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European Journal of Haematology

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https://www.readbyqxmd.com/read/28419558/age-but-not-philadelphia-positivity-impairs-outcome-in-older-elderly-patients-with-acute-lymphoblastic-leukemia-in-sweden
#1
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
#2
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
#3
Roman Hájek, Viera Sandecká, Zdeněk Adam, 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, Marta Krejčí, Ján Straub, Jiří Minařík, Luděk Pour, 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 into 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
#4
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/genome-wide-association-study-on-monoclonal-gammopathy-of-unknown-significance-mgus
#5
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 germline variants contributing to the development of monoclonal gammopathy of undetermined significance (MGUS), an asymptomatic pre-malignant precursor for multiple myeloma (MM). METHODS: We conducted the first genome-wide 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
#6
Reinhold Munker, Ali Baghian, Yordanka Koleva, Patricia Andrews, Gunita S Matharoo, Ato E Wright, Nakhle Saba, Roy Weiner, Hanah 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/socio-economic-burden-of-participation-in-clinical-trials-in-patients-with-myeloproliferative-neoplasms
#7
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 section 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
#8
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 mulitple myeloma (RMM). METHODS: Peripheral blood stem cells 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 (1(st) to 3(rd) relapse) treated within the ReLApsE trial of the German-Speaking Myeloma Multicenter Group (GMMG). RESULTS: Mobilization was initiated with high dose cyclophosphamide and G-CSF in 30 patients...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370385/familial-chronic-lymphocytic-leukemia-in-israel-a-disproportionate-distribution-among-ashkenazi-jews
#9
Mor Zada, Daniele Lerner, Yuval Piltz, Chava Perry, Irit Avivi, Yair Herishanu
BACKGROUND: Relatives of patients with chronic lymphocytic leukemia (CLL) are at increased risk of developing CLL. Familial CLL is defined as more than one case of CLL among blood relatives, a phenomenon reported in approximately 5-10% of all CLL patients. OBJECTIVE: Given the known predisposition of CLL among Ashkenazi Jews, we studied the features of familial CLL in an Israeli population. METHODS: This is a retrospective study, in which we reviewed the demographics, clinical characteristics and outcomes of a total of 332 patients with CLL/small lymphocytic lymphoma...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370306/is-allogeneic-stem-cell-transplantation-for-myelofibrosis-still-indicated-at-the-time-of-molecular-markers-and-jak-inhibitors-era
#10
Elsa Lestang, Pierre Peterlin, Yannick Le Bris, Viviane Dubruille, Jacques Delaunay, Catherine Godon, Olivier Theisen, Nicolas Blin, Beatrice Mahe, Thomas Gastinne, Alice Garnier, Cyrille Touzeau, Maud Voldoire, Marie C Bene, Steven Le Gouill, Noel Milpied, Mohamad Mohty, Philippe Moreau, Thierry Guillaume, Patrice Chevallier
OBJECTIVE: The role of allogenic stem cell transplantation (ASCT) is still debated in myelofibrosis. METHOD: A retrospective analyzed was performed to compare the outcome of 71 patients with intermediate-2 or high-risk Dynamic International Prognosis Scoring System+ (DIPSS+) primary (PMF) or secondary (SMF) myelofibrosis with an indication of ASCT as they ultimately underwent the procedure (n=34) or not (n=37). RESULTS: Five-year overall survival (OS) was not statistically different between both groups (allograft: 52% vs no allograft: 34%, p=0,12)...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28349614/progressive-histoplasmosis-with-hemophagocytic-lymphohistiocytosis-and-epithelioid-cell-granulomatosis-a-case-report-and-review-of-the-literature
#11
A B Schulze, B Heptner, T Kessler, B Baumgarten, V Stoica, M Mohr, R Wiewrodt, V Warneke, W Hartmann, J Wüllenweber, C Schülke, M Schäfers, D Wilmes, K Becker, L H Schmidt, A H Groll, W E Berdel
Histoplasmosis in central Europe is a rare fungal disease with diverse clinical presentations. Apart from acute pulmonary histoplasmosis and involvement of the central nervous system, the most serious clinical presentation is progressive disseminated histoplasmosis which is generally associated with severe immunodeficiency and in particular, advanced HIV-infection. Here we report on an immunocompetent female residing in a non-endemic area, presenting with progressive disseminated histoplasmosis after a remote travel history to Thailand and Costa Rica...
March 27, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28332730/multicenter-validation-of-a-simplified-method-for-paroxysmal-nocturnal-hemoglobinuria-screening
#12
Arianna Gatti, Luigi Del Vecchio, Massimo Geuna, Matteo G Della Porta, Bruno Brando
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) diagnostic guidelines recommend single-tube five- to six-color or two-tube four-color assays. PNH clones are detectable in only a fraction of patients at risk, and screening for new PNH cases can be complex and expensive. In this multicenter study, we have validated a simplified, one-tube two-color FLAER-based assay suitable for PNH screening. METHODS: Six laboratories received samples containing spiked PNH leukocyte clones to be analyzed in parallel with a common six-color cocktail (FLAER/CD24/CD45/CD64/CD15/CD14) and a simplified two-color mixture (FLAER/CD15), a shared calibration procedure, and a common analysis protocol...
March 23, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28332238/outcome-measures-for-adult-and-pediatric-hemophilia-patients-with-inhibitors
#13
REVIEW
Cedric Hermans, Günter Auerswald, Gary Benson, Gerry Dolan, Anne Duffy, Victor Jiménez-Yuste, Rolf Ljung, Massimo Morfini, Thierry Lambert, Mehdi Osooli, Silva Zupančić Šalek
Recent advancements in almost all aspects of hemophilia treatment have vastly improved patient care and management, and new and emerging treatments hold the promise of further progress. However, there remains a scarcity of data on long-term outcomes in hemophilia, particularly among those patients with inhibitors, for whom no validated outcome assessment tools are currently available. At the 15(th) Zürich Haemophilia Forum, an expert panel reviewed the most important outcome measures in inhibitor patients and considered the challenges associated with assessing outcomes in this population...
March 22, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28322018/a-systematic-review-of-health-related-quality-of-life-in-longitudinal-studies-of-myeloma-patients
#14
REVIEW
Lene Kongsgaard Nielsen, Mary Jarden, Christen Lykkegaard Andersen, Henrik Frederiksen, Niels Abildgaard
OBJECTIVES: Multiple myeloma (MM) patients report high symptom burden and reduced health-related quality of life (HRQoL) compared to patients with other haematological malignancies. The aim of this review was to analyze published longitudinal studies including MM patients according to a change in HRQoL scores, which is perceived as beneficial to the patient according to two published guidelines. METHODS: A literature search was performed May 2016. Publications with longitudinal follow-up using the EORTC QLQ-C30 instrument for HRQoL measurement of physical functioning, global quality of life, fatigue and/or pain were included...
March 21, 2017: European Journal of Haematology
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
#15
REVIEW
Markus Radsak, Uwe Platzbecker, Cornelia S Schmidt, Wolf-Karsten Hofmann, Florian Nolte
Myelodysplastic Syndromes are oligo-clonal stem cell disorders that are associated with cytopenias in the peripheral blood. Major causes for morbidity and mortality in myelodysplastic syndromes (MDS) patients are infections mostly due to bacteria or fungi. Beside leucopenia per se in affected patients, function of white blood cells particularly that of neutrophils seems to be impaired. Here we summarize the available data on infections in MDS patients in general and particularly those treated with 5-azacitidine...
March 21, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28321922/retrospective-evaluation-of-venous-thromboembolism-vte-are-all-transient-provoking-events-the-same
#16
Chong Chyn Chua, Hui Yin Lim, Mark Tacey, Harshal Nandurkar, Prahlad Ho
OBJECTIVES: Venous thromboembolism (VTE) provoked by transient risk factors has traditionally been classified as a single entity with lower risk of recurrence. We evaluated the association between different categories of transient provoking factors and the relative risk of recurrence. METHODS: Retrospective evaluation of VTE events in non-cancer patients from July 2011 to December 2012 at two tertiary institutions in Australia with a minimum follow up of 24 months...
March 21, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28306177/frequency-and-functional-characterization-of-exhausted-cd8-t-cells-in-chronic-lymphocytic-leukemia
#17
Saeid Taghiloo, Esmaeil Allahmoradi, Mohsen Tehrani, Hadi Hossein-Nataj, Ramin Shekarriz, Ghasem Janbabaei, Saeid Abediankenari, Hossein Asgarian-Omran
OBJECTIVES: The phenotypic and functional properties of Tim-3(+) /PD-1(+) /CD8(+) cells as exhausted T cells were investigated in chronic lymphocytic leukemia (CLL). METHODS: Frequency of CD8(+) /Tim-3(+) /PD-1(+) exhausted cells was determined by flow cytometry. For functional analysis, magnetic beads-isolated CD8(+) T cells were stimulated with PHA and PMA/ionocymin to assess their proliferative responses and cytokine production by MTT and ELISA, respectively...
March 17, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28306171/barriers-to-hydroxyurea-adherence-and-health-related-quality-of-life-in-adolescents-and-young-adults-with-sickle-cell-disease
#18
Sherif M Badawy, Alexis A Thompson, Frank J Penedo, Jin-Shei Lai, Karen Rychlik, Robert I Liem
OBJECTIVES: To identify barriers to hydroxyurea adherence (negative beliefs, access, and/or recall barriers), and their relationship to adherence rates and health-related quality of life (HRQOL) among adolescents and young adults (AYA) with sickle cell disease (SCD). METHODS: A cross-sectional survey was administered to 34 AYAs (12-22 years old) in SCD clinics from January to December 2015. Study measures included Brief Medication Questionnaire, Modified Morisky Adherence Scale 8-items, visual analog scale, and Patient Reported Outcomes Measurement Information System...
March 17, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28306170/impact-of-atrial-fibrillation-on-platelet-gene-expression
#19
Waldemar E Wysokinski, Alfonso Tafur, Naser Ammash, Samuel J Asirvatham, Yanhong Wu, Izabella Gosk-Bierska, Diane E Grill, Joshua P Slusser, Jozef Mruk, Robert D McBane
AIMS: Platelets retain cytoplasmic messenger RNA and are capable of protein biosynthesis. Several diseases are known to impact the platelet transcriptome but the effect of non-valvular atrial fibrillation (NVAF) on platelet RNA transcript is essentially unknown. The aim of this study was to evaluate the impact of NVAF on platelet RNA transcript by measuring platelet genes expression in consecutive NVAF patients before and 3-4 months after pulmonary vein isolation (PVI) and compared to normal sinus rhythm controls (NSR)...
March 17, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28306169/economic-burden-of-relapsed-or-refractory-multiple-myeloma-results-from-an-international-trial
#20
Don Robinson, Robert Z Orlowski, Michael Stokes, Jianming He, Samuel Huse, Abhishek Chitnis, Britte Kranenburg, Annette Lam
OBJECTIVE: The direct cost of relapsed or refractory multiple myeloma (RRMM) is documented; indirect costs are being explored. Healthcare payers seek cost offsets from therapies that improve clinical outcomes but challenge budgets; employers seek lower absenteeism and better productivity. Study goals were to: 1) Identify direct and indirect economic factors of RRMM and, 2) Explore longitudinal relationships between clinical, economic, and health-related quality of life (HRQoL) assessments...
March 17, 2017: European Journal of Haematology
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