Background. Modern medicine and highly specialized care are impossible without a successfully functioning state blood donation system. Solving the practical problems of organizing voluntary donation in a single country requires research to identify and take into account its economic, national and law characteristics. At the moment, the sociodemographic characteristics of the current donor in Russia and the CIS remain poorly understood. The key indicators that should be measured in the first place are the following: socio-demographic characteristics of donors, factors that attract/ put off donors, donor motivation, and quality control of the donor organization.

Aim. The purpose of the study was to determine the socio-demographic characteristics, some psychological characteristics and perceptions of donation among visitors to the blood donation service of the National Research Center for Hematology (Moscow, Russia).

Materials and methods. The authors conducted a study of the characteristics of blood donors (n = 568).

Results. Based on the results of the research, we describe a portrait of a modern donor: men and women from 20 to 25 years, mainly managers / specialists and students. The motivation of most donors can be described as prosocial (“humanistic”). The views on the donor, donor activity, the level of awareness of the problems of donorship in Russia among the respondents are detailed in the “Results” and “Discussion”. We provided data on the preferences of different types of compensation, the effectiveness of information dissemination channels and the degree of involvement in the activities of the service.

Conclusion. Prospective directions of development of voluntary donation organization were discussed and the possibilities of conducting additional interdisciplinary research were considered in detail.

Introduction. The use of European reference standard with the known structure (СRS Нuman coagulation factor) as a reference allows to confirm identity of the primary structure of new batches of the recombinant protein. Manufactures normally use an industry standard sample due to the high cost of the European reference standard and to make sure that national samples are independent from the international reference standards. Regulatory requirements for the qualification procedure of an industry standard sample are absent.

Materials and methods. Batch of recombinant activated clotting factor VII (rFVIIa) (Eptacog alpha) («Koagil») was selected as a candidate material. The method of strong hydrolysis with the initial deglycosylation of the molecule was used. Amino acid sequence of rFVIIa, described in the monograph 01/2015:2534 of European Pharmacopoeia (Ed. 9.0) was used as a reference. Peptide mapping and mass-spectrometric assay were used in the study.

Results. Qualification procedure of industry standard sample of rFVIIa was developed. The standard sample was used for the proof of identity of rFVIIa by RP-HPLC peptide mapping. Qualification procedure of an industry standard sample of rFVIIa allows to obtain fully characterized reference sample with the established structure. The use of this standard sample guarantees accurate assessment of the identification by peptide mapping of the new batches of rFVIIa with or without European reference standard.

Conclusion. Peptide mapping and mass-spectrometric assay allows us to confirm amino acid sequence of light and heavy chains of rFVIIa with 100 % coverage. The peptide map was established for the candidate material in comparison with the original drug NovoSeven and the selection of сharacteristic peaks was justified. These peaks were characterized by stability and symmetry factors. Amino acid sequence of peptides corresponding to the reference peaks and their primary localization in the molecule were established by the use of tandem mass-spectrometry.

Conflict of interest. The authors Smolov M. А., Shukurov R. R., Vishnevskiy A. Yu. are the employees of the International Biotechnology Center «Generium».

This article reviews the data on the changes of erythroid cells intracellular organization during maturation (including cytoskeleton and surface determinants formation, enucleation, and reticulocytes migration into the bloodstream), and primary transcription factors involved in the realization of erythropoietin signaling. The features of erythrocytes movement in the bloodstream; their deformability, form and membrane ion permeability variations depending on the bloodstream sector are described. The erythrocytic factors capable of modulating the vascular tonus, hemostasis, and inflammation degree, as well as the erythrocytes receptors and surface determinants are discussed. The mechanisms and consequences of intravascular and intracellular hemolysis; eryptosis molecular mechanisms, inductors and inhibitors; the pathologies associated with enhanced eryptosis and principle of «splenic filter» functioning are analyzed. 61 sources were analyzed, which were identified through systematic literature search in PubMed (Medline, PreMedline), scientific electronic library (eLIBRAR.ru) and among printed publications appeared from 1997 to May 2017.

Cytokines are an integral part of normal hematopoiesis and affect virtually all of its stages, causing and regulating the processes of cell proliferation, differentiation and apoptosis. According to recent studies, it has been shown that cytokines also mediate complex interactions between hematopoietic, immune systems and a growing tumor. On the one hand, cytokines take part in the activation of antitumor immunity aimed at eliminating malignant cells; on the other hand, they are synthesized by tumor cells and participate in the progression and metastasis of tumors. The article summarizes the data of modern literature on the characteristics of changes in the cytokine profile in patients with acute myeloid leukemia (AML). The role of cytokines in leukemogenesis is shown: in activation of signal transmission pathways, interaction with bone marrow microenvironment, implementation of antitumor immunity and maintenance of persistence of clonal blast cells. The connection of cytokines with the results of treatment and the prognosis of the disease, taking into account the presence in the patient’s genotype of various allelic variants of cytokine genes encoding high or low production of these factors, is highlighted. The data presented in the review on the participation of cytokines in the formation, development and elimination of tumor cells in hemoblastoses are sometimes contradictory. However, these contradictions may be explained by the concept of tumor immunoediting, according to which the cells of the immune system can be transformed by a tumor in the process of carcinogenesis and start actively promoting its growth.

Myeloproliferative neoplasms (MPNs) are a group of clonal hematopoietic disorders of stem cells characterized by aberrant proliferation of one or more myeloid lines. MPNs are extremely rare in patients younger than 20 years old, for example, polycythemia vera (PV) is about 2 cases per 10 million people per year. The true prevalence of PV and treatment standards for pediatric patients are not defined. The goal is to analyze the identified cases of polycythemia vera (PV) in patients younger than 20 years and create an algorithm for the choice of therapy.

Materials and methods. The analysis of 7  patients with PV at the age under 18 years (3 months — 14 years), 6 of them are boys and 1 is girl. The patients underwent general clinical studies, morphological, histological, cytogenetic, molecular genetic studies of the bone marrow, ultrasound studies of the abdominal organs and vessels. Cytoreductive therapy was performed with pegylated interferon, and in the absence of a response — ruxolitinib.

Results. In the debut of the disease, splenomegaly of various degrees was detected in all patients, the total number of leukocytes (WBC) > 10.0  × 109/L, the number of neutrophils 6.2—13.5  × 109/L, the number of red blood cells (RBC) 5.6—8.9 × 1012/L, in 4 patients — platelet count (PLT) > 1000 ×109/L (1103—3000 × 109/L). No cases of throm bosis or bleeding were noted. In 100% of cases, a mutation was detected in the JAK2 gene (6 patients with the mutation JAK2V617F, 1 patient with a mutation in exon 12 of the JAK2 gene p.1613_1616delACAAinsT). The allelic load in the debut of the disease was 14—33% (n = 4) and 35—66% (n = 3). With pegylated interferon α2 (peg-INF α-2a) therapy, a full response to therapy was obtained in 2 cases, a partial response — in 2 cases, in one of them the therapy was discontinued due to pronounced toxicity. Second-line therapy (ruxolitinib) was performed in 3 patients and after 6 months partial remission was achieved in the form of a hematocrit decrease of less than 45% without bloodletting. The tolerability of ruxolitinib is satisfactory; no adverse events requiring dose reduction or complete withdrawal were noted.

Conclusion. Considering the extremely rare occurrence of PV in patients younger than 18  years of age, as well as the results of long-term follow-up (disease outcomes: frequency of progression in acute myeloid leukemia or myelofibrosis), it is necessary to continue collecting information on patients with debut of the disease earlier than 18 years. For patients younger than 18 years old, it is advisable to use peg-INF α-2a as the first line of cytoreductive therapy, in the absence of response and/or in case of intolerance to peg-INF α-2a, switch to the second line of therapy, ruxolitinib, in the absence of response or progression of bone marrow fibrosis (MF2 and more) it is necessary to consider the transplantation of allogeneic hematopoietic stem cells as the only curative method.

Adherence to proper indications for red blood cells (RBC) transfusion is essential because of its potential adverse effects and costs of therapy. Aim of these recommendations is to summarize typed of RBC concentrates and indications for RBC transfusions among different categories of the patients. Methods. The methodological approaches are based on the recommendations of the Russian expert council (leading specialists of the Russian Federation) and literature search for randomized clinical trials evaluating RBC storage duration, hemoglobin thresholds and clinical indications for RBC transfusion without language restrictions. Results. The draft clinical guidelines were reviewed on February 1, 2018 at First Russian Transfusiology Congress of the (Vladivostok). The main types of RBC concentrates, storage duration, transport conditions and indications for RBC transfusions are presented. The indications for RBC transfusions are analyzed for various clinical conditions (in obstetrics, neonatology, hematology, cardiology, neurosurgery, nephrology, in patients with sepsis and septic shock, patients with acute blood loss, in patients after hematopoietic stem cell and organ transplantation). Conclusion. The recommendations are intended for doctors of various specialties, health administrators, medical students.