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Relevance. Despite the overall decline in the use of auto-HSCT, there remains a need for treatment intensification in pediatric patients with highrisk solid tumors. Tandem auto-HSCT represents a promising approach to improve therapeutic efficacy, but its toxicity and impact on long-term outcomes require further investigation, particularly in the Russian patient population.The aim of study – to evaluate the feasibility, toxicity, and long-term outcomes of tandem autologous hematopoietic stem cell transplantation (auto-HSCT) in children with high-risk solid malignant neoplasms in the Russian Federation.Materials and methods. This retrospective multicenter study included 108 (100 %) pediatric patients (median age – 2 years) with solid malignant neoplasms who underwent tandem high-dose chemotherapy with auto-HSCT at 9 centers in the Russian Federation (2004–2024). Main diagnoses included brain tumors – n = 66 (61.1 %), germ cell tumors – n = 25 (23 %), and neuroblastoma – n = 12 (11.1 %). Toxicity was assessed using CTCAE v5.0 criteria, and survival analysis was performed using the Kaplan–Meyer method.Results. The most common complications were febrile neutropenia (88.9 %) and oropharyngeal mucositis (87.1 %), with severe toxicities (Grade 3–4) being rare. The 3-year overall survival was 72.2 %, and event-free survival was 62.7 %.Conclusion. The study demonstrated that tandem auto-HSCT is a feasible procedure with manageable toxicity. These findings suggest the potential of tandem auto-HSCT for children with high-risk solid tumors, though further prospective studies with long-term outcome assessments are needed to define optimal indications.

The article provides useful and necessary information about port systems for patients and their parents.

Retinoblastoma (RB) is the most common malignant tumor of the eye found in pediatric practice. Currently, in developed countries, the overall survival rate is approaching 100 % with a high rate of eye preservation up to 90 %, but such a treatment method as enucleation remains relevant today. The study of prognostic morphological factors in eyes removed after enucleation has been the subject of study for several decades in order to optimize adjuvant therapy. On the one hand, to intensify treatment to prevent relapse of RB, on the other hand, to refuse chemotherapy to avoid “overtreatment” of the patient.Our article presents an analysis of the experience of different countries in determining morphological risk factors that influence the appointment of adjuvant chemotherapy after primary enucleation.

Neuroblastoma (NB) is a malignant embryonic tumor arising from undifferentiated neuroectodermal cells of the neural crest, often exhibiting highly aggressive behavior. Secondary acute myeloid leukemia (AML) following NB is a rare and complex therapy-related complication characterized by chemoresistance and a poor prognosis.This article presents a clinical case of successful personalized consolidation therapy with venetoclax in combination with 5-azacytidine in a patient with AML after NB treatment. Despite the primary refractory course of AML and the failure of standard therapeutic regimens, the use of allogeneic hematopoietic stem cell transplantation from a haploidentical donor, combined with consolidation therapy in the post-transplant period, enabled the achievement of sustained remission of both malignancies.The article also discusses the challenges of treating secondary AML, including chemoresistance and the high risk of relapse. Consolidation therapy based on venetoclax and hypomethylating agents represents a promising approach to improving outcomes in patients with secondary AML after NB, offering the potential for long-term remission with a favorable safety profile.

Neurofibromatosis type 1 (NF1) is a genetically determined multisystem rare disease with a neonatal morbidity of around 1/300–1/4000. The most typical feature for NF1 is a predisposition to the development of tumors that are variable in histological types and age of occurrence. One of the most common variants of benign neoplasms in NF1 are plexiform neurofibromas (PN) with typical manifestation and maximum growth rates in childhood. The features of PN are the diffuse and multifocal growth, high vascularization, which in most cases limits the possibilities of surgical treatment. Despite the relatively slow growth rate, PN can reach large, and in some cases huge sizes, leading to severe disability of patients, quality of life impairment, and often life threatening. Another feature of PN is the risk of transformation into a malignant peripheral nerve sheaths tumor, which requires delicate lifelong monitoring of patients at risk.Today, the use of the selective MEK inhibitor selumetinib is a key method of conservative therapy for patients with symptomatic NF1-associated PN, with demonstrated high efficacy and safety in international clinical trials. Taking into account all features of NF1, as well as range of associated diseases, it is obvious the necessity for monitoring and decision-making by multidisciplinary team of specialists. Many questions in the aspects of continuity of medical care with the involvement of federal centers, compliance with practical recommendations, diagnostic/ treatment algorithms and transfer from pediatric into the adult-focused primary care system remain unclear, which will be discussed in this article

Introduction. Pathogen reduction technologies (PRT) in donor blood components have become a new milestone in ensuring the safety of transfusions. However, it is widely acknowledged that the use of PRT can compromise the quality of blood components and potentially diminish the efficiency of transfusion.The purpose of this retrospective analysis is the presentation of the results of the use of pathogenic-reduced components of donor blood in comparison with standard transfusion practice in hematopoietic stem cell transplantation (HSCT) recipients.Materials and methods. An analysis was conducted of the results of transfusions to recipients of allogeneic HSCT performed in 2018–2022. We presented results of 1901 red blood cell transfusions (1848 of which were gamma-irradiated and 53 were pathogen-reduced), 8192 platelet concentrates transfusions (7654 of which were gamma-irradiated and 538 were pathogen-reduced) and donor plasma transfusions (freshly frozen plasma (FFP) – 1381, pathogen-reduced – 169).Results. The estimated laboratory efficiency of the transfusion of pathogen-reduced red blood cells was comparable to that of gamma-irradiated. Platelet concentrates subjected to pathogen reduction, as well as those prepared using an additional solution, demonstrated the lowest results of post-transfusion increase compared to gamma-irradiated platelet concentrates. Plasma processed using the pathogen reduction method resulted in a smaller increase in fibrinogen concentration. All other hemostasis indices were comparable to those of FFP.Conclusion. The clinical use of pathogen-reduced donor blood components is an effective transfusion practice, but further improvements in TRP are needed.

Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm with uncontrolled production of megakaryocytes. It is characterized by the presence of large and giant megakaryocytes in the bone marrow, which leads to an increase in platelet count. This condition can cause both thrombosis and bleeding. In children, the clinical presentation of ET can vary. Unlike adults, who often experience hemorrhagic and thrombotic events, most pediatric patients do not have any symptoms. Instead, they may only have changes in their blood count. The diagnostic criteria and risk factors for ET in adults are not directly applicable to children. Similarly, the treatment recommendations for adults with ET cannot be directly applied to children. The genetic profile of ET in children also differs from that in adults, leading to differences in the frequency of specific driver mutations and the number of cases of the disease.The aim of this study is to describe the clinical and laboratory features, course, and treatment of ET in children and adolescents.

16 июня 2025 г. в Москве состоялся Совет экспертов, посвященный применению препарата эфлорнитин (DFMO, IWILFIN) в терапии детей с нейробластомой (НБ) группы высокого риска.Целями Совета явились обсуждение консолидации международного и российского опыта применения DFMO у пациентов с НБ группы высокого риска, а также обсуждение показаний и рекомендуемых сроков начала DFMO.

Introduction. Galectins are glycan-binding proteins containing one or two carbohydrate domains and performing many biological functions. It has been established that galectins synthesized by tumor tissue have the ability to autostimulate and paracrine stimulate cells of the microenvironment. Literature data indicate that changes in galectin production and secretion are observed in many types of malignant neoplasms and often contribute to their rapid progression. Interestingly, there is increasing evidence that a family of β-galactoside-binding lectins, known as galectins, plays a key role in the adhesion of circulating tumor cells to the vascular endothelium, contributing to the dissemination of the tumor process. For a number of solid tumors, the role of some galectins, especially galectin-3, has been studied and described fairly well, while in tumors of the bone system, the clinical and laboratory significance of proteins of this family has not yet been determined.Purpose of the study – comparative analysis of the content of galectins-1, -3, -4, -7, -9 in the blood serum of bone tumors and healthy donors in pediatric oncology practice, connection with the main clinical and morphological characteristics of the disease and determination of their diagnostic potential.Materials and methods. The retrospective study included 95 patients with bone tumors: malignant (n = 66), benign (n = 25) and borderline (n = 4), who were treated at the N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia. The clinical and radiological diagnosis of the tumor was confirmed by the data of a morphological study of the tumor according to the International Histological Classification of Bone Tumors (World Health Organization, 2020). The concentration of galectins-1, -3, -4, -7, -9 was determined in blood serum obtained according to standard methods before the start of specific treatment, using reagent kits for enzyme-linked immunosorbent assays. The obtained data were processed using the GraphPad Prizm 10.0 program. When comparing indicators and analyzing their relationships, nonparametric Mann–Whitney and Kruskal–Wallis tests were used. Analysis of the information content of the diagnostic method by assessing its sensitivity and specificity was carried out by constructing ROC curves and calculating the area under them (AUC). Correlation analysis was carried out by determining the Spearman correlation coefficient. Differences and correlations were considered statistically significant at p < 0.05.Results. It was found that the median galectin-3 content in the control group was 6.69 (4.15–9.94) ng/ml, which is significantly lower than in patients with bone tumors – 7.85 (5.85–11.75) ng/ml (p = 0.033). Significant changes in content were detected for galectin-9. In patients with bone tumors, the median concentration of galectin-9 is 5.35 (4.28–6.89) ng/ml, which is statistically significantly lower compared to the control group – 7.37 (6.09–8.870) ng/ml (p = 0.001). The ROC-analysis showed that despite significant changes in the content of galectin-3 and galectin-9 in the blood serum of patients with bone tumors, the study of their concentrations does not allow using the data obtained in the clinic for diagnostic purposes. The analysis showed that for galectin-1 and galectin-7 there is no change in their concentration during the development of benign and malignant bone tumors. For galectin-3, a significant increase in its content was shown in the case of the development of malignant bone tumors, while for galectin-4 similar patterns were noted in the development of benign bone tumors. The content of galectin-9 was significantly reduced in patients with both benign and malignant tumors compared to controls. Correlation analysis showed that in the control group the content of galectin-9 inversely correlates with the level of galectin-1 and galectin-7 (r = –0.533; p = 0.019 and r = –0.473; p = 0.041, respectively). In the group of patients with bone tumors the content of galectin-1 directly correlates with the level of galectin-3 (r = 0.360; p = 0.004), and the level of galectin-7 with galectin-9 (r = 0.420; p = 0.001), which indicates a change balance of the content of these proteins during the development of tumor pathology of skeletal bones.Conclusion. Our work revealed changes in the balance of galectins-1 , -3, -4, -7, -9 in the blood serum of children with tumors of the musculoskeletal system. Despite the ambiguous results, galectins, due to their widespread occurrence in the human body, remain promising molecules for use in clinical, laboratory and scientific practice as biological markers. However, further studies are needed to confirm their clinical significance.