Introduction. A system of specialized transfusiology care in a day hospital has been created in the Novosibirsk Region, the system for ensuring the immunological safety of allogeneic transfusions has been improved, and a new method for harvesting hematopoietic stem cells (HSCs) has been mastered.

Aim: to evaluate the possibility of organizing and providing specialized medical care in the field of transfusiology in a day hospital.

Material and methods. The study included the results of substitution transfusion therapy in 1,877 cases of day hospital admissions, 32,883 laboratory studies conducted in 10,276 patients who needed individual selection of RBC-containing blood components, 138 immunological studies for 28 patients with refractory transfusion who needed individual selection of platelet concentrate, 279 immunogenetic studies in 76 patients who needed selection of a compatible, HLA-gene-identical donor relative, examination of 10 HSC donors and quality control of the obtained cell products.

Results. The Outpatient Transfusion Care Center provided replacement transfusion therapy with RBC containing components to hematological and oncological patients in the amount of 6,495 doses (efficacy — 99.5 %), platelet concentrates — 986 doses (efficacy — 91.2 %); individual selection of 22,385 doses of RBC–containing blood components (99.8 % selection efficiency), individual selection of 63 doses of platelet concentrates (71.4 % selection effectiveness for individuals with HLA antibodies), selection of an HLA-identical/partially identical relative donor for 19 patients (25 % effectiveness), preparation of 10 therapeutic doses of allogeneic HSCs from healthy donors.

Conclusion. The results of the functioning and clinical effectiveness of the new form of specialized medical care in the field of transfusiology prove the possibility of providing transfusion care in out-of-hospital settings, ensuring the immunological safety of allogeneic transfusions and harvesting hematopoietic stem cells by a blood service institution.

Introduction. For strategic planning, it is necessary to differentiate the impact of demographic changes in the population itself and the specific behavioral patterns of different age and gender groups of donors. The combination of these factors affects the structure and reliability of the donor reserve, making the analysis of long-term trends important for the strategic planning of the blood service.

Aim: to analyze the changes of the age and gender composition of blood donors, compare it with the age structure of the Moscow population and assess the loyalty of various demographic groups.

Materials and methods. In a retrospective study, the age and gender structure was analyzed: indicators and relative proportions of donors in six age groups (18–25, 26–30, 31–35, 36–45, 46–55, 56–99 years) separately for men and women. For each age and gender group, the proportion of repeat donors is calculated for each year of follow-up — the ratio of the number of donors who made more than one donation during the registration period to the total number of donors in this group for the corresponding period. To differentiate the influence of demographic and behavioral factors on the age structure of the donor population, a comparative analysis was carried out with the data on the age composition of the Moscow population for 2015–2024. For comparability, donors (18–30, 31–45, 46–55, 56–99 years old) were compared with the corresponding population groups (20–29, 30–49, 50–59, 60 years and older).

Results. The aging of the donor population was revealed (the core shift from the 18–25-year-old group to the 36–45-yearold group, which by 2024 amounted to 30.9 %), a change in gender dominance from female (54.4 % in 2015) to male (58.9 % in 2024), as well as the superiority of loyalty of male donors aged 18–55 years. The formation of a new age core of donors was an independent behavioral phenomenon, rather than a consequence of demographic aging of the population. The COVID-19 pandemic period (2020–2022) acted as a catalyst for growth, but did not change the underlying demographic trends.

Conclusion. It is necessary to adapt blood service strategies to the identified changes in the donor population, where behavioral factors play a key role along with demographic trends.

Introduction. Regulatory control contributed to the development of hematology, the strengthening of infrastructure and human resources, as well as the regulation of general organizational issues. Currently, the quality of the regulatory framework for the functioning and development of medicine as an industry is not completely perfect and requires development and harmonization.

Aim: to study the regulatory documents regulating the provision of medical care in the field of “hematology”.

Materials and methods. Legislation in the field of healthcare has been studied based on search queries in the State Archive of the Russian Federation, the Consultant Plus and Garant databases. The research methods were an analytical review, analysis and synthesis of relevant information.

Results. Work has been carried out on the analysis of normative and historical documents, including those not available in open sources, concerning the formation and development of hematology as part of the healthcare system. References are provided not only to archival documents, but also to current regulations governing various aspects of the organization of hematological care for patients with diseases of the blood system: infrastructural, laboratory diagnostic, documentary, personnel, registration and accounting of hematological diseases. The difficulties in the application of regulatory legal acts are shown today, when documents contradict each other at the federal level, and at the same time are ambiguously interpreted at the level of subjects of the Russian Federation, the concept of financial and organizational regulatory document management is mixed.

Conclusion. Regulatory issues are the basis of the organization of both healthcare in general and care for hematological patients. The work on improving and harmonizing the regulatory framework and regulatory system is an important aspect of the work of the entire professional community in close cooperation with the Russian Ministry of Health.

Introduction. Internal tandem duplication (ITD) in the FMS-like tyrosine kinase 3 (FLT3) gene is a poor prognostic factor in acute myeloid leukemia (AML). Identification of a group of such patients raises the question of prescribing targeted therapy to them.

Aim: to assess the significance of minor FLT3-ITD clones in AML with NPM1 mutation.

Materials and methods. The study included 58 patients belonging to the intermediate or favorable risk group. Patients underwent therapy according to the AML-21 protocol. Molecular genetic diagnostics of FLT3 mutations was performed by polymerase chain reaction (PCR) with fragment analysis (PCR-FA), PCR-FA with double label and tandem duplication PCR (TD-PCR), NPM1 mutations — by PCR-FA (primary diagnostics) and allele-specific PCR to assess minimal residual disease (MRD).

Results. Using double-labeled PCR-FA and TD-PCR, allowed to identify minor FLT3-ITD clones in 12 (36 %) of 33 patients in whom these clones were not detected by the standard method. We also identified additional FLT3-ITD mutations in 13 (52 %) of 25 FLT3-positive patients. In the studied cohort no significant association of minor FLT3-ITD clones with the dynamics of MRD reduction, the percentage of patients achieving MRD-negative status, overall and relapse-free survival among patients with NPM1(+) AML was found.

Conclusion. The association of minor FLT3-ITD clones with clinical features of the disease in patients with AML and NPM1 gene mutations is minimal or absent. It can be assumed that these patients do not require the use of additional more sensitive methods for detecting FLT3-ITD mutations at the primary stage of diagnosis and the inclusion of tyrosine kinase inhibitors in program therapy. However, final conclusions can be made if it is shown that the detection of minor clones with FLT3-ITD mutation at the onset is not associated with the occurrence of relapses, taking into account the treatment protocol.

Introduction. Chronic pruritus (CP) is one of the symptoms of cutaneous T-cell lymphomas (CTCL), which significantly reduces the quality of life of patients.

Aim: to identify the factors associated with CP and describe the phenotypes of CP in patients with CTCL to determine the factors associated with CP and describe the phenotypes of CP in patients with CTCL.

Material and Methods. A total of 58 CTCL patients were examined. The assessment included psychiatric evaluation according to ICD-10 and psychometric instruments: the Eppendorf Itch Questionnaire, Beck Anxiety and Depression Inventories, Toronto Alexithymia Scale (TAS-26), and Symptom Checklist-90-R (SCL-90-R).

Results. CP was found in 84.5 % of patients. CP severity correlated with lymphoma stage and depressive symptoms (p < 0.05). Psychiatric disorders were diagnosed in 39.7 %, mainly affective and cognitive. Gender, age, and education had no effect on itch occurrence, whereas longer disease duration was observed in patients without CP. In 58.8 % of cases, CP was accompanied by pleasure during scratching, indicating ambivalent affective regulation. One-third of patients exhibited formication phenomena, and almost half showed social phobia associated with shame and self-stigmatization due to visible skin lesions.

Conclusion. CP in CTCL patients is a dermatological and psychoneuroimmune phenomenon Treatment should include psychiatric evaluation, correction of depression and anxiety, and psychotherapeutic support.

Introduction. The prevalence of preoperative anemia among children with congenital heart defects (CHD) has not been sufficiently studied. Anemia may be associated with postoperative complications and unfavorable outcomes after cardiac surgery. Erythrocyte suspension (ES) transfusion can also lead to undesirable side effects. There is no generally accepted approach to the preoperative preparation of patients with anemia that could improve the outcomes of surgical treatment of CHD.

Aim: to study the prevalence of preoperative anemia in children with CHD and to evaluate their effect on the course of the postoperative period.

Materials and methods. A retrospective analysis of the data of 253 CHD patients aged 0.1 to 215.5 months who underwent cardiac surgery between January 2022 and December 2023 was performed, of which 177 (70 %) patients underwent cardiopulmonary bypass. Among the most numerous and homogeneous group of patients classified as RACHS-1 category II, the effect of preoperative anemia on the duration of hospitalization and stay in the intensive care unit (ICU), the incidence of acute kidney injury (AKI) was analyzed.

Results. Preoperative anemia was diagnosed in 25.7 % of patients. Among patients with anemia, the incidence of AKI in the postoperative period was higher than in the group of patients without anemia: 30.8 % versus 9.7 %. The presence of anemia did not affect the duration of hospitalization and ICU stay.

Conclusion. The prevalence and structure of anemia before cardiac surgery in children have not been sufficiently studied. It is necessary to further search for the optimal approach to preoperative preparation of CHD patients in order to reduce the risks of postoperative complications, reduce the financial burden on healthcare institutions, and improve treatment outcomes.

Introduction. Hypoferremia is an underrecognized clinical and laboratory syndrome. Its routine markers, ferritin and transferrin saturation (TSAT), are influenced by multiple factors and may not always provide an objective assessment of iron metabolism.

Aim: to evaluate the impact of preoperative hypoferremia on the development of adverse postoperative outcomes in patients undergoing elective cardiac surgery with cardiopulmonary bypass.

Materials and methods. This retrospective cohort study included 233 non-anemic patients (hemoglobin concentration >130 g/L) who underwent cardiac surgery. The isolated effects of ferritin and TSAT levels on postoperative outcomes were assessed. Subsequently, patients were stratified into four groups according to ferritin (<30 or ≥30 ng/mL) and TSAT (<20 % or ≥20 %) levels. The primary endpoints were mortality and a composite endpoint including mortality, acute coronary and cerebrovascular events, heart failure, renal failure, and respiratory failure. Secondary endpoints included the duration of mechanical ventilation (MV), length of stay in the intensive care unit (ICU), and total hospital stay.

Results. Isolated analysis of ferritin and TSAT revealed no significant association with the risk of adverse outcomes. Stratification demonstrated that patients with ferritin < 30 ng/mL and TSAT < 20 % had a significantly higher risk of acute kidney injury (AKI) compared with the reference group (p = 0.001). After merging groups II–IV (due to the absence of significant differences among them) and performing multivariate analysis, hypoferremia was found to be associated with an increased risk of CE (OR 3.317; 95 % CI 1.024–10.750; p = 0.045), acute heart failure (OR 4.784; 95 % CI 1.169–19.582; p = 0.030), AKI (OR 6.868; 95 % CI 1.550–30.460; p = 0.011), and red blood cell transfusion (OR 3.407; 95 % CI 1.002–11.579; p = 0.050). Hypoferremia was also associated with prolonged СMV duration and length of hospitalization.

Conclusion. Hypoferremia is associated with an increased risk of adverse hospital outcomes in patients undergoing cardiac surgery. These findings support the inclusion of iron status assessment in preoperative risk stratification and underscore the importance of early detection and correction of latent iron deficiency before surgery.

Introduction. Oncohematological patients are a vulnerable category in relation to orthopneumovirus (OPV), which affects cells of the respiratory epithelium and lung tissue.

Aim: to present a clinical observation of the identification of OPV and successful ribavirin treatment of a patient with Diffuse large B-cell lymphoma (DLBCL) and acute respiratory failure (ARF) due to viral pneumonia.

Main findings. Patient S., 56 years old, was diagnosed with DLBCL of the central nervous system. After the first course of chemotherapy, he developed acute kidney injury, fever, and bilateral pneumonia with ARF. DNA of Pneumocystis jirovecii was found in bronchoalveolar lavage (BAL) fluid. Therapy with co-trimoxazole and micafungin had no effect. Repeated examination of the BAL fluid did not reveal the DNA of Pneumocystis jirovecii. An extended virological examination revealed OPV RNA in a nasal smear, and ribavirin was added to the therapy. During the following days, normalization of body temperature was noted for the first time, after 3 days there was a regression of ARF. Thus, ribavirin has been shown to be highly effective in cases of confirmed OPV infection.

Introduction. Monoclonal gammapathy is a heterogeneous group of diseases associated with the formation of a clone of plasma cells in the bone marrow and the secretion of paraprotein.

Aim: to present clinical observations demonstrating the effectiveness of therapy in patients with scleromyxedema associated with monoclonal gammapathy.

Main findings. Scleromyxedema is one of the dermatological manifestations of monoclonal gammapathy. It is characterized by thickening of the skin due to excessive formation and deposition of mucin in the dermis, involvement of internal organs in the process. Without therapy, this condition can cause complications from the gastrointestinal tract, respiratory, cardiovascular and nervous systems. An effective treatment method is clone-reducing therapy using proteasome inhibitors, antitumor immunomodulators, alkylating drugs, glucocorticoids, as well as subsequent melphalan chemotherapy, and autologous hematopoietic table cell transplantation. As a result of the treatment, regression of dermatological manifestations and complete reduction of the clone of plasma cells were achieved.

Introduction. Acute myeloid leukemia (AML) is characterized by a complex clonal structure formed as a result of clonal evolution. The study of clonal evolution is dictated by the need to improve the effectiveness of therapeutic strategies for AML.

Aim: to review the current understanding of the clonal evolution of AML and analyze clonal evolution in real clinical practice using the example of three clinical cases.

Main findings. A description of clinical cases with AML clonal evolution included 3 patients with newly diagnosed AML. Molecular genetic analysis of FLT3 and NPM1 genes was performed using commercial kits. The article reviews the main models of clonal evolution of AML: linear and parallel. The data from the study of leukemic stem cells are presented. Clonal evolution is demonstrated using the example of three clinical observations, explanations and recommendations for the selection of therapy are given. The clonal evolution of AML is a long and continuous process, which results in the formation of a complex branching pattern of molecular genetic breakdowns involving the leukemia stem cells. In this regard, at all stages of AML development (onset, remission, relapse), it is necessary to diagnose the maximum number of molecular genetic markers. Treatment taking into account knowledge of the patterns of clonal evolution is likely to improve the effectiveness of therapy.