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The inherited haemoglobin disorders and the clinical laboratory is essential in the diagnosis. Phlebotomy is the therapeutic procedure indicated to reduce blood viscosity and decreasing the risk of thrombotic events in the haemoglobin variants with high affinity for O2 or vaso-occlusive crises in Sickle Cell Disease (SCD) with double heterozygosity HbSC. We describe how therapeutic bleeding remains the treatment in two variant haemoglobinopathies, Haemoglobin Regina and the Haemoglobin Trollhattan, reported for the first time in the Italian population and a double heterozygosity HbSC. In patients with high haematocrit level when other secondary conditions of polyglobulia are excluded, a High-Performance Liquid Chromatography (HPLC) analysis for haemoglobin study should be suggested. Also in children with erythrocytosis frequently, such as the finding of Haemoglobin Okazaky.

Figure S1. Hematologic recovery after NK cell infusion in all treated patients.

Table S1. Percentage and absolute number of alloreactive NK cells in responders (A) and non-responders (B) Table S2. Description of cell processing results in responders (A) and non-responders (B)

Purpose: In acute myeloid leukemia (AML), alloreactive natural killer (NK) cells are crucial mediators of immune responses after haploidentical stem cell transplantation. Allogeneic NK cell infusions have been adoptively transferred with promising clinical results. We aimed at determining whether the composition of NK graft in terms of frequency of alloreactive NK cells influence the clinical response in a group of elderly AML patients undergoing NK immunotherapy.Experimental Design: Seventeen AML patients, in first complete remission (CR; median age 64 years, range 53–73) received NK cells from haploidentical KIR-ligand–mismatched donors after fludarabine/cyclophosphamide chemotherapy, followed by IL2. To correlate donor NK cell activity with clinical response, donor NK cells were assessed before and after infusion.Results: Toxicity was moderate, although 1 patient died due to bacterial pneumonia and was censored for clinical follow-up. With a median follow-up of 22.5 months (range, 6–68 months), 9 of 16 evaluable patients (0.56) are alive disease-free, whereas 7 of 16 (0.44) relapsed with a median time to relapse of 9 months (range, 3–51 months). All patients treated with molecular disease achieved molecular CR. A significantly higher number of donor alloreactive NK cell clones was observed in responders over nonresponders. The infusion of higher number of alloreactive NK cells was associated with prolonged disease-free survival (0.81 vs. 0.14, respectively; P = 0.03).Conclusions: Infusion of purified NK cells is feasible in elderly AML patients as post-CR consolidation strategy. The clinical efficacy of adoptively transferred haploidentical NK cells may be improved by infusing high numbers of alloreactive NK cells. Clin Cancer Res; 22(8); 1914–21. ©2016 AACR.See related commentary by Muntasell and López-Botet, p. 1831

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Supplementary Figure 1 from Synergistic Proapoptotic Activity of Recombinant TRAIL Plus the Akt Inhibitor Perifosine in Acute Myelogenous Leukemia Cells

Supplementary Figure 2 from Synergistic Proapoptotic Activity of Recombinant TRAIL Plus the Akt Inhibitor Perifosine in Acute Myelogenous Leukemia Cells

Purpose The purpose of this paper is to provide an early assessment of India’s South-South cooperation for trade and technology (SSTT) with East Africa, focusing on Ethiopia, Rwanda, Kenya, Uganda and Tanzania. It aims to analyse the role of SSTT in providing support to targeted sectors. Design/methodology/approach The paper examines SSTT, focusing on India and East Africa over a specific period (2000–2016) of its emergence, and extends the public sponsorship literature in international business (IB) to better understand the relationship between SSTT and value addition – applying to a particular case study of SSTT interventions in spices. Findings The paper highlights SSTT as a pathway to support value addition in global value chains (GVCs). Trade between India and East African countries has grown, with three developments over the period of analysis in particular: shifting trade patterns, growing share of intermediate goods trade and differences in GVC insertion. However, East African exports are largely of lower value. Capacity building to support processing capability and thriving markets can encourage greater value addition. Preliminary findings suggest early gains at the margins, as SSTT interventions have been focusing on capacity boosting with buffering and bridging mechanisms for increased volume of trade. Moving up the value chain however requires that specific value-enhancing activities continue to be targeted, building on regional capacities. Our high-level case study for spices suggests that activities are starting to have a positive effect; however, more focus is needed to specifically target value creation before export and in particular higher levels of processing. Practical implications While findings are preliminary, policy implications emerge to guide SSTT interventions. There is capacity for building higher value-added supply chains as is evident among East African countries that trade with each other – future SSTT programmes could tap into this and help build capacity in these higher-value value chains. Future SSTT programmes can take a comprehensive approach by aiming at interventions at key points of the value chain, and especially at points that facilitate higher value addition than initial processing. An example is that Ethiopia and Rwanda are likely to benefit from an expanded spice industry, but the next phase should be towards building processing for value-addition components of the value chain, such as through trade policies, incentivising exporters to add value to items before export. From a development perspective, more analysis needs to be done on the value chain itself – for instance, trade facilitation measures to help processers engage in value chains and to access investments for increasing value add activities. (iv), Future research should examine more closely the development impacts of SSTT, namely, the connection between increased trade, local job creation and sustained innovation, as it is these tangible benefits that will help countries in the Global South realise the benefits of increased trade. Originality/value The paper underlines how the SSTT approach can contribute to the critical IB and GVCs literature using a theoretical grounded approach from public sponsorship theory, and with a unique lens of development cooperation between countries in the global south and its emerging impact on development outcomes in these countries.

We distinguish the geographical source and destination of value added contributed by each country-industry to each country-global-value-chain (GVC) from both input sourcing and output destination perspectives. We define indicators that capture the relative intensity of regional vis-à-vis global (i.e., extra-regional) components of foreign value added and employment. Europe is increasingly sourcing value added from within the region ("nearshoring") but exporting value added globally ("farsharing"). European GVCs mostly generate foreign employment outside Europe. In contrast, most of European employment is activated by European GVCs: an important labor market policy implication arguing for more intensive intra-EU trade and integration. Europe and its economic policy should not only focus on the sourcing of value added across production processes, but also on the final demand that generates economic activity in Europe

We study green specialisation across EU countries and detailed 4-digit industrial sectors over the period of 1995-2015 by harmonizing product-level data (PRODCOM). We propose a new list of green goods that refines lists proposed by international organizations by excluding goods with double usage. Our analysis reveals important structural characteristics of green specialisation in the manufacturing sector. First, green production is highly concentrated, with 13 out of 119 4-digit industries, which are high-tech and account for nearly 95% of the total. Second, green and polluting productions do not occur in the same sectors, and countries specialise in either green or brown sectors. Third, our econometric analysis identifies three key drivers of green specialisation: (i) first-mover advantage and high persistence of green specialisation, (ii) complementarity with non-green capabilities and (iii) the degree of diversification of green capabilities. Importantly, once we control for these drivers, environmental policies are not anymore positively associated with green specialisation.

This paper provides empirical evidence on the complex role played by technology in affecting the relationship between the participation of EU countries and industries in Global Value Chains (GVCs) and their employment structure over the period 2000-2014. The empirical analysis is based on country/industry level data for 21 EU countries on employment, trade in value added, patents and investments in intangible assets, and focusses on backward linkages within GVCs. The role of technology is analysed by taking into account both the technological intensity of offshoring industries and that of their GVC partners. We study the employment structure by looking at the shares of managers and manual workers, which reflect the “functional specialisation” of the country-sector within GVCs. We find that pre-existing asymmetries in the functional specialisation are highly persistent over time, with little sign of convergence over our observed period. Furthermore, GVC participation is not related to changes in the employment structure. However, this relationship appears to be mediated by country-industries’ initial technological performance. Technological leader industries exhibit, in fact, larger shares of employment in headquarter functions, and this functional specialisation tends to be strengthened as they increase their integration into GVCs. In contrast, country-industries that start off as technological laggards see integration into GVCs accompanied by an increase in the share of employment in fabrication functions. The technological profile of the partners is also found to play a role in the relationship between GVC integration and the functional specialisation of the offshoring country/industry, although different patterns emerge depending on the nature of the partner (manufacturing vs service).

This paper provides empirical evidence on the relationship between patterns of integration in global value chains (GVCs), the ‘quality’ of such integration in terms of technological capabilities and the structure of employment in European countries and sectors. We study employment shares in fabrication and headquarter occupations in terms of functional specialisation and employment upgrading, making two specific contributions. First, we explicitly account for pre-existing asymmetries in employment structure. Second, we account for the complex role of technology both in terms of country-industries’ own initial technological intensity and that of their GVC partners. To achieve this we blend data on employment, trade in value added, patents and intangible asset stocks, for the period 2000-2014. We find that such pre-existing asymmetries are highly persistent over time with little sign of convergence in employment structure over our observed period. Furthermore GVC participation is not related, in and of itself, to changes in employment structure. However, this relationship appears to be mediated by country-industries’ initial technological position. Technological leaders exhibit greater shares of employment in headquarter functions as they increase their integration in GVCs; in contrast country-industries that start off as technological laggards see further integration in GVCs alongside an increase in the share of employment in fabrication functions.

This article investigates whether trade specialization explains economies’ trade performance within the context of global value chains (GVCs). We consider trade specialization in natural resources (NR), high- and low-tech manufacturing (LTM and HTM) and business services, before and after the financial crisis. The aimed contribution of this article is to shed light on the effects of trade specialization as measured in domestic value added embodied in exports rather than gross exports. We add to the literature on GVCs by (i) studying the role of the domestic productive structure in countries’ trade specialization and performance, (ii) accounting for the rate of changes in trade specialization as affecting GVC performance. We employ Balassa indexes based on value added flows in a GMM dynamic panel framework. Our results suggest that trade specialization in LTM and NR relate to a decline in the growth of domestic value-added per capita exports relative to other countries. HTM and knowledge-intensive business services exhibit a positive effect during the crisis period. We discuss these findings in relation to the recent debates on the role of manufacturing and premature de-industrialization in developing countries.