) and ristocetin-triggered platelet glycoprotein Ib binding (VWF:GPIbR), and apply it in a clinical setting. METHODS: Microbeads, coated with monoclonal antibodies for SZ29 or SZ151 IgG, were incubated with diluted plasma...
May 18, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28509395/da-epoch-r-for-post-transplant-lymphoproliferative-disorders-ptld
#5
Christin B DeStefano, Vera Malkovska, Hind Rafei, Aarthi Shenoy, Kelly Fitzpatrick, Anita Aggarwal, Joseph P Catlett
Post-transplant lymphoproliferative disorders (PTLD) are a heterogeneous and potentially fatal group of neoplasms arising in an immunodeficient environment in the background of viral antigenic stimulation. PTLD is rare, with an incidence in solid organ transplant recipients typically less than 20% [1]. For aggressive, monomorphic cases, current guidelines recommend reduced immunosuppression in addition to chemoimmunotherapy, which generally consists of R-CHOP [1]. Nevertheless, prognosis is poor with 5-year overall survival (OS) rates of only 40-60% [1]...
May 16, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28504846/adverse-event-management-in-patients-with-relapsed-and-refractory-multiple-myeloma-taking-pomalidomide-plus-low-dose-dexamethasone-a-pooled-analysis
#6
Philippe Moreau, Meletios A Dimopoulos, Paul G Richardson, David S Siegel, Michele Cavo, Paolo Corradini, Katja Weisel, Michel Delforge, Peter O'Gorman, Kevin Song, Christine Chen, Nizar Bahlis, Albert Oriol, Markus Hansson, Martin Kaiser, Pekka Anttila, Reinier Raymakers, Cristina Joao, Gordon Cook, Lars Sternas, Tsvetan Biyukov, Ana Slaughter, Kevin Hong, Jennifer Herring, Xin Yu, Mohamed Zaki, Jesus San-Miguel
OBJECTIVES: Heavily pretreated patients with relapsed and refractory multiple myeloma are susceptible to treatment-related adverse events (AEs). Managing AEs is important to ensure patients continue therapy long enough to receive the best clinical benefit. Data from the MM-002, MM-003, and MM-010 trials were pooled to further characterize the safety profile of pomalidomide plus low-dose dexamethasone and AE management. METHODS: This analysis included 1088 patients who received ≥ 2 prior therapies, including lenalidomide and bortezomib, and progressed ≤ 60 days of last therapy...
May 15, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28470777/cd38-negative-relapse-in-multiple-myeloma-after-daratumumab-based-chemotherapy
#7
Jiri Minarik, Martin Novak, Patrik Flodr, Jana Balcarkova, Miroslava Mlynarcikova, Petra Krhovska, Tomas Pika, Zuzana Pikalova, Jaroslav Bacovsky, Vlastimil Scudla
We present a case report of a patient relapsing after anti-CD38 treatment (daratumumab). The phenotype of the disease changed during this treatment and the myeloma clone became CD38 negative and daratumumab refractory. We expected clonal shift, however, based on immunophenotyping, cytogenetics and arrayCGH, the clone was identical as before daratumumab based treatment with the exception of CD38 negativity. We suggest that the downregulation or loss of CD38 might be an epigenetic "escape mechanism" of malignant plasma cells from antibody based treatment...
May 4, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28467652/potential-roles-of-mirnas-and-their-target-genes-in-human-multiple-myeloma
#8
Yong Yang, Jisheng Lin, Zhao Ma, Jinjun Li, Dong Li, Bingqiang Wang, Qi Fei
OBJECTIVES: The etiology of multiple myeloma (MM) is unknown and it remains incurable. We sought to elucidate the mechanisms underlying miRNAs involvement in MM pathogenesis. METHODS: Public mRNA and miRNA expression datasets for MM were collected from the Gene Expression Omnibus database. By integrated bioinformatics analysis, the expression signatures were identified and the miRNA-mRNA interaction network was constructed. The potential functions of target genes were then explored by functional enrichment analysis...
May 3, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28467615/management-of-patients-with-malignancies-and-secondary-immunodeficiencies-treated-with-immunoglobulins-in-clinical-practice-long-term-data-of-the-signs-study
#9
Marcel Reiser, Michael Borte, Dörte Huscher, Ulrich Baumann, David Pittrow, Claudia Sommer, Martin Stangel, Maria Fasshauer, Ralf Gold, Manfred Hensel
OBJECTIVE: We aimed to describe the current management and outcomes of patients with secondary immunodeficiencies (SID) on intravenous (IV) or subcutaneous (SC) immune-globulins (IG) as maintenance therapy to prevent infections. METHODS: Non-interventional, prospective study (average follow-up 20.5 months). RESULTS: Of the 307 SID patients (mean age 63.7 ± 14.4 years, 52% males, in 31% IG newly initiated), 95.4% received IV (mean dosing interval 4...
May 3, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28452421/paroxysmal-nocturnal-hemoglobinuria-clones-are-not-infrequent-in-patients-with-inherited-bone-marrow-failure-syndromes
#10
LETTER
Sreejesh Sreedharanunni, Neelam Varma, Man Updesh Singh Sachdeva, Shano Naseem, Pankaj Malhotra, Deepak Bansal, Anil Sood, Parveen Bose, Subhash Varma
The distinction of inherited bone marrow failure syndromes (IBMFS) from other acquired forms of aplastic anemia (AA) is very important for instituting proper therapy. However this is not often straight forward. A proper confirmatory genetic diagnosis is possible only in less than 50% of the patients even with the use of massive parallel sequencing (1). Moreover its application is still limited due to the cost and several logistical issues. Unfortunately, diethyl epoxy butane (DEB) or mitomycin C induced chromosome breakage studies (CBS) is the only test, which is widely employed, for the diagnosis of IBMFS in many resource-limited settings...
April 28, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28452416/low-crebbp-expression-is-associated-with-adverse-long-term-outcomes-in-paediatric-acute-lymphoblastic-leukaemia
#11
Chao Gao, Rui-Dong Zhang, Shu-Guang Liu, Xiao-Xi Zhao, Lei Cui, Zhi-Xia Yue, Wei-Jing Li, Zhen-Ping Chen, Zhi-Gang Li, Qing Rao, Min Wang, Hu-Yong Zheng, Jian-Xiang Wang
OBJECTIVES: CREBBP alterations are associated with many diseases including leukaemia. However, CREBBP expression and its clinical relevance in paediatric acute lymphoblastic leukaemia have not been elucidated. METHODS: We studied CREBBP mRNA expression in 349 patients treated with either the BCH-2003 or CCLG-2008 protocol. Using a receiver operating characteristic curve, patients were divided into low- or high-CREBBP. The association among clinicobiological characteristics, outcomes, and CREBBP level was analysed...
April 28, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28449187/distinctive-clinical-characteristics-and-favorable-outcomes-in-patients-with-large-granular-lymphocytosis-after-allo-hct-12-years-follow-up-data
#12
Marc Poch Martell, Nada Hamad, Elizabeth Shin, Joon Ho Moon, Sang Kyun Sohn, Jieun Uhm, Fotios V Michelis, Auro Viswabandya, Jeffrey H Lipton, Hans A Messner, Dennis Dong Hwan Kim
An increase in large granular lymphocytes (LGL) is frequently seen in patients following allogeneic hematopoietic cell transplantation (allo-HCT) and it has been associated with better outcomes in some reports. We assessed 826 consecutive patients at our institution with over 12 years of follow-up for the occurrence of LGL lymphocytosis after allo-HCT. The 3 year cumulative incidence of LGL lymphocytosis was 14.5% with a median duration of over 3.5 years. The developmnet of LGL lymphocytosis was strongly correlated with CMV viremia and GVHD...
April 27, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28419558/age-but-not-philadelphia-positivity-impairs-outcome-in-older-elderly-patients-with-acute-lymphoblastic-leukemia-in-sweden
#13
Piotr Kozlowski, Emma Lennmyr, Lucia Ahlberg, Per Bernell, Erik Hulegårdh, Holger Karbach, Karin Karlsson, Beata Tomaszewska-Toporska, Maria Åström, Heléne Hallböök
OBJECTIVES: Older/elderly patients with acute lymphoblastic leukemia (ALL) are poorly represented in clinical trials. METHODS: Using Swedish national leukemia registries, we investigated disease/patient characteristics, treatment choices, outcome, and the impact of an age-adapted protocol (introduced in 2009) in this population-based study of patients aged 55-85 years, diagnosed with ALL 2005-2012. RESULTS: Of 174 patients, 82% had B-phenotype, 11% Burkitt leukemia (excluded), and 7% T-phenotype...
April 18, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28401591/dynamics-of-bkpyv-reactivation-and-risk-of-hemorrhagic-cystitis-after-allogeneic-hematopoietic-stem-cell-transplantation
#14
Konstantin Höller, Lavinia Fabeni, Marco Herling, Udo Holtick, Christof Scheid, Elena Knops, Nadine Lübke, Rolf Kaiser, Herbert Pfister, Veronica Di Cristanziano
OBJECTIVES: Aim of this retrospective study was to analyze the dynamics of BKPyV reactivation in allogeneic hematopoietic stem cell transplant recipients in order to identify patients with higher risk to develop BKPyV-associated hemorrhagic cystitis (BKPyV-associated HC). METHODS: The study included 58 allo-HSCT recipients from the University Hospital of Cologne detected BKPyV positive by real-time PCR between 2009 and 2015. For correlative analysis, the first detected BKPyV-DNA load in urine, in plasma, as well as the onset and severity of HC following the first day of conditioning regimen were considered...
April 12, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28384387/a-first-czech-analysis-of-1887-cases-with-monoclonal-gammopathy-of-undetermined-significance
#15
Viera Sandecká, Roman Hájek, Luděk Pour, Ivan Špička, Vlastimil Ščudla, Evžen Gregora, Jakub Radocha, Lenka Walterová, Petr Kessler, Lenka Zahradová, Dagmar Adamová, Kamila Valentova, Ivan Vonke, Jarmila Obernauerová, David Starostka, Marek Wróbel, Lucie Brožová, Jiří Jarkovský, Aneta Mikulášová, Lucie Říhová, Sabina Ševčíková, Ján Straub, Jiří Minařík, Zdeněk Adam, Marta Krejčí, Zdeněk Král, Vladimír Maisnar
INTRODUCTION: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant condition with a risk of malignant conversion. PATIENTS AND METHODS: With the aim to estimate the cumulative risk MGUS progression to hematologic malignancies, we analyzed a nationwide population-based cohort of 1887 MGUS patients from the Czech Registry of Monoclonal Gammopathies (RMG) between 2007 and 2013. RESULTS: During the follow-up period (median 4 years; range 0...
April 6, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28382662/successful-targeted-treatment-of-mast-cell-activation-syndrome-with-tofacitinib
#16
Lawrence B Afrin, Roger W Fox, Susan L Zito, Leo Choe, Sarah C Glover
Mast cell (MC) activation syndrome (MCAS) is a collection of illnesses of inappropriate MC activation with little to no neoplastic MC proliferation, distinguishing it from mastocytosis. MCAS presents as chronic, generally inflammatory multisystem polymorbidity likely driven in most by heterogeneous patterns of constitutively activating mutations in MC regulatory elements, posing challenges for identifying optimal mutation-targeted treatment in individual patients. Targeting commonly affected downstream effectors may yield clinical benefit independent of upstream mutational profile...
April 6, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28375557/genomewide-association-study-on-monoclonal-gammopathy-of-unknown-significance-mgus
#17
Hauke Thomsen, Chiara Campo, Niels Weinhold, Miguel Inacio da Silva Filho, Luděk Pour, Evžen Gregora, Pavel Vodicka, Ludmila Vodickova, Per Hoffmann, Markus M Nöthen, Karl-Heinz Jöckel, Christian Langer, Roman Hajek, Hartmut Goldschmidt, Kari Hemminki, Asta Försti
OBJECTIVES: To identify germ line variants contributing to the development of monoclonal gammopathy of undetermined significance (MGUS), an asymptomatic premalignant precursor for multiple myeloma (MM). METHODS: We conducted the first genomewide association study (GWAS) on MGUS on 243 German cases with a replication on 294 Czech cases. Identified loci were further analyzed in 1508 German MM patients. New MM loci recently reported in a meta-analysis were also tested in the MGUS GWAS...
April 4, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370630/long-term-follow-up-of-patients-with-multiple-myeloma-treated-with-total-body-irradiation-melphalan-conditioning
#18
Reinhold Munker, Ali Baghian, Yordanka Koleva, Patricia Andrews, Gunita S Matharoo, Ato E Wright, Nakhle S Saba, Roy S Weiner, Hana Safah
BACKGROUND AND OBJECTIVES: Since a study published in 2002 showed a survival advantage of melphalan-only conditioning for stem cell transplantation (HSCT) over melphalan-total body irradiation (mel-TBI) in patients with multiple myeloma (MM), most centers abandoned mel-TBI. Mel-TBI causes more early toxicity and is more complicated to administer, but we speculated it may result in longer term survival with radiation as an independent treatment modality. Therefore, we analyzed the long-term outcome of patients with MM who received mel-TBI as part of conditioning at our center...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370510/socioeconomic-burden-of-participation-in-clinical-trials-in-patients-with-myeloproliferative-neoplasms
#19
Swati Goel, Chiara Paoli, Alessandra Iurlo, Arturo Pereira, Fabio Efficace, Tiziano Barbui, Ayalew Tefferi, Alessandro M Vannucchi, Francisco Cervantes
OBJECTIVE: To determine the financial and psycho-social impact of participation in clinical trials of patients with BCR/ABL-negative myeloproliferative neoplasms (MPN). METHODS: An international, observational cross-sectional study was performed in 143 consecutive MPN patients (51% myelofibrosis, 36% polycythemia vera, 13% essential thrombocythemia), 68% from Italy, 17% from USA, and 15% from Spain. RESULTS: Thirty-five percent of patients reported having spent more money during the trial than in previous treatments and 21% having missed more workdays...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370401/cyclophosphamide-based-stem-cell-mobilization-in-relapsed-multiple-myeloma-patients-a-subgroup-analysis-from-the-phase-iii-trial-relapse
#20
Marc-Andrea Baertsch, Jana Schlenzka, Katharina Lisenko, Julia Krzykalla, Natalia Becker, Katja Weisel, Richard Noppeney, Hans Martin, Hans W Lindemann, Mathias Haenel, Axel Nogai, Christof Scheid, Hans Salwender, Roland Fenk, Ullrich Graeven, Peter Reimer, Martin Schmidt-Hieber, Martin Goerner, Ingo G H Schmidt-Wolf, Stefan Klein, Anthony D Ho, Hartmut Goldschmidt, Patrick Wuchter
OBJECTIVE: Analysis of the efficiency and toxicity of cyclophosphamide-based stem cell mobilization in patients with relapsed multiple myeloma (RMM). METHODS: Peripheral blood stem cells (PBSCs) were mobilized with high dose cyclophosphamide (2 g/m(2) daily on days 1 and 2) and G-CSF plus pre-emptive/rescue plerixafor in RMM patients (first to third relapse) treated within the ReLApsE trial of the German-Speaking Myeloma Multicenter Group (GMMG). RESULTS: Mobilization was initiated with high-dose cyclophosphamide (HD-CY) and G-CSF in 30 patients...
March 31, 2017: European Journal of Haematology
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