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Currently there is no established standard of care for Hodgkin's disease (HD) in Russian Federation (RF). Th e mortality from HD in RF is 28,3%, thus improvement of care is required. Here we describe the design and the rational for the fi rst cooperative prospective study in the Nort-West region of RF, RNWOHG-HD1. Th e key points of the protocol are discussed, including escalation from ABVD to BEACOPPesc in case of PET-positive disease aft er fi rst two cycles in the favorable prognosis group, and de-escalation to randomized ABVD/AVD in case of PET(-) status aft er fi rst two BEACOPPesc courses in the unfavorable prognosis group. Th e protocol also is planned to facilitate access to second and third line treatments, including brentuximab, as well as autologous and allogeneic stem cell transplantation.

Background. Secondary hemophagocytic lymphohystiocytosis (sHLH) is a hyperinflammatory reaction provoked by some trigger (cancer, autoimmune or infection). The majority of affected patients are at high risk of fatal multiple organ failure without getting immunsupressive treatment.Objective. Clinical and laboratory profile of sHLH patients.Materials and methods. Retrospective study included clinical, instrumental and lab data from the 91 patients followed between June 2009 and June 2019. Diagnosis sHLH had been based on HLH-2004 and H-Score criteria. The analyzed parameters had been fever chart, liver and spleen enlargement, changes in the bone marrow; values levels of glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, alkaline phosphatase, bilirubin, triglycerides, total ferritin with percentage of glycosylation. All patients with rheumatic disorders or malignancies had received either immunosuppressive or cytotoxic therapy. Febrile patients received anti-infective treatment according to the local routine protocols.Results. The data from 91 patients (41 male and 50 female) had been analyzed. Median age was 58 (2–90) years. The sHLH trigger-diseases spectrum included leukemia/lymphoma (n = 52), infection diseases (n = 11), autoimmune disorders (n = 5), allogenic bone marrow transplantation (n = 13), unidentified (n = 10). A fever with an unknown origin and refractory to antibacterial treatment had been observed in 87 (96 %) patients. Morphological hemophagocytic evidences in the bone marrow had been found in 83 %. Breath shortening, liver failure, neurologic disturbances, systemic effusions, rash, heart failure had been registered in 83 % patients. Detected splenomegaly presented in 56 %. Laboratory changes, median were as following: serum glutamic-pyruvic transaminase (alanine aminotransferase, SGPT) – 92 (39.2–1060.8) IU/L; serum glutamic oxaloacetic transaminase (aspartate aminotransferase, SGOT) – 105 (40–4177) IU/L; alkaline phosphatase – 225 (120.9–989) IU/L; bilirubin – 50.5 (22–559) µmol/L; triglycerides – 3.2 (1.95–8.6) mmol/L; total ferritin – 10000 (597–255000) ng/mL with glycosylation percentage – 20.45 (0–37.8) %. 71 patients received various of HLH-directed therapy courses. The overall survival rate was 27 %, median follow-up – 540 days.Conclusion. The main clinical and instrumental findings in sHLH are fever, refractory to anti-infective treatment, elevation of transaminases, serum alkaline phosphatase, triglycerides, total ferritine with low glycosylated fraction. Early diagnosing and immunesupression are the main factors of survival.

Introduction. Successful solution of the tasks of socio-economic development in the modern world is possible only in the case of effective functioning of the education system, which would provide training for a competitive personality, focused on continuous development and capable of “complying” with rapidly changing conditions of social reality. It is assumed that building an education development strategy, setting its goals and objectives should be based on the needs and problems of society, creating the basis for sustainable prosperity of the country and taking into account the needs of individual regions and territories. One of the most important mechanisms for the development of the regions of the Russian Federation remained the state educational order – officially developed task by the authorities for the creation of a specific educational product.The aims of the present article are the following: to determine the relationship between the educational state order, the volume of investments in the education system and the level of socio-economic condition of the regions; to identify the territories, which require support.Methodology and research methods. In the course of the study, the methods of comparative, statistical and system analysis, deduction and induction, generalisation and theoretical modelling were used. The object of the content analysis was the regional programmes for the development of education in 40 regions of the Russian Federation, covering the entire territory of Russia and reflecting the specific development of various regions depending on the remoteness of the capital, the status of the subject of the Russian Federation and the socio-economic development of the region.Results and scientific novelty. Russian regions were classified into four groups according to the results of the analysis of regional educational development – very high, high, moderate and low. A number of general and specific problems of education in the regions were identified: the lack of a single educational space and, as a result, the heterogeneity of educational programmes; regional leaders disregard for opportunities to expand socio-economic potential of the territories through the implementation of such programmes; the gap between the financing of education and its socio-economic importance, etc. The authors conclude that state educational order at the regional level is not considered as a vector of social and economic development of the territories, and its role is reduced to an educati onal level. It is established that the rate of development of education in the Russian regions does not always depend on their key socio-economic indicators. The greatest differentiation of the trend is recorded, when comparing the rate of development of the regional education systems and the gross regional product.Practical significance. The authors give recommendations to resolve disagreements over the formation of state education orders in the context of the socioeconomic development of regions. The need to develop regional economic development programmes, taking into account the specific social structure of the region, is emphasised. It is proposed to organise permanent social monitoring, which measures the quality of implementation of educational state order and strengthen the role of public participation when determining its content. The research materials and findings might be used by the executive authorities of the constituent entities of the Russian Federation, whose competence includes educational development issues.