tbuckley/cabot-search huggingface dataset:
AB0336 COMPARATIVE ANALYSIS OF ESTIMATED BONE MASS AND BONE MINERAL DENSITY IN PATIENTS WITH RHEUMATOID ARTHRITIS FROM THE CHIKARA STUDY
Authors: Koji Mandai, Yutaro Yamada, Tatsuya Koike, Tadashi Okano, Noriaki Hidaka, Masahiro Tada
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Patients with rheumatoid arthritis (RA) have lower muscle mass and a higher rate of sarcopenia than healthy individuals. The relationship between rheumatoid arthritis and bone loss is well known. Dual energy X-ray absorptiometry (DEXA) and bioelectrical impedance analysis (BIA) are used to diagnose sarcopenia, but the correlation between bone mineral density and estimated bone mass is unknown. <h3>Objectives</h3> To investigate the correlation between bone mineral density and bone mass in RA patients. <h3>Methods</h3> Data from a prospective observational study (CHIKARA study) were analyzed. Bone mineral density (BMD) was measured by DEXA, and bone mass was measured by BIA on the same day, and the correlations between BMD at each measurement site and bone mass were evaluated. <h3>Results</h3> A total of 90 RA patients (women, 83%; mean age, 67.2±12.1 years) were included. The median disease duration was 8.5 (interquartile range (IQR): 4.9, 17) years, the mean DAS28-ESR was 2.92±1.2, and the median MTX dose was 10 (IQR: 8, 12) mg/week. Nineteen patients (21%) used a biologic agent. There was a significant correlation between total hip BMD and bone mass (r=0.64, p&lt;0.001). The details are shown in Figure 1. Bone mass was significantly correlated with femoral neck BMD (r=0.62, p&lt;0.001), whole body BMD (r=0.60, p&lt;0.001), and lumbar BMD (r=0.49, p&lt;0.001). Bone mass was significantly lower by DEXA (1.9±0.5 kg) than by BIA (2.0±0.4 kg) (P&lt;0.001). <h3>Conclusion</h3> There was a significant correlation between bone mass by BIA and BMD by DEXA. Bone mass by BIA and total hip BMD had the strongest correlation. Bone mass is likely to be overestimated by BIA. Thus, we need to evaluate it with appropriate caution. <h3>Disclosure of Interests</h3> Koji Mandai: None declared, Yutaro Yamada Speakers bureau: Abbvie, Chugai, Mitsubishi Tanabe, Tatsuya Koike Speakers bureau: AbbVie, Astellas Pharma Inc., Bristol-Myers Squibb, Chugai Pharmaceutical, Eisai, Janssen, Lilly, Mitsubishi Tanabe Pharma Corporation, MSD, Ono Pharmaceutical, Pfizer, Roche, Takeda Pharmaceutical, Teijin Pharma, and UCB, Tadashi Okano Speakers bureau: AbbVie, Noriaki Hidaka: None declared, Masahiro Tada Speakers bureau: Abbvie, Astellas Pharma, Bristol-Myers Squibb, Chugai Pharmaceutical, Eisai, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Ono Pharmaceutical, Pfizer Japan, Takeda Pharmaceutical
PS1507 INCREASED VOC FREQUENCY ASSOCIATED WITH REDUCED HRQOL AMONG PATIENTS WITH SCD
Authors: M. Besser, M. Bailey, S. Brown, M. Thompson
Journal: HemaSphere (2019)

Background: Sickle cell disease (SCD) is a global health concern. Each year as many as 200,000 infants are born with this disease worldwide. Despite improvements in outcomes over the last few decades, patient life expectancy remains 10 to 30 years shorter than that of the healthy population. A major cause of morbidity among patients with SCD is the occurrence of intense, recurrent, acute, severely painful episodes, known as vaso‐occlusive crisis (VOCs). As patients age and SCD progresses, an increased frequency of VOCs and other disease‐related complications is correlated with an increased risk of morbidity and mortality. Aims: The aim of the current analysis was to determine whether VOC frequency is associated with a decrease in the health‐related quality of life (HRQoL) and associated utility of patients with SCD. Methods: LEGACY was a prospective, multicenter, observational study that documented the outcomes of 181 adult patients with SCD living in the United States. Patients were assessed at baseline and every six months for 3 years, on several health outcomes, including the number of VOCs experienced and patient health as assessed by the Short Form‐36 (SF‐36) Health Survey. For the cohort of patients aged 18 years and older, the total number of VOCs experienced over the previous 12 months was determined for each administration of the SF‐36. Patients were categorized into three groups: ‐ 0 VOCs in the previous 12 months ‐ 1 to 2 VOCs in the previous 12 months ‐ 3 or more (3+) VOCs in the previous 12 months The SF‐36 scores for individual questionnaires were then mapped to the EuroQoL five‐dimension (EQ‐5D) questionnaire, which is a standardized instrument that can be used to derive a single utility value. The mapping was done using the random effects GLS mapping algorithm published by Rowan and colleagues (2009). Mean EQ‐5D scores were compared between groups using the Student's T‐test. Differences between the groups on the SF‐36 component scores and SF‐36 domains were also assessed. Results: Patients with 3+ VOC events in the previous 12 months had mapped mean EQ‐5D scores that tended to be lower (15%) than those of patients who had no VOC events (see Figure 1 ). Patients who had 3+ VOC events also showed decreases in both SF‐36 Physical Component Scores and Mental Component Scores compared to patients with no VOC events. Summary/Conclusion: To our knowledge, this is the first analysis to examine the relationship between VOC frequency and HRQoL in patients with SCD. The results of this analysis indicate that patients who have a higher number of VOC events experience a worse HRQoL compared to patients with no events. Additional research is required to understand the drivers of this difference in HRQoL. image
PB2056 ORBITAL LYMPHOMA: CLINICAL AND EPIDEMIOLOGICAL PATTERNS WITHIN THE UNITED STATES
Authors: M. khalafAllah, M. gouda
Journal: HemaSphere (2019)

Background: Lymphomas are relatively common tumors that may affect the orbit through tissue infiltration. However, data available about tumors possibly originating in orbit (also called orbital lymphoma) are scarce. Aims: In this study, we aimed to spotlight on this type of malignancy using the using data from American National Cancer Institute, Surveillance, Epidemiology, and End Results (SEER) Program. Methods We used SEER∗Stat version 8.3.5, Microsoft Excel, as well as SPSS IBM SPSS Statistics for Windows, Version 25.0 for data analysis. In SEER∗Stat, we used the (Incidence ‐ SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2017 Sub (1973‐2015 varying)) database to obtain data. We included cases diagnosed with lymphoma between 2006 and 2015 at site related to orbital cavity C69.0‐C69.9. Results: A total of 2340 cases were identified with a median age of 65.2. Females and patients of white race represented the majority of the analyzed cohort (n = 1249, 53.4%; n = 1846, 78.9%, respectively). Age‐adjusted Incidence rate was 3 per 10,000. 5‐years observed survival was 82% (95% CI: 79.8% ‐ 84%) while the 5‐years relative survival was 94.3% (95% CI: 91.3% ‐ 96.2%). The most common pathology of studied cases was extranodal marginal zone lymphoma of mucosal‐associated lymphoid tissue (MALT) (n = 1310, 56%) [Table 1]. Median survival was not reached at the study cutoff (Figure 1). Summary/Conclusion: Orbital lymphoma has an incidence rate of 3 per 10,000. These tumors are more likely to occur in old age, females, and white race. Survival data are relatively good with the 5‐years relative survival being 94.3%. image
Abstract 12892: Safety and Efficacy of LHW090 in Patients With Resistant Hypertension: Results of a Randomized, Double Blind, Parallel Group, Placebo-Controlled Study
Authors: B. Alexander Yi, Neda Huseinovic, Douglas Hom, Anisha E Mendonza, Kenneth M Kulmatycki, Muneto Mogi, Vidya V Kunjathoor, Yaritza Ruiz, Hansjoerg Moest, Arvind G Kinhikar, Blanca C Luna, Tina Gaskin, Bert-Jan van den Born, Yvonne Doerffel, Jan Menne, Hermann Haller, William B Smith, Craig T Basson
Journal: Circulation (2018)

Background: Patients with resistant hypertension have a higher risk of cardiovascular morbidity and mortality. The current treatment options for patients with resistant hypertension are limited by ...
AB1082 IATROGENIC INFECTIOUS SPONDYLODISCITIS: 6 CASES
Authors: Rim Dhahri, Maroua Slouma, Leila Metoui, Souha Hannachi, Imen Gharsallah, Bassem Louzir
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> The increasing proportion of iatrogenic spondylodiscitis (SD) is a prominent feature of infectious DS in recent decades <h3>Objectives</h3> To describe iatrogenic clinical, aetiological and evolutive SD features. <h3>Methods</h3> We report 6 cases of iatrogenic DS collected in our department in 2001-2012 period. <h3>Results</h3> All patients were male. The average age was of 49 (35 to 68 years). Four cases of SD were caused by direct inoculation (surgical in 3 cases, chemonucleolysis in 1 case) and 2 cases of hematogenous SD from an initial outbreak. The seat of infection is lumbar in all cases. Spinal pain is almost constant. The admission examination noted apyrexia in 5 cases. A biological inflammatory syndrome was present in 5 cases and hyperleucocytosis in 2 cases. The bacteriological investigation was able to isolate a methicillin-sensitive Staphylococcus aureus (SAMS) and a proteus mirabilis in one patient and SAMS in another. The radiological assessment made it possible to objectify a para vertebral abscess in 5 cases and an epilepitis in 1 case. The evolution was favorable in all cases under antibiotherapy of average duration of 4.5 months and immobilization of the spine by corset. <h3>Conclusion</h3> The detection of an anaerobic germ causing infectious spondylodiscitis should search for an iatrogenic portal chronologically and anatomically close to the vertebral disc infection. <h3>Disclosure of Interests</h3> None declared
SP0024 STANDARDISATION OF NORMAL VERSUS ABNORMAL AND PATHOLOGICAL CAPILLAROSCOPIC IMAGES
Authors: Vanessa Smith
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Abstract:</h3> Medical doctors frequently get patients with Raynaud9s phenomenon (RP), a frequent symptom in the general population, referred. The importance of distinguishing normal capillaroscopic findings from (pathognomonic) abnormal (pathological) findings (scleroderma pattern), lies in the fact that this distinction allows the differentiation between a primary RP (not connected to any connective tissue disease [CTD]) from a secondary RP due to systemic sclerosis (SSc) and diseases of the scleroderma spectrum. What is normal in primary RP? A normal capillaroscopic pattern, by qualitative assessment, is characterized by a homogeneous distribution of hairpin shaped capillaries as a "comb-like structure", with a density of &gt;7capillaries per mm, with a normal dimension and absence of large hemorrhages Yet, there exists a wide intra- and inter- individual variety in a normal population which will be discussed in this session. What is pathognomonic abnormal in patients with RP due to SSc? Patients with the RP who have an underlying clinically recognizable (= with skin involvement) SSc show a very characteristic combination of capillary abnormalities in the nailfold, which can easily be assessed through qualitative assessment (= pattern recognition). Maricq et al. described last century, with the widefield technique (magnification X12–14) the scleroderma pattern. This pathognomonic combination contains the following: a striking widening of all three segments of the capillary loop (arterial, venous and intermediate), loss of capillaries and disorganization of the nailfold capillary bed. Many branched "bushy" capillaries may also be observed. In 2000, Cutolo et al. qualitatively assessed the nailfolds of an SSc cohort with patients fulfilling the American College of Rheumatology (ACR) criteria for SSc with the nailfold videocapillaroscopic (NVC) technique (magnification X200). According to the different proportions of the hallmark parameters of the scleroderma pattern (giants, capillary loss, hemorrhages and abnormal shapes: (neo)angiogenesis, Cutolo et al. defined three patterns "early", "active" and "late". The central role of capillaroscopy in distinction between a primary and secondary RP due to SSc is reflected by the fact that capillaroscopy is one of the new ACR/EULAR criteria for classifying a patient as having SSc. In this lecture the standard "FAST TRACK" recognition system of the EULAR Study Group on Microcirculation in Rheumatic Diseases to discern scleroderma patterns from non-scleroderma patterns will be taught to the attendees. Suggested further reading: Smith V, et al. Rheumatology (Oxford). 2016;55(5):883-90. Cutolo M, et al. Rheumatology (Oxford). 2018;57(4):757-759. Cutolo M, et al. Autoimmun Rev. 2018;17(4):344-352. <h3>Disclosure of Interests:</h3> None declared
“Good Fences Make Good Neighbors”: How does the Human Gut Microchip Unravel Mechanism of Intestinal Inflammation?
Authors: Woojung Shin, Landon A. Hackley, Hyun Jung Kim
Journal: Gut Microbes (2019)

A microengineered human gut-on-a-chip has demonstrated intestinal physiology, three-dimensional (3D) epithelial morphogenesis, and longitudinal host-microbiome interactions in vitro. The modular accessibility and modularity of the microphysiological gut-on-a-chip can lead to the identification of the seminal trigger in intestinal inflammation. By coupling microbial and immune cells in a spatiotemporal manner, we discovered that the maintenance of healthy epithelial barrier function is necessary and sufficient to demonstrate the homeostatic tolerance of the gut. Here, we highlight the breakthrough of our new disease model and discuss the future impact of investigating the etiology and therapeutic targets in the multifactorial inflammatory bowel disease.
PS1115 THE RELATIONSHIP BETWEEN THE PRETREATMENT PNH CLONE SIZE AND CLINICAL COURSE IN PATIENTS WITH BONE MARROW FAILURE SYNDROMES: INTERIM ANALYSIS OF JAPANESE MULTICENTER PROSPECTIVE STUDY
Authors: Y. Ueda, K. Hosokawa, K. Ishiyama, H. Takamori, Y. Yonemura, N. Obara, H. Noji, H. Takahashi, Y. Shirasugi, T. Matsuda, K. Ando, T. Shichishima, T. Ikezoe, S. Chiba, H. Ninomiya, T. Kawaguchi, J.-I. Nishimura, Y. Kanakura, S. Nakao
Journal: HemaSphere (2019)

Background: Gaucher Disease (GD) is a congenital disorder secondary to GBA1 gene mutations that cause a defective function of the catabolic enzyme β-glucocerebrosidase (GBA) and a progressive accumulation of its substrate- glucocerebroside (GC) in various organs, in particular in mononuclear phagocite system. Bone infiltration, hepatosplenomegaly and cytopenia represent the most common features of the disease. Since clinical manifestation can be subdolous, in some cases, phenotype can overlap with Autoimmune Lymphoproliferative Sindrome (ALPS). Nonetheless, GD patients were also shown to have hyperinflammatory features- secondary to machrophages engorgement and actviation- and an aspecific impaiment involving B, T and NK cells. In addition, some patients also showed more specific ALPS-like immune-dyregulation features suggesting an underlying apoptosis defect. Aims: To evaluate specific ALPS-like immunological and serological pattern (Double Negative T-cells, TCR alfa/beta B220, B-memory cells, T-regs/HLA-DR ratio, IL-10, IL-18,) in a cohort patients with GD and to test apoptosis pathway function in those showing ALPS-like features. Methods: All patients were diagnosed with GD by enzyme assay on lymphocytes culture or fibroblasts and by characterization of pathogenetic variants of GBA1. Lymphocytes subsets and ALPS-related serum biomarkers (IL-10, IL-18, sFAS, Vit.B12) were analyzed in 41 GD patients followed-up at Istituto Giannina Gaslini. Furthermore, in those patients who showed an ALPS-like phenotype, apoptosis function was analyzed after FAS–induced stimulation of EBV-immortalized B-cells and Western blot analysis of CASP10, CASP8, and PARP proteins. Results: 10/41 (24%) patients showed an ALPS-like immunological pattern which was more frequent in naïve patients (p = 0.003) and in patients with an earlier onset of the diesease (p = 0.010). EBV-immortalized B-cells of 7 out of these 10 patients were further studied and all of them showed a defective FAS-induced apoptosis and caspases activation. Fig 1 shows apoptosis evalutation of the selected population.Summary/Conclusion: To the best of our knowledge, this study shows for the first time that untreated subjects with early-onset GD present an immune-dysregulation pattern secondary to a defect of apoptosis as seen in ALPS patients. These results suggest that diagnostic work-up of both diseases must take into consideration their clinical and biochemical overlap. Further studies are needed to evaluate the potential role of cell-membrane lipids impairment on FAS receptor structure as a cause of such defect.
PB1742 AGE, KARYOTYPES AND WHETHER RECEIVING INDUCTION CHEMOTHERAPY ARE INDEPENDENT PROGNOSTIC FACTORS IN ELDERLY PATIENTS WITH AML
Authors: W. Wang, Z. Jiang, J. Ma, H. Sun, L. Sun, X. Meng
Journal: HemaSphere (2019)

Background: Acute myeloid leukemia (AML) is an aggressive disease with an unfavorable prognosis. AML is more common in elderly patients, and the treatments for these patients are limited, particularly for those with poor performance status, comorbidities, and unfavorable cytogenetic abnormalities. There are no universally accepted, standard approaches to treat AML in elderly patients. Aims: To analyze the clinical characteristics and prognostic factors of elderly patients with acute myeloid leukemia(AML). Methods: A total of 232 AML patients were enrolled in this retrospective study. The clinical characteristics were collected and analyzed retrospectively. Factors affecting complete remission (CR) were analyzed by using chi square test. Univariate and multivariate analyses of prognostic factors were performed by using Kaplan‐Meier and Cox hazard regression model respectively. Results: 195 of 232 patients received induction chemotherapy, among whom 8 patiens died in early phase. 162 patients were able to be evaluated the efficacy, the CR rate after the first circle was 37.0 % (60/162). Overall CR rate was 54.9% (60/162). 37 patiens received palliative treatment, among whom 6 patients died in early phase and none achieved CR. Age between 60‐69 (χ 2 =4.102, P = 0.043), ECOG score≤2 (χ 2 =9.917, P = 0.002), NPM1 + FLT3‐ITD ‐ (χ 2 =6.423, P = 0.038), favorable karyotypes (χ 2 =6.033, P = 0.049) were related to a higher CR rate. The median overall survival (OS) was 205 day. Univariate analysis results demonstrated that age≥70 years (χ 2 =8.7, P = 0.003), white cell count≥100 × 10 9 /L (χ 2 =4.249, P = 0.039), unfavorable karyotypes (χ 2 =4.807, P = 0.028), palliative treatment (χ 2 = 191.221, P = 0.000) were influence factors on OS. In multivariable analysis, age between 60‐69 ( HR = 0.464,95 % CI 0.245∼0.877, P = 0.018), unfavorable karyotypes ( HR = 3.618,95 % CI 1.491∼6.728, P = 0.003) and receiving induction chemotherapy ( HR = 0.076,95 % CI 0.030∼0.194, P = 0.000) were independent prognostic factors for OS in elderly patients with AML. Summary/Conclusion: The prognosis of elderly patients with AML is the result of the combined effect of many factors. Age, karyotypes and whether receiving induction chemotherapy are independent prognostic factors in elderly patients with AML.
Copy number signatures and mutational processes in ovarian carcinoma
Authors: Geoff Macintyre, Teodora E. Goranova, Dilrini De Silva, Darren Ennis, Anna M. Piskorz, Matthew Eldridge, Daoud Sie, Liz-Anne Lewsley, Aishah Hanif, Cheryl Wilson, Suzanne Dowson, Rosalind M. Glasspool, Michelle Lockley, Elly Brockbank, Ana Montes, Axel Walther, Sudha Sundar, Richard Edmondson, Geoff D. Hall, Andrew Clamp, Charlie Gourley, Marcia Hall, Christina Fotopoulou, Hani Gabra, James Paul, Anna Supernat, David Millan, Aoisha Hoyle, Gareth Bryson, Craig Nourse, Laura Mincarelli, Luis Navarro Sanchez, Bauke Ylstra, Mercedes Jimenez-Linan, Luiza Moore, Oliver Hofmann, Florian Markowetz, Iain A. McNeish, James D. Brenton
Journal: Nature Genetics (2018)
Corrections to “Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium”
Authors: P Quaglino, M Maule, H M Prince, P Porcu, S Horwitz, M Duvic, R Talpur, M Vermeer, M Bagot, J Guitart, E Papadavid, J A Sanches, E Hodak, M Sugaya, E Berti, P Ortiz-Romero, N Pimpinelli, O Servitje, A Pileri, P L Zinzani, T Estrach, R Knobler, R Stadler, M T Fierro, S Alberti Violetti, I Amitay-Laish, C Antoniou, C Astrua, S Chaganti, F Child, A Combalia, S Fabbro, P Fava, V Grandi, C Jonak, E Martinez-Escala, M Kheterpal, E J Kim, C McCormack, T Miyagaki, D Miyashiro, S Morris, C Muniesa, V Nikolaou, G Ognibene, F Onida, S Osella-Abate, S Porkert, C Postigo-Llorente, C Ram-Wolff, S Ribero, K Rogers, M Sanlorenzo, R Stranzenbach, N Spaccarelli, A Stevens, D Zugna, A H Rook, L J Geskin, R Willemze, S Whittaker, R Hoppe, J Scarisbrick, Y Kim
Journal: Annals of Oncology (2018)

Ann Oncol 2017; 28: 2517–2525 (doi: 10.1093/annonc/mdx352) In the original article, the affiliation number 16 for authors E. Berti and S. Alberti Violetti was incorrect. It originally read: ‘Dermatologic Clinic, University of Milano, Milano, Italy’ and has now been changed to ‘Department of Dermatology, Fondazione Cà Granda, IRCCS, OMP, Milano, Italy’. Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International ConsortiumAnnals of OncologyVol. 28Issue 10PreviewAdvanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival. Full-Text PDF Open Archive
Hospitals are using “escalation” beds routinely, not just in winter, BMA finds
Authors: Gareth Iacobucci
Journal: BMJ (2019)

Many NHS hospitals in England are routinely putting patients in beds that should be reserved for emergencies or busy periods, shows a new analysis by the BMA.1

Data obtained by the BMA from hospital trusts through freedom of information requests show that many are regularly using so-called “escalation beds” for routine care in a response to ever growing patient demand. Escalation beds are used in addition to permanent bed stock and are brought in by hospitals to provide capacity for limited periods in temporary or repurposed wards or as additions to existing wards. The vast majority …