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Additional file 1. Table S1. Number of enrolled patients for each enrolling center; Table S2. Estimated incidence of TTP in the Region of Lombardy.

Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare life-threatening thrombotic microangiopathy (TMA) affecting more frequently women of 30–50 years of age. There is scarce information on the clinical features of aTTP occurring in the elderly. Our goal was to evaluate the impact of an elderly-onset disease on the expression, severity and management of aTTP. Materials and methods: We performed a cross-sectional study of patients enrolled in the Milan TTP Registry (www.ttpdatabase.org) after a first acute episode of aTTP from January 2002 to March 2018. The aTTP diagnosis was suspected on the basis of the presence of thrombocytopenia and microangiopathic hemolytic anemia with no alternative causes, and was confirmed centrally by a severe plasma deficiency of ADAMTS13 activity (

© 2020 The Author(s). In the context of trade, the EU emerges as a singular global actor from a fundamental rights perspective: unlike other international actors, the EU’s external action is to be guided by interests as much as values. The Treaty of Lisbon does not erase the tension between market goals and respect of fundamental rights, it opens up the possibility for the EU to pursue fundamental rights both in and through trade. Yet for a very long time, the EU has been a global actor through trade: it has taken for granted an understanding of human rights in trade that sees them as a development issue for third countries. No more sophisticated conceptualisations have been explored, making the EU’s current approach heavily reliant on this legacy. The way fundamental rights are provided protection in the Post-Lisbon new generation EU trade agreements emerges as outdated and not fit for purpose. The aim of this paper is to urge new conceptualisations of the relationship between trade agreements and fundamental rights. https://www.asser.nl/cleer/publications/cleer-papers/cleer-paper-20201-kassoti-wessel-eds/

International audience

The paper explores the descriptive and normative intersection of Data Protection rights and Regulatory Cooperation chapters in the new generation of EU Trade Agreements. While the EU has repeatedly refused to consider data protection as part of trade negotiations, it has sought deeper institutionalised forms of regulatory cooperation: the question then arises as to what extent data protection rights would come across with these mechanisms and the consequences on their protection. In a context of global trade requiring data to flow freely, regulatory cooperation has been advanced as a way to deal with regulatory divergences in data. How should the EU deal with global demands pooling data flows and regulatory cooperation? The paper finds that data protection emerges as a very much cross-cutting issue liable to be affected by Regulatory Cooperation activities. It is argued that the EU should acknowledge this state of play whereby data protection cannot be isolated or only elusively addressed; instead, mechanisms should be secured so as to ensure that data protection, as a fundamental right, is not undermined, and is instead embedded and enhanced in the regulatory cooperation chapters.

The EU increasingly sets new international agendas, standards and rules and is referred to in a vast literature as a ‘norm promoter’. An increasingly explicit endeavour of the EU is to drive global data convergence. The EU now has data transfer regimes which count as some of the largest in the globe and this article focuses upon how the EU and Japan have recently agreed on a reciprocal recognition of the adequate level of protection, which is said to create the world's largest area of safe data flows. The recent EU-Japan adequacy decision represents an another significant global endeavour, ostensibly creating global reach. This innovation is worthy of reflection given the scale of data transfer involved, but most importantly to study the assessment of “equivalence” of two legal orders, and the processes of convergence and institutionalisation at play. We contend that it is of significance and an important coincidence that the adequacy decision was a side-product of the EU-Japan Economic Partnership Agreement (EPA): despite the EU’s initial goal of excluding data from the trade negotiations, Japan insisted on data dialogues and the EU eventually accommodated the demands, hence triggering the process for an assessment of equivalence. This process is of interest given the scale of the agreement but also the broader parameters of how a partner proposes a field not aligning with EU interests and ends up becoming subject to significant EU institutionalisation procedures. The EU-Japan efforts at reaching convergence provide a unique setting to study the EU as an emerging global legal actor in data. How do we understand then the EU as a global actor in data and its efforts and willingness to be so? How does the EU nudge convergence more or less intentionally towards its own standards? Alternatively, which mechanisms make convergence happen as a result of “unintended consequences” as opposed to a more ‘hard’ power of the EU? What lessons do we learn from the adequacy decision negotiations as to the EU as a global data convergence actor? We argue that convergence and institutionalisation appear as outcomes of the EU accepting to engage in data dialogues with Japan, thus resulting in ‘accidentality’ of ending up with negotiating an adequacy decision with Japan. The convergence and institutionalisation are important outcomes of the EU and Japan efforts to reach a mutual adequacy decision and resonate with how we understand the EU as a global convergence actor. We argue that convergence and institutionalisation are important ‘accidental’ outcomes of the adequacy decision and show the EU to be a flexible global trade actor.

This paper explores the institutional dimension of the implementation of the new generation EU Free Trade Agreements (FTAs) from a fundamental rights perspective. The creation of an institutional architecture of EU FTAs is what makes them operational. It enables the other dimensions to become “alive” and not to remain words within a text. Given the plethora of entities created by the new generation of EU FTAs, this paper investigates the extent to which the institutional architecture of EU FTAs is adequate to protect and promote fundamental rights. This investigation shows that, despite significant novelties, several limitations remain. Gaps in the mandate and deficiencies in the decision-making processes lead to foresee little consideration of fundamental rights at the implementation stage.

This paper outlines an exploratory workshop at City Law School, City, University of London funded by HEIF/ ‘EUTIP’ Marie Skłodowska-Curie Innovative Training Network (ITN) on understanding of the EU as a Good Global Actor.1 The EU has as its mission to be a good global governance actor yet is continuously challenged in the world. As a global actor, the EU is both a weak and strong actor in a divergent range of global governance areas. It is not comparable to study the EU as a global trade actor for example to its efforts in human rights, data, cyber or the environment. EU international relations constitutes arguably a booming field of law where the EU appears often to be a victim of its own success. The range of the subjects and objects of EU law continues to expand and the EU is arguably increasingly a victim of its own success, increasingly taking decisions with impacts on third countries or parties, subjecting more entities to sanctions regimes, being bound to consult more entities and have more third countries, parties and entities such as lobbyists interested in the directions of EU law. The assessment of the EU as a global actor includes broad checks on normative action ex ante and ex post facto- yet it is no less harsh. Ex ante metrics of EU global action include court-centred ones such as an opinion from the CJEU on legality of an international agreement, often precluded in most constitutional systems on account of its conflict with pacta sunt servanda. The contours of the principle of the autonomy of EU law have the capacity to put more stringent parameters on EU institutionalised evolutions as to international engagement. How can we assess the EU as a global actor given these realities? The aim of the event was to explore informally the nexus between trade and security, trade and economics and trade and human rights as a future research agenda with input from a variety of scholars It reflected upon four major themes: 1) The EU’s Contribution to the Democratisation of Global Governance 2) Deeper Trade Agreements and New Normative Foundations 3) The EU as a Global Actor in Trade and Fundamental Rights 4) EU’s Trade in the Era of Global Data Flows.