tbuckley/cabot-search huggingface dataset:
THU0499 Neutrophil Extracellular Traps (NETS): A Shared Feature of Acute Microcrystalline Arthritis
Authors: M. Bardelli, S. Lorenzini, C. Baldi, A. Simpatico, A. Gamberucci, G. Berti, E. Garcia Gonzalez, M. Galeazzi, E. Selvi
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Neutrophil extracellular traps (NETs) are web-like structures, composed of nucleic acids, histones and granular enzymes, including myeloperoxidase and elastase, that have a physiological crucial role in innate immunity response by trapping microbes. In addition, NETs have been identified as regulators of many inflammatory and autoimmune disorders, including crystal-induced arthritis.[1,2] In particular, a key role in the pathogenesis of gout flares has been postulated for NETs. During gout attacks, NET aggregates should package MSU crystals with a concomitant degradation of proinflammatory cytokines, leading to the rapid resolution of the inflammatory phase.[3] <h3>Objectives</h3> To evaluate NET expression in synovial fluid from acute CPPD arthritis and to compare NET release between acute MSU and CPPD arthritis. <h3>Methods</h3> Synovial fluid was collected from patients with active gout or pseudogout attack (n=5 for each group) and immediately centrifuged (805 rad) to separate cells from supernatant. <i>Polymorphonuclear leukocytes</i> were counted and immediately processed for morphological evaluation under transmission electron microscopy (Zeiss EM 109; 4000x). NET expression was evaluated by fluorescence microscopy using SYTOX Green (Life Technologies) and neutrophil elastase specific antibody (ABCAM). Fluorometric quantification of NET components, DNA and neutrophil elastase, was performed in supernatant (Cary Eclipse Varian; exc/em 503/526). Results were normalized to cells/mm<sup>3</sup>. Peripheral blood neutrophils from healthy donors stimulated with PMA were used as positive controls whereas PMA-untreated neutrophils were used as negative ones. All the experiments were performed according with Helsinki guidelines. <h3>Results</h3> Extracellular material morphologically compatible with NETs was observed by electron microscopy in samples from both acute MSU and CPPD arthritis. NET structures were confirmed by the presence of extracellular DNA and neutrophil elastase at immunofluorescence. Fluorometric quantification of extracellular DNA and neutrophil elastase showed an increased NET release in both gout and pseudogout samples compared with negative controls (p&lt;0.01). No significant differences in NET release were measured between acute MSU and CPPD arthritis samples. <h3>Conclusions</h3> Beyond confirming NETs in acute MSU arthritis, we have evidence of NET release in synovial fluid from acute CPPD arthritis. Our preliminary data suggest that the release of NETs could be a common physiophatological pathway to both crystral induced arthropaties. <h3>Disclosure of Interest</h3> None declared
Inhibition of the Pan-Bcl-2 Family by the Small Molecule GX15-070 Induces Apoptosis in Mantle Cell Lymphoma (MCL) Cells and Enhances the Activity of Two Proteasome Inhibitors (NPI-0052 and Bortezomib), and Doxorubicin Chemotherapy.
Authors: Victor Y. Yazbeck, Georgios V. Georgakis, Yang Li, David McConkey, Michael Andreeff, Anas Younes
Journal: Blood (2006)
An In Vivo Evidence That the CD45negative Adult Marrow-Derived CXCR4+ SSEA-1+ OCT-4+ Very Small Embryonic-Like (VSEL) Stem Cells May Differentiate into CD45positive Long Term Repopulating Hematopoietic Stem Cells.
Authors: Marcin Wysoczynski, Magda Kucia, Ewa K. Zuba-Surma, Wu Wan, Mariusz Z. Ratajczak, Janina Ratajczak
Journal: Blood (2007)
AB0650 Survival and Organ Involvement in Patients with Limited Cutaneous Systemic Sclerosis and Anti-Topoisomerase Antibodies
Authors: P. Kranenburg, W.M.T. Van Den Hombergh, H.K. Knaapen-Hans, F.H. van den Hoogen, J. Fransen, M.C. Vonk
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> In systemic sclerosis (SSc) there are two main subgroups based on skin involvement: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). LcSSc is associated with the presence of anti-centromere antibodies (ACA) and a more subtle disease course, whereas dcSSc is associated with anti-topoisomerase antibodies (ATA) and often a more severe disease course. Nevertheless, despite the associations between clinical subtype and autoantibodies, there are quite a number of SSc patients who have limited skin involvement and antibodies associated to diffuse skin involvement (ATA). The question is whether these patients have a disease more like lcSSc or more like dcSSc. <h3>Objectives</h3> The aim of this study is to determine whether survival and organ involvement in lcSSc patients who are ATA positive are similar to lcSSc ATA negative patients or to dcSSc ATA positive patients. Furthermore, the relation between ATA positivity and time to transition from lcSSc to dcSSc was evaluated. <h3>Methods</h3> Data from The Nijmegen Systemic Sclerosis cohort were used. Survival analysis was performed to show cumulative survival and time to event for Interstitial Lung Disease (ILD), Pulmonary Arterial Hypertension (PAH), cardiac involvement, Scleroderma Renal Crisis (SRC), and transition to lcSSc. Cox proportional hazard modelling was performed to adjust for confounders. <h3>Results</h3> Survival up to 15 years of follow-up was highest in lcSSc ATA positive patients, whereas dcSSc ATA positive patients had lowest survival (table 1). Interstitial Lung Disease (ILD) occurred more frequently in lcSSc ATA positive patients compared to lcSSc ATA negative patients. However, a higher frequency of ILD was seen dcSSc ATA positive patients. No significant differences were seen between the four (table 1) subgroups in occurrence of PAH, cardiac involvement and SRC. At the moment of diagnosis, 343 patients were classified as lcSSc and 48 of these patients evolved from lcSSc to dcSSc, 24 (9%) in the ATA negative subgroup and 24 (29%) in the ATA positive subgroup (figure 1). <h3>Conclusions</h3> This study showed that lcSSc ATA positive patients differ from lcSSc ATA negative patients and dcSSc ATA positive patients concerning both survival and occurrence of ILD. Therefore, in everyday clinical practice, the prognosis of lcSSc ATA positive patients should be considered separately. <h3>Disclosure of Interest</h3> None declared
A Prospective Study on Incidence, Risk Factors and Long-Term Outcome of Osteonecrosis in 694 Patients with Pediatric Acute Lymphoblastic Leukemia,
Authors: Mariël L. te Winkel, Rob Pieters, Wim C.J. Hop, Hester A. de Groot-Kruseman, Maarten H. Lequin, Inge M. van der Sluis, Jos P.M. Bökkerink, Jan A. Leeuw, Marrie C. A. Bruin, R. Maarten Egeler, Anjo J.P. Veerman, Marry M. van den Heuvel-Eibrink
Journal: Blood (2011)
Diagnostic Utility of PRV-1 mRNA Quantification in Polycythemia Vera and Essential Thrombocythemia.
Authors: Johan Billiet, Sofie Tilleman, Jan Van Droogenbroeck, Friedel Nollet, Dominik Selleslag, Kurt Geldhof, Melanny Hidajat, Achiel Van Hoof, Arnold Criel
Journal: Blood (2004)
Bortezomib Has No. Ex Vivo Activity in Chronic Myeloid Leukemia: Individual Tumor Response Testing Comparative Study in Pediatric and Adult Acute and Chronic Leukemia.
Authors: Krzysztof Czyzewski, Lidia Gil, Beata Kolodziej, Beata Rafinska, Krzysztof Lewandowski, Michal Gniot, Maria Lewandowska, Mieczyslaw Komarnicki, Mariusz Wysocki, Jan Styczynski
Journal: Blood (2009)
AB0131 Tissue Beta 2 glycoprotein I in Brain Ischemic Injury
Authors: C. Artusi, S. Fumagalli, M. Oggioni, M.O. Borghi, C. Grossi, P.L. Meroni, F. Tedesco, M.G. De Simoni
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Thrombosis of arteries in the central nervous system (CNS) represents the most frequent CNS manifestation and the most frequent arterial event in antiphospholipid syndrome (APS). Previous in vivo studies demonstrated that beta2 glycoprotein I (β2GPI) - the main antigenic target for anti-phospholipid antibodies (aPL) - does not localize in brain in resting conditions. However, β2GPI is detectable on the brain vascular endothelium in association with IgG and complement elements in immunized mice treated with lipopolysaccharide. These data suggest that after an appropriate second hit, β2GPI can play a critical role of in APS CNS damage, likely interacting with the complement system. To date, the pathophysiological functions of β2GPI in brain ischemic injury have not been investigated. <h3>Objectives</h3> To investigate the presence and the distribution of the activated form of β2GPI in the ischemic brain at different time points. <h3>Methods</h3> Eleven-week old male mice underwent focal cerebral ischemia induced by 609 transient middlecerebral artery occlusion (tMCAo), or sham-operation. Mice were sacrified at 909, 6h, 24h, 48h, 4d or 7d after tMCAo to obtain brains and plasma. The presence and time course of β2GPI deposition within the ischemic territory and its spatial distribution relative to blood vessels and brain cells were defined the by <i>post mortem</i> immunofluorescence and confocal microscopy. The activated β2GPI was detected by anti-beta2 glycoprotein I minibody (MBB2) – a human monoclonal IgG antibody targeting β2GPI domain I. <h3>Results</h3> β2GPI was present in the ischemic brain tissue starting from 909 after ischemic onset and was still detectable at 7d. β2GPI localized within blood vessels and in brain parenchyma at all times. Notably, at 24h β2GPI was mainly extravascular and colocalized with neurons (NeuN positive cells). Sham-operated mice showed only a faint signal for β2GPI. <h3>Conclusions</h3> β2GPI is present in its activated conformation in the brain vessels and tissue after experimental cerebral ischemia. It localizes on neurons in brain ischemic areas. These data suggest that an ischemic/inflammatory hit may up-regulate β2GPI tissue presence and may expose the N-terminal domain I of the molecule which is thougth to be the main target for pathogenic aPL. <h3>Disclosure of Interest</h3> None declared
JUN is a key transcriptional regulator of the unfolded protein response in acute myeloid leukemia
Authors: C Zhou, E Martinez, D Di Marcantonio, N Solanki-Patel, T Aghayev, S Peri, F Ferraro, T Skorski, C Scholl, S Fröhling, S Balachandran, D L Wiest, S M Sykes
Journal: Leukemia (2016)
Treatment of 11q23/MLL + AML with Gemtuzumab Ozogamicin: Results from the Randomized Phase III Children's Oncology Group Trial AAML0531
Authors: Jessica Pollard, Todd A. Alonzo, Robert B. Gerbing, Susana C. Raimondi, Betsy A. Hirsch, Lillian Sung, Richard Aplenc, Erin M. Guest, Irwin D. Bernstein, Michael R. Loken, Soheil Meshinchi, Alan S. Gamis
Journal: Blood (2015)
Biallelic Mutations in PARP4 Are Linked to a Variant Form of Congenital Dyserythropoietic Anemia
Authors: Paola Bianchi, Elisa Fermo, Jennifer C Eng, Jacob C Ulirsch, Cristina Vercellati, Paola Braidotti, Gordon Hildick-Smith, Timothy J Satchwell, Stephanie Pellegrin, Alberto Zanella, Wilma Barcellini, Barry H. Paw, Ashley Mark Toye, Vijay G Sankaran
Journal: Blood (2015)
Long-Term Cryopreservation of Autologous Stem Cell Grafts: Impact of Patient Age, Initial Collection, and Stem Cell Mobilization Regimen.
Authors: Hien Duong, Carol Dumont, Paul Elson, Edward A. Copelan, Brian J. Bolwell, Matt Kalaycio, Sharon Mannochio, Cheryl Sterle, Laura Zydiak, Priscilla Figueroa
Journal: Blood (2010)
Improved Immune Reconstitution Following Pre-Emptive CD8-Depleted Donor Lymphocyte Infusions (DLIs) after Haploidentical Stem Cell Transplantation (SCT) Using a Reduced-Intensity Conditioning (RIC) Regimen.
Authors: Paolo Corradini, Anna Raganato, Matteo Carrabba, Lorenza Gandola, Claudia Lombardo, Cristiana Carniti, Lucia Farina, Raffaella Milani, Anna Dodero
Journal: Blood (2005)
Essentials of Tropical Medicine.
Journal: JAMA (1921)

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Ploidy, as Detected by Fluorescence In Situ Hybridization, Defines Different Subgroups in Multiple Myeloma.
Authors: Herve Avet-Loiseau, Soraya Wuilleme, Nelly Robillard, Laurence Lode, Florence Magrangeas, Helen Beris, Jean-Luc Harousseau, John Proffitt, Stephane Minvielle
Journal: Blood (2004)
PTU-089 Portal Hypertensive Complications and Clinical Outcomes in Paediatric and Adolescent Patients Presenting with Portal Vein Thrombosis
Authors: S Direkze, A Bancil, A Dawan, M Samyn, N Heaton, H Velez-Mendes, M Davenport, P Kane, J Karani, D Joshi
Journal: Gut (2016)

<h3>Introduction</h3> Portal vein thrombosis (PVT) has multiple aetiologies which can lead to the development of portal hypertension and variceal bleeding. Data on the long term sequelae of PVT in paediatric and adolescent patients is limited. <h3>Methods</h3> Patients included had a diagnosis of PVT from Jan 2000- Dec 2014. Data collection included patient demographics, aetiologies, presentation and initial treatment of PVT. Data was also collected on further variceal bleeds, shunt surgery, liver transplantation and long term mortality. <h3>Results</h3> 123 patients (63 male) were identified. Median age at first presentation was 5years 9 months (range 2 days to 25 years). Overall survival was 95.9, 94.3 and 93.5% at 1, 10 and &gt;20 years from PVT diagnosis, respectively. Median age at follow up was 13years 3 months (range 6 months to 33 years 2 months). In the majority of cases (52%) no cause for PVT was identified. 78% of patients had extrahepatic PVT (EHPVT), with no extension in to the superior mesenteric vein. 19 patients (15.4%) were anticoagulated or had received a course of anticoagulation therapy. Initial presentation was usually due to oesophageal variceal bleeding (52.8%) of which 24 patients had further variceal bleeds (18.7%, oesophageal/duodenal/rectal). At follow up, 26% of patients (N = 32) were on a beta blocker. Of these, 10 patients (31.3%) had further variceal bleeds versus 14/77 patients, not on a beta blocker (18.2%, p = 0.07). Portal biliopathy was also present in 17.9% (N = 22) as was splenomegaly (82.9%). Ascites and hepatic encephalopathy were uncommon (&lt;7%). 18 patients had shunt surgery, majority of which were meso-caval Rex, of which 7/18 (38.8%) had further variceal bleeds. Two patients underwent transplantation, one of which was for variceal bleeding. Overall mortality in this group of patient with PVT was low (6.5% N = 8) though only 2 of these patients had a previous history of recurrent variceal bleeds. <h3>Conclusion</h3> Oesophageal variceal bleeding is a common index presentation of PVT in paediatric and adolescent patients. Approximately 20% of patients will go on to have further variceal bleeds despite medical intervention. Beta blocker use is associated with recurrent variceal bleeding which may suggest that it is ineffective in preventing further variceal bleeding in patients with established portal cavernomas. Overall, long term survival is good. <h3>Disclosure of Interest</h3> None Declared
Complex Karyotype (CK) Is Associated with a Shortened Progression-Free Survival (PFS) in Patients (pts) with Newly Diagnosed Mantle Cell Lymphoma (MCL).
Authors: Jonathon B. Cohen, Amy S. Ruppert, Nyla A. Heerema, Jeffrey A. Jones, Pierluigi Porcu, Robert A. Baiocchi, Beth Christian, John C. Byrd, Joseph M. Flynn, Sam Penza, Leslie A Andritsos, Steven M. Devine, Kristie A. Blum
Journal: Blood (2012)
OC-088 Associations Between Microbiota, Colonic Volume and Transit During A Low Fodmap Diet
Authors: J Jalanka, T Sloan, G Major, S Krishnasamy, S Pritchard, C Mulvenna, M Lomer, P Gowland, R Spiller
Journal: Gut (2016)

<h3>Introduction</h3> Ingestion of poorly digested carbohydrates can induce functional gastrointestinal symptoms, which may be relieved by a diet low in fermentable oligo-, di-, mono-saccharides and polyols (FODMAPs). How dietary changes alter intestinal microbiota and gut function remains unclear. This abstract reports changes in microbiota seen in a randomised controlled trial presented at DDW 2014<sup>1</sup> and associations with colonic volume (CV), transit and dietary oligofructose. <h3>Methods</h3> All subjects (healthy adults) followed a low FODMAP diet for 7 days, supervised by a registered dietitian. They supplemented their diet with oligofructose (OF) or maltodextrin (MD) 7 g twice daily. CV and transit were assessed by magnetic resonance imaging pre- and post-intervention, as were fasting breath hydrogen(H<sub>2</sub>) and methane(CH<sub>4</sub>). Stool samples were collected before and after intervention for exploratory analysis of microbiota by 16 S Miseq sequencing. <h3>Results</h3> 37 subjects (19 OF: 18 MD) completed the trial. All reported results were significant at corrected level p = 0.05 unless stated. At baseline the combined abundance of 3 bacterial genera - Bifidobacteria, Ruminococcus and Oscillospira - correlated positively with CV (r = 0.49) and transit time (r = 0.29, p &lt; 0.1) while the levels of Faecalibacteria and Lachnospiraceae correlated negatively with CV (r = 0.57) and transit (r = 0.35). CV increased in both groups but changes in microbial composition were different. Relative abundance of Actinobacteria, predominantly Bifidobacterium, decreased in the MD + low FODMAP diet group while the relative abundance of Coprococcus, Oscillospira and uncultured Clostridia increased. Abundance of the latter two correlated with fasting breath CH<sub>4</sub>. In contrast Bifidobacteria and breath H<sub>2</sub> both increased in the OF + low FODMAP diet group while abundance of Lachnospiraceae decreased. <h3>Conclusion</h3> Increase in Bifidobacteria and H<sub>2</sub> confirm previous reports after OF or the low FODMAP diet. The increase in certain bacterial taxa after MD may result from polysaccharides included in the low FODMAP diet. Larger volumes were associated with specific bacteria but the mechanism is unclear. Future research should include assessment of volumes, transit and microbiota to provide new insights into the complex relationship between microbiology, physiology and therapy. <h3>Reference</h3> 1 Major, <i>et al</i>. OC-114, DDF 2014. <h3>Disclosure of Interest</h3> None Declared
Excessive Rate of Late Infections and Treatment Related Mortality Associated with the Use of Alemtuzumab in Reduced Intensity Conditioning for Allogeneic Hematopoietic Stem Cell Transplantation: Results of a Prospective Trial.
Authors: Munira Shabbir, Luciano J Costa, Christine Schaub, Carrie Maxwell, Theo Fouts, Ashley Miller, Cindy Kramer, Karen Rogers, Louan Russ, Emily Kistner-Griffin, Robert K Stuart
Journal: Blood (2009)
THU0388 The Associations between Age with The Clinical and Radiological Activity in Spondyloarthritis
Authors: P.H. Li, C. Ho, H.Y. Chung, C.S. Lau
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Spondyloarthritis (SpA) is an umbrella term which includes several inflammatory arthritides sharing common clinical features, such as sacroiliitis, spondylitis, peripheral arthritis/enthesitis and other extra-articular manifestations. Although typically presenting at a young age, SpA is often a relentlessly progressive disease even in this new era of biologics. In the last few years, much research has been generating on discovering new and more sensitive modalities in the use of magnetic resonance imaging (MRI) for assessing SpA. The advent of MRI has enabled clinicians to detect objective features of active inflammation, thereby allowing earlier diagnosis and more quantitative assessment of disease severity. Despite these rapid advancements, there have been few reports on examining the association between age and disease activity in SpA, especially with the use of these various new modalities of imaging. In this cross-sectional analysis, we examine the associations between age and the clinical and radiological disease activities in axial SpA using radiographs as well as different modalities of MRI. <h3>Methods</h3> One hundred and twenty one patients fulfilling the Assessment of SpondyloArthritis International Society Classification Criteria for axial SpA were included in analyses. Patient demographics, disease activity and radiographic scores, as well as magnetic resonance imaging (MRI) with diffusion weighted imaging derived apparent diffusion coefficient values ((DWI(ADC)), were compared between patients older than and younger/equal to 40 years of age at a cross-sectional level. Variables with significant differences in univariate analyses were used as dependent variables in multivariate linear regression models adjusted for potential confounding/contributing factors. <h3>Results</h3> Multivariate analysis showed that increasing age was significantly associated with higher Bath Ankylosing Spondylitis Functional Index (B=0.04, p&lt;0.01) and Bath Ankylosing Spondylitis Metrology Index scores (B=0.03, p&lt;0.01); as well as higher modified Stoke Ankylosing Spondylitis Spine Score (B=0.57, p&lt;0.01). On MRI, increasing age was associated with a lower SpondyloArthritis Research Consortium of Canada MRI index (B=(-0.2), p=0.01) and DWI(ADC) (B=(-0.01), p&lt;0.01) of the SI joints, but higher DWI(ADC) values of the axial spine (B=0.01, p=0.01). <h3>Conclusions</h3> Increasing age in SpA was associated with greater functional impairment and structural damage, more inflammation of the axial spine, but less inflammation of the SI joints. Our findings are consistent with the traditional belief of SpA being an “ascending disease” and highlights the importance of different modalities of MRI in the diagnosis and disease monitoring of SpA. <h3>Disclosure of Interest</h3> None declared
Novel Nanomolar Potency Mitochondrial Complex I Inhibitor Iacs-1131 Selectively Kills Oxphos-Dependent AML Cells
Authors: Polina Matre, Marina Protopopova, Ningping Feng, Jason Gay, Jennifer Greer, Juliana Velez, Hong Mu, Alessia Lodi, Surendar Tadi, Shannon Sweeney, Michael Andreeff, Stefano Tiziani, M. Emilia DiFrancesco, Joseph Marszalek, Marina Konopleva
Journal: Blood (2014)
The Identification of ERG Related Pathways Pinpoints to Mechanisms Implicated in the Inferior Outcome of Acute T-Lymphoblastic Leukemia (T-ALL) with High ERG Expression.
Authors: Claudia D. Baldus, Michael D. Radmacher, Clara D. Bloomfield, Peter Martus, Stefan Schwartz, Nicola Goekbuget, Dieter Hoelzer, Eckhard Thiel, Wolf K. Hofmann
Journal: Blood (2006)
Unexpected role for p19INK4d in posttranscriptional regulation of GATA1 and modulation of human terminal erythropoiesis
Authors: Xu Han, Jieying Zhang, Yuanliang Peng, Minyuan Peng, Xiao Chen, Huiyong Chen, Jianhui Song, Xiao Hu, Mao Ye, Jianglin Li, Vijay G. Sankaran, Christopher D. Hillyer, Narla Mohandas, Xiuli An, Jing Liu
Journal: Blood (2016)