Search

Objective: Calcium pyrophosphate deposition (CPPD) disease is prevalent and has diverse presentations, but there are no validated classification criteria for this symptomatic arthritis. The American College of Rheumatology (ACR) and EULAR have developed the first‐ever validated classification criteria for symptomatic CPPD disease. Methods: Supported by the ACR and EULAR, a multinational group of investigators followed established methodology to develop these disease classification criteria. The group generated lists of candidate items and refined their definitions, collected de‐identified patient profiles, evaluated strengths of associations between candidate items and CPPD disease, developed a classification criteria framework, and used multi‐criterion decision analysis to define criteria weights and a classification threshold score. The criteria were validated in an independent cohort. Results: Among patients with joint pain, swelling, or tenderness (entry criterion) whose symptoms are not fully explained by an alternative disease (exclusion criterion), the presence of crowned dens syndrome or calcium pyrophosphate crystals in synovial fluid are sufficient to classify a patient as having CPPD disease. In the absence of these findings, a score >56 points using weighted criteria, comprising clinical features, associated metabolic disorders, and results of laboratory and imaging investigations, can be used to classify as CPPD disease. These criteria had a sensitivity of 92.2% and specificity of 87.9% in the derivation cohort (190 CPPD cases, 148 mimickers), whereas sensitivity was 99.2% and specificity was 92.5% in the validation cohort (251 CPPD cases, 162 mimickers). Conclusion: The 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field. [ABSTRACT FROM AUTHOR]

Background: In asylum seekers and refugees, the frequency of mental disorders, such as depression, anxiety and post-traumatic stress disorder, is higher than the general population, but there is a lack of data on risk factors for the development of mental disorders in this population. Aim: This study investigated the risk factors for mental disorder development in a large group of asylum seekers and refugees resettled in high- and middle-income settings. Methods: Participant-level data from two randomized prevention studies involving asylum seekers and refugees resettled in Western European countries and in Turkey were pooled. The two studies randomized participants with psychological distress, but without a diagnosis of mental disorder, to the Self-Help Plus psychological intervention or enhanced care as usual. At baseline, exposure to potentially traumatic events was measured using the Harvard Trauma Questionnaire-part I, while psychological distress and depressive symptoms were assessed with the General Health Questionnaire and the Patient Health Questionnaire. After 3 and 6 months of follow-up, the proportion of participants who developed a mental disorder was calculated using the Mini International Neuropsychiatric Interview. Results: A total of 1,101 participants were included in the analysis. At 3- and 6-month follow-up the observed frequency of mental disorders was 13.51% (115/851) and 24.30% (207/852), respectively, while the frequency estimates after missing data imputation were 13.95% and 23.78%, respectively. After controlling for confounders, logistic regression analysis showed that participants with a lower education level (p =.034), a shorter duration of journey (p =.057) and arriving from countries with war-related contexts (p =.017), were more at risk of developing mental disorders. Psychological distress (p =.004), depression (p =.001) and exposure to potentially traumatic events (p =.020) were predictors of mental disorder development. Conclusions: This study identified several risk factors for the development of mental disorders in asylum seekers and refugees, some of which may be the target of risk reduction policies. The identification of asylum seekers and refugees at increased risk of mental disorders should guide the implementation of focused preventative psychological interventions. [ABSTRACT FROM AUTHOR]

The black soldier fly (BSF), Hermetia illucens L. (Diptera: Stratiomyidae), has a great bioconversion potential and ability to develop on diverse substrates. Although the use of catering waste and food by-products containing meat and fish would reduce the footprint of the insect sector, to date, in Europe, their use is still facing legal obstacles for insects as food and feed. Since a major request from the EU insect sector is to diversify the spectrum of allowed substrates, and considering that variations in diet composition could influence insect immune responses, we evaluated the impact of different catering wastes on BSF prepupae immunity. Insects were reared on five diets: one based on feed for laying hens and four based on catering waste containing (i) fruits and vegetables; (ii) fruits, vegetables and bread; (iii) fruit, vegetables, bread and dairy products; (iv) fruits, vegetables, bread, meat and fish. The gene expression of two antimicrobial peptides (AMPs), one defensin and one cecropin, was assessed. Moreover, the hemolymph inhibitory activity against Escherichia coli DH5α and Micrococcus yunnanensis HI55 was evaluated using diffusion assays in solid media. The up-regulation of both AMPs' encoding genes was observed in insects fed a bread-added and dairy product-added diet. All hemolymph samples showed inhibitory activity against both bacteria, affecting the colony size and number. The obtained results show how catering waste positively influences the H. illucens immune system. The possibility of modulating AMP expression levels through the diet opens up new perspectives in the management of insect health in mass rearings. [ABSTRACT FROM AUTHOR]

The Europe 2020 strategy has been implemented since 2010 to recover from the Great Recession which has affected European Union countries. One pillar of this strategy is that economic growth should be sustainable and inclusive. Focusing on the concept of inclusiveness, a set of economic indicators observed over the 1995–2019 period in a subgroup of member countries is selected. To track the evolution of inclusive growth in each country, we propose an approach towards three-way data analysis. Using such an approach, a time trajectory showing the dynamics of labour market inclusion and economic well-being in each country can be plotted. This graphical analysis allows us to examine the extent to which economic growth has been inclusive in each country and compare the growth paths of different countries. The results show that economic growth has been heterogeneous across countries and has been coupled with a more inclusive labour market only in some countries. [ABSTRACT FROM AUTHOR]

The oldest-old population is increasing in Europe, and greater focus is placed on promoting mental well-being (MWB) in this population. The European Welfare Models and Mental Wellbeing in Final Years of Life project aims to develop a better understanding of how best to promote positive MWB in the oldest-old population. Using a resources approach, the present study aimed to provide empirical evidence about the structure of MWB in the 80 + year age group and to compare this with the structure of MWB in the old (65–79 years) and adult (18–64 years) population. Twenty-eight items reflecting a focus on positive aspects of MWB were selected from the European Social Survey data (24 countries). After application of an exploratory approach using Exploratory Structural Equation Modelling, five- and six-factor model solutions were found to be statistically appropriate, and the results are consistent with the most widely studied dimensions of MWB. Despite specific differences in the factor models and item loadings, evaluation of formal invariance showed that dimensions built in the same way are comparable across age groups. Although explorative and not conclusive, the results of this study contribute insights into the multidimensional structure of MWB in the oldest-old population and provide a starting point for further research on promoting MWB in the later stages of life. [ABSTRACT FROM AUTHOR]

• The values of parameters of a well-calibrated model are useful in detecting micro behavior. • We illustrate the evolution of banks strategy in response to different economic conditions. • It explores the banks reactions to financial shocks. • The result is an early warning indicator which anticipates phases of instability. In this paper, we show that the values of parameters of a well-calibrated model are useful in detecting micro behavior. We use a calibration procedure suitable for validating agent-based models to show how the evolution of model parameters, obtained via a rolling window estimation, illustrates the evolution of agents' strategies in response to different economic conditions. In this regard, we calibrate the well-known financial model of Brock and Hommes using three banking indices (i.e., the S&P SmallCap 600 Financials Index, the STOXX Europe 600 Banks, and the STOXX Asia/Pacific 600 Banks) running from 1994 to 2016. The choice of a spatially and temporally diversified dataset allows us to analyze differences and similarities in the behavior of banks belonging to the different macro areas, as well as to capture agents' reaction to the several economic phases characterizing the time series investigated. [ABSTRACT FROM AUTHOR]

Aims.: There is a need of more quantitative standardised data to compare local Mental Health Systems (MHSs) across international jurisdictions. Problems related to terminological variability and commensurability in the evaluation of services hamper like-with-like comparisons and hinder the development of work in this area. This study was aimed to provide standard assessment and comparison of MHS in selected local areas in Europe, contributing to a better understanding of MHS and related allocation of resources at local level and to lessen the scarcity in standard service comparison in Europe. This study is part of the Seventh Framework programme REFINEMENT (Research on Financing Systems' Effect on the Quality of Mental Health Care in Europe) project. Methods.: A total of eight study areas from European countries with different systems of care (Austria, England, Finland, France, Italy, Norway, Romania, Spain) were analysed using a standard open-access classification system (Description and Evaluation of Services for Long Term Care in Europe, DESDE-LTC). All publicly funded services universally accessible to adults (≥18 years) with a psychiatric disorder were coded. Care availability, diversity and capacity were compared across these eight local MHS. Results.: The comparison of MHS revealed more community-oriented delivery systems in the areas of England (Hampshire) and Southern European countries (Verona – Italy and Girona – Spain). Community-oriented systems with a higher proportion of hospital care were identified in Austria (Industrieviertel) and Scandinavian countries (Sør-Trøndelag in Norway and Helsinki-Uusimaa in Finland), while Loiret (France) was considered as a predominantly hospital-based system. The MHS in Suceava (Romania) was still in transition to community care. Conclusions.: There is a significant variation in care availability and capacity across MHS of local areas in Europe. This information is relevant for understanding the process of implementation of community-oriented mental health care in local areas. Standard comparison of care provision in local areas is important for context analysis and policy planning. [ABSTRACT FROM AUTHOR]

Objective: Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. Methods: Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE‐mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. Results: Data were collected on 389 patients with early SLE and 227 patients with SLE‐mimicking conditions. Unexplained fever was more common in early SLE than in SLE‐mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti–double‐stranded DNA, anti–β2‐glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE‐mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). Conclusion: In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE‐mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria. [ABSTRACT FROM AUTHOR]

Background A suitable definition of primary care to capture the variety of prevailing international organisation and service-delivery models is lacking. Aim Evaluation of strength of primary care in Europe. Design and setting International comparative cross-sectional study performed in 2009-2010, involving 27 EU member states, plus Iceland, Norway, Switzerland, and Turkey. Method Outcome measures covered three dimensions of primary care structure: primary care governance, economic conditions of primary care, and primary care workforce development; and four dimensions of primary care service-delivery process: accessibility, comprehensiveness, continuity, and coordination of primary care. The primary care dimensions were operationalised by a total of 77 indicators for which data were collected in 31 countries. Data sources included national and international literature, governmental publications, statistical databases, and experts' consultations. Results Countries with relatively strong primary care are Belgium, Denmark, Estonia, Finland, Lithuania, the Netherlands, Portugal, Slovenia, Spain, and the UK. Countries either have many primary care policies and regulations in place, combined with good financial coverage and resources, and adequate primary care workforce conditions, or have consistently only few of these primary care structures in place. There is no correlation between the access, continuity, coordination, and comprehensiveness of primary care of countries. Conclusion Variation is shown in the strength of primary care across Europe, indicating a discrepancy in the responsibility given to primary care in national and international policy initiatives and the needed investments in primary care to solve, for example, future shortages of workforce. Countries are consistent in their primary care focus on all important structure dimensions. Countries need to improve their primary care information infrastructure to facilitate primary care performance management [ABSTRACT FROM AUTHOR]

Background: Scientific research has provided evidence on benefits of well developed primary care systems. The relevance of some of this research for the European situation is limited. There is currently a lack of up to date comprehensive and comparable information on variation in development of primary care, and a lack of knowledge of structures and strategies conducive to strengthening primary care in Europe. The EC funded project Primary Health Care Activity Monitor for Europe (PHAMEU) aims to fill this gap by developing a Primary Care Monitoring System (PC Monitor) for application in 31 European countries. This article describes the development of the indicators of the PC Monitor, which will make it possible to create an alternative model for holistic analyses of primary care. Methods: A systematic review of the primary care literature published between 2003 and July 2008 was carried out. This resulted in an overview of: (1) the dimensions of primary care and their relevance to outcomes at (primary) health system level; (2) essential features per dimension; (3) applied indicators to measure the features of primary care dimensions. The indicators were evaluated by the project team against criteria of relevance, precision, flexibility, and discriminating power. The resulting indicator set was evaluated on its suitability for Europe-wide comparison of primary care systems by a panel of primary care experts from various European countries (representing a variety of primary care systems). Results: The developed PC Monitor approaches primary care in Europe as a multidimensional concept. It describes the key dimensions of primary care systems at three levels: structure, process, and outcome level. On structure level, it includes indicators for governance, economic conditions, and workforce development. On process level, indicators describe access, comprehensiveness, continuity, and coordination of primary care services. On outcome level, indicators reflect the quality, and efficiency of primary care. Conclusions: A standardized instrument for describing and comparing primary care systems has been developed based on scientific evidence and consensus among an international panel of experts, which will be tested to all configurations of primary care in Europe, intended for producing comparable information. Widespread use of the instrument has the potential to improve the understanding of primary care delivery in different national contexts and thus to create opportunities for better decision making. [ABSTRACT FROM AUTHOR]

The article presents several information regarding different Italian artists, their works and art exhibitions. They include a symposium held in 2005 that discussed three polychrome wood-relief sculptures in Milan, Italy. Meanwhile, the teaching collection formed in Oxford, England by professor John Ruskin was studied in by Robert Hewison in his "The Ruskin Art Collection at Oxford: Catalogue of the Rudimentary Series, in the Arrangement of 1873, with Ruskin's Comments of 1878." Furthermore, the etchings and drypoints of artist Ernest Herbert Whydale had been the subject of an exhibition in Hertfordshire, England.

In the last decades, an increase in bronchial asthma and type 1 diabetes occurrence has been observed in affluent countries, and a positive association between the two disorders has been demonstrated at the population level. This association could be explained by common risk factors predisposing to both disorders. Altered environmental and lifestyle conditions, possibly related to socio-economic status, might account for the rising trend of the two disorders. To test this hypothesis, we calculated the correlation between the occurrence of type 1 diabetes and asthma, the gross national product (GNP) and the infant mortality rate, in several European and extra-European countries. GNP was positively correlated with the incidence of type 1 diabetes and with symptoms of asthma in European (rsp: 0.53 and 0.69; p = 0.001 and p < 0.0001, respectively) and extra-European countries (rsp: 0.44 and 0.46; p = 0.04 for both diseases). Infant mortality rate was inversely correlated with GNP and with the occurrences of the two diseases in Europe (rsp: −0.66, p < 0.0001 for type 1 diabetes; rsp:− 0.51, p = 0.01 for asthma). In extra-European countries, a significant relationship was found between infant mortality and asthma (rsp: −0.46; p = 0.03); a trend towards a negative correlation between infant mortality and type 1 diabetes was also found, although no statistical significance was reached (rsp: −0.21; p = 0.31). This analysis indicates that type 1 diabetes and asthma are positively associated with the GNP at the population level. Similarly, countries with low infant mortality rates tend to have a higher incidence of these immune-mediated diseases. Although GNP reflects many societal and lifestyle differences, it is notable that a high socio-economic status implies a reduced or delayed exposure to infectious agents. The reduced pressure of infectious agents on the immune system throughout life might contribute to increase the susceptibility to bronchial asthma and type 1 diabetes. [ABSTRACT FROM AUTHOR]

The effects of harvest on European forest net ecosystem exchange (NEE) of carbon and its photosynthetic and respiratory components (GPP (gross primary production) and TER (total ecosystem respiration)) were examined by comparing four pairs of mature/harvested sites in Europe via a combination of eddy covariance measurements and empirical modeling. Three of the comparisons represented high coniferous forestry (spruce in Britain, and pines in Finland and France), while a coppice-with-standard oak plantation was examined in Italy.While every comparison revealed that harvesting converted a mature forest carbon sink into a carbon source of similar magnitude, the mechanisms by which this occurred were very different according to species or management practice. In Britain, Finland, and France the annual sink (source) strength for mature (clear-cut) stands was estimated at 496 (112), 138 (239), and 222 (225) g C m−2, respectively, with 381 (427) g C m−2 for the mature (coppiced) stand in Italy. In all three cases of high forestry in Britain, Finland, and France, clear-cutting crippled the photosynthetic capacity of the ecosystem– with mature (clear-cut) GPP of 1970 (988), 1010 (363), and 1600 (602) g C m−2– and also reduced ecosystem respiration to a lesser degree– TER of 1385 (1100), 839 (603), and 1415 (878) g C m−2, respectively. By contrast, harvesting of the coppice oak system provoked a burst in respiration– with mature (clear-cut) TER estimated at 1160 (2220) gC m−2– which endured for the 3 years sampled postharvest. The harvest disturbance also reduced GPP in the coppice system– with mature (clear-cut) GPP of 1600 (1420) g C m−2– but to a lesser extent than in the coniferous forests, and with near-complete recovery within a few years. Understanding the effects of harvest on the carbon balance of European forest systems is a necessary step towards characterizing carbon exchange for timberlands on large scales. [ABSTRACT FROM AUTHOR]

Optimity Advisors conducted the study "Health system performance assessment - Integrated Care Assessment", commissioned by Chafea and DG SANTE, assessing: (i) the state of integrated care (IC) implementation in the EU28 Member States, Norway and Iceland; and (ii) developing an evidence-based Integrated Care Performance Assessment (ICPA) framework. The framework, supported by an Excel tool, was co-designed with stakeholders across Europe. The ICPA tool will be tested by members of the European Innovation Partnership on Active and Healthy Ageing (EIPonAHA), B3 Action Group on Integrated Care. From a review of literature in 24 languages, an extensive mapping of 560 IC initiatives in 30 countries and the maturity assessment of 12 health systems, we inferred that IC strategies, policies, models and projects are present across Europe, but their characteristics, depth and breadth of integration vary considerably. Taking this heterogeneity into account, the framework was codesigned drawing on user-centred design and agile methodologies. Following an iterative approach with regular stakeholder engagement (questionnaires, video-conferences, peer-review webinars, presentations at events, and a validation workshop), experts and practitioners helped us determine which indicators sourced from existing performance assessment frameworks were considered "core" to IC assessment, with the rest proposed as "optional" indicators in the performance assessment model. The core indicators selected were categorised under four domains - Advancement of integration; Use of care services; Health outcomes; Experiences of care- with a fifth overarching domain considering financial issues. Indicators have been re-worded and redefined to make them applicable across the wealth of IC initiatives while offering measures for international comparison. The heterogeneity of the context in which IC initiatives operate in Europe was a key challenge for the development of a framework that had to be relevant and potentially used in all included countries. This was addressed by providing an additional Excel-based ICPA tool offering flexibility in the use of the framework and allowing users to adapt indicators to their context. The tool allows users to compare performance to a set target for each indicator in the ICPA framework. The use of the ICPA framework and tool will help healthcare systems across Europe to further develop their IC system - based on their current state and context - and could significantly help them achieve better health outcomes and care experiences. The use of user-centred design and agile methodologies are proved methods of finding useful solutions to common challenges. Our research indicates that these principles also apply in the area of health performance assessment and can support IC implementation. Without user engagement, there was a risk that the output of the study would not provide the value sought. As IC evolves, the framework will need to be updated and adapted to new circumstances. Further user engagement and testing is recommended to ensure usability. With the research concluded, the framework and tool will need to be tested in practice through the involvement of members of the B3 Action Group of the EIPonAHA. Preliminary results will be resented at ICIC19. A web-based tool could facilitate uptake and data collection and analysis. [ABSTRACT FROM AUTHOR]

Offshore wind energy is growing continuously and already represents 12.7% of the total wind energy installed in Europe. However, due to the variable and intermittent characteristics of this source and the corresponding power production, transmission system operators are requiring new short-term services for the wind farms to improve the power system operation and security of supply. For this purpose, the incorporation of energy storage systems to provide those services with no or minimum disturbance to the wind farm is a promising alternative. Taking into account the rapid progress of the energy storage sector, this review assesses the technical feasibility of a variety of storage technologies for the provision of several services at distinct locations of a point-to-point high-voltage direct-current connected offshore wind farm. In pursuit of this goal, a novel multidimensional systematic assessment is presented. The results provide a comprehensive representation of the present state of the art and indicate potential research avenues for the enhancement of the technical viability of the technology. [Display omitted] • Critical review of relevant services provided by HVDC-connected offshore wind farms. • Critical review of storage types that can be operated in offshore wind farms. • Research state analysis of the combination of storage types, locations, and services. • Color-coded tables summarizing the research state of the aforementioned combinations. • Identification of future research directions based on a sensitivity analysis. [ABSTRACT FROM AUTHOR]

One emerging disease of grapevine in Europe is Bois noir (BN), a phytoplasmosis caused by "Candidatus Phytoplasma solani" and spread in vineyards by the planthopper Hyalesthes obsoletus (Hemiptera: Cixiidae). Here we present the first full characterization of the bacterial community of this important disease vector collected from BN-contaminated areas in Piedmont, Italy. Length heterogeneity PCR and denaturing gradient gel electrophoresis analysis targeting the 16S rRNA gene revealed the presence of a number of bacteria stably associated with the insect vector. In particular, symbiotic bacteria detected by PCR with high infection rates in adult individuals fell within the "Candidatus Sulcia muelleri" cluster in the Bacteroidetes and in the "Candidatus Purcelliella pentastirinorum" group in the Gammaproteobacteria, both previously identified in different leafhoppers and planthoppers. A high infection rate (81%) was also shown for another symbiont belonging to the Betaproteobacteria, designated the HO1-V symbiont. Because of the low level of 16S rRNA gene identity (80%) with the closest relative, an uncharacterized symbiont of the tick Haemaphysalis longicornis, we propose the new name "Candidatus Vidania fulgoroideae." Other bacterial endosymbionts identified in H. obsoletus were related to the intracellular bacteria Wolbachia pipientis, Rickettsia sp., and "Candidatus Cardinium hertigii." Fluorescent in situ hybridization coupled with confocal laser scanning microscopy and transmission electron microscopy showed that these bacteria are localized in the gut, testicles, and oocytes. As "Ca. Sulcia" is usually reported in association with other symbiotic bacteria, we propose that in H. obsoletus, it may occur in a bipartite or even tripartite relationship between "Ca. Sulcia" and "Ca. Purcelliella," "Ca. Vidania," or both. [ABSTRACT FROM AUTHOR]

Limited information is available on the efficacy of front-line bendamustine and rituximab (BR) in chronic lymphocytic leukemia (CLL) with reduced renal function or coexisting conditions. We therefore analyzed a cohort of real-world patients and performed a matched adjusted indirect comparison with a cohort of patients treated with ibrutinib. One hundred and fifty-seven patients with creatinine clearance (CrCl) <70 mL/min and/or CIRS score >6 were treated with BR. The median age was 72 years; 69% of patients had ≥2 comorbidities and the median CrCl was 59.8 mL/min. 17.6% of patients carried TP53 disruption. The median progression-free survival (PFS) was 45 months; TP53 disruption was associated with a shorter PFS (P = 0.05). The overall survival (OS) at 12, 24, and 36 months was 96.2%, 90.1%, and 79.5%, respectively. TP53 disruption was associated with an increased risk of death (P = 0.01). Data on 162 patients ≥65 years treated with ibrutinib were analyzed and compared with 165 patients ≥65 years treated with BR. Factors predicting for a longer PFS at multivariable analysis in the total patient population treated with BR and ibrutinib were age (HR 1.06, 95% CI 1.02-1.10, P < 0.01) and treatment with ibrutinib (HR 0.55, 95% CI 0.33-0.93, P = 0.03). In a post hoc analysis of patients in advanced stage, a significant PFS advantage was observed in patient who had received ibrutinib (P = 0.03), who showed a trend for OS advantage (P = 0.08). We arrived at the following conclusions: (a) BR is a relatively effective first-line regimen in a real-world population of unfit patients without TP53 disruption, (b) ibrutinib provided longer disease control than BR in patients with advanced disease stage., (© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background and Objectives: This study explored variations in the clinical manifestations of intravascular lymphoma (IVL) on the bases of the association with hemophagocytosis and the country where the diagnosis was made., Design and Methods: The clinical features of 50 Western patients with IVL were compared with those of 123 patients with IVL diagnosed in Eastern countries (87 diagnosed in Japan and 36 in other Asian countries), previously reported in English literature, and collected by an electronic bibliographic search., Results: Hemophagocytosis was absent in Western patients, but reported in 38 (44%) Japanese patients (p=0.00001) and in seven (19%) patients from other Asian countries (p=0.002). No clinical differences were evident between patients with hemophagocytosis-negative IVL diagnosed in Western countries, Japan and other Asian Countries. Conversely, Japanese and non-Japanese patients with hemophagocytosis-related IVL more frequently had stage IV disease, fever, hepato-splenic involvement, marrow infiltration, dyspnea, anemia, and thrombocytopenia, and rarely exhibited cutaneous or central nervous system involvement. Lymph node and peripheral blood involvement was uncommon in all subgroups. In Western patients, anthracycline-based chemotherapy was associated with a 52% remission rate, and a 2-year overall survival of 46%., Interpretation and Conclusions: The clinical features of IVL vary according to the association with hemophagocytosis, regardless of the country in which the diagnosis is made. Western, Japanese and other Asian patients with hemophagocytosis-negative IVL display similar clinical characteristics and should be considered as having classical IVL. Patients with hemophagocytosis-related IVL show significantly different clinical features. Both forms have a poor prognosis. Extensive molecular studies are needed to explore whether these clinical differences might reflect discordant biological entities within IVL.