The purpose of the study is to assess the frequency of occurrence of the most frequent cytogenetic rearrangements in neuroblastoma (NB) and their correlation with clinical and biological characteristics and outcome of therapy in the Republic of Belarus. Materials and methods. 280 patients with primary diagnosed NB from 10.10.2007 to 01.04.2018 were studied. The material for the study was the primary tumor or tumor metastases in the bone marrow (damage more than 60 %) according to the criteria of the protocol. The study was conducted by the method of fluorescent hybridization in situ. Results. The frequency of occurrence of such cytogenetic aberrations as MYCN amplification (20.1 %), 11q deletion/imbalance (19.7 %) and 1p aberration (28%) in the studied population corresponds to literature data. The frequency of occurrence of gain17q (34.5 %) was lower than the literature data. The negative effect on event-free survival (EFS) of patients with NB was statistically confirmed and the presence of such genetic aberrations in the tumor, such as MYCN amplification, 11q deletion/imbalance, 1p aberration. Statistically reliably confirmed higher rates of event-free survival for patients with near-triloid set of chromosomes in the tumor, as compared with near-/tetroploid (97 ± 3 % versus 63 ± 5 %). For patients who lacked structural aberrations in the tumor, EFS was 75 ± 4 % versus 51 ± 9 % in the group with aberrations (p < 0.0001).

The purpose of the study was to create a certification of the service of pediatric hematology-oncology of the constituent entities of the Russian Federation (RF) using infographics and digitalization. Materials and methods. For the first time, infographic maps were used as a tool for certification, as a way of visualizing a large amount of data on the organization of pediatric hematology-oncology service in each subject of the RF. They allowed to show the specifics of the medical and organizational structure of the profile service in the subjects of the RF, their interaction with other subjects and federal centers. Processing of the cartographic image of the subject of the RF was carried out in the Adobe Illustrator graphic editor. A modifiable tool for creating infographic maps created in the MS Power Point program, with saving the results in PDF and PNG formats. Results. In the course of the study, a “Passport of Service” was prepared, containing infographic maps of 77 constituent entities of the RF. The maps in a graphic form provide information on resources available in the subject of the RF: a medical organization of the 3rd level with bed structure and the possibility of visualization, its personnel; the number of multicenter protocols and clinical guidelines used in the subject of the RF; logistics of patients, taking into account the territorial distance from the specialized beds, the presence in the region of the station/department of blood transfusion; the possibility of radiotherapy in the region; the presence of institutions where they provide palliative and rehabilitation assistance; the name of the federal and regional specialized centers, with whom the interaction is carried out, the well-established telemedicine consultation system with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The database of contacts of the main freelance children’s specialists of hematologists and oncologists of the RF was also updated. Conclusion.Thus, data were collected for visual display and to create specific conditions for developing optimal patient routing schemes and forming proposals for optimizing the service. The obtained “Passport of the Children’s Hematology-Oncology Service of the Russian Federation” can be used in the work of hospitals, scientific centers and professional communities.

Introduction. Immune thrombocytopenia (ITP) is a benign hematological disease characterized by an isolated decrease in platelet count, with different course options requiring both an urgent therapeutic decision and clinical observation of patients. At the present stage in the treatment of children with immune thrombocytopenia, especially with the chronic form, significant success has been achieved. Therapeutic options are determined by an individual approach to the patient and are based on the experience of a hematologist. Materials and methods. For the period from 01.01.2013 to 12.31.2017, in the Oncohematological Department of Chemotherapy of the Voronezh Regional Children’s Clinical Hospital № 1, 153 children with various forms of ITP, aged between 1 and 6 months to 17 years, received treatment. Diagnosis and stage of the disease were carried out on the basis of modern clinical guidelines and ITP classification. The analysis of the severity of hemorrhagic syndrome in the diagnosis of ITP was carried out. The principles of patient therapy were based on the pathogenetic aspects of ITP, the stage of the disease. Patients with newly diagnosed ITP (n = 106; 69.4 %), persistent form (n = 26; 16.9 %) received different lines of therapy, provided for by clinical guidelines, some patients were only under the supervision of a hematologist. The chronic form of the course of the disease was formed in 21 (14 %) children out of 153, regardless of the type of treatment. Children with the chronic form received various lines of therapy, including thrombopoietin agonists. The decision to manage such patients was based on a joint discussion with specialists from the largest federal centers. Splenectomy for the above period was performed for 1 child with a pronounced and chronic “wet” component of the disease. Discussion. Treatment of ITP in children, despite the emergence of a new class of drugs with a targeted effect, in some cases is a difficult task for a hematologist. The adoption of a specific therapeutic decision is based on the severity of the clinical and hematological data of the patient, the search for a possible cause of the thrombocytopenic condition, as well as the individual experience of the doctor. Prevention and prevention of bleeding in patients with different ITP, in particular with chronic ITP, with the absence or minimal manifestation of hemorrhagic syndrome according to the principle “0 bleeding per year” is the ultimate therapeutic task. Conclusion. ITP in children, in particular its chronic form, belongs to orphan diseases, therefore, the experience of managing such patients may be useful in determining the overall tactics of treating patients.

Introduction. Therapy of malignant tumors is one of the most important problems of modern pediatric oncology. The presence of distant metastases is considered as the main sign of generalization of the tumor process. In order to improve the results of treatment of these patients, we proposed to supplement cytoreductive surgery with intraoperative isolated chemoperfusion of the lung and/or pleura with cytostatic. The purpose of the study is to determine the effectiveness of metastasectomy combined with isolated lung/pleura chemoperfusion (ILCP/PCP) under conditions of hyperoxia in children. Materials and methods. For 10 years of research (2008–2018) on the basis of the N.N. Petrov National Medical Research Center of Oncology produced 45 ILCP and PCP in 30 patients with intrapulmonary and intrapleural metastases of various malignant bone tumors. The ILCP method is a surgical procedure, during which the lung is temporarily switched off from the circulation and perfused with modified saline containing high concentrations of an antitumor drug (melphalan or cisplatin), which allows delivery of high doses of cytostatics while avoiding systemic exposure to the drug. In cases of detection of metastatic lesions of the parietal pleura after metastasectomy, PCP was performed, consisting in drainage of the pleural cavity and intraoperatively conducting, after suturing the surgical wound, hyperthermic (42 o С) chemoperfusion of the pleural cavity with a cisplatin solution for 2 hours. Results. In children with malignant bone tumors, 37 isolated chemoperfusions with cisplatin and 8 with melphalan were performed. In 27 patients, 36 ILCP were performed, in 7 patients – 9 PCP. Nine (20 %) patients underwent bilateral perfusion with an interval from 1.5 to 31 months. There were no lethal outcomes during operations and in the postoperative period. The average duration of surgical interventions was 270 ± 90 (120–520) minutes. The number of remote foci – from 1 to 56 (average value – 9). The average blood loss was 300 ± 200 (150–1000) ml. The mean follow-up was 45 months; median overall survival – 38 months; 3-year overall survival – 65.5 ± 9.4 %. Survival without progression – 40.5 ± 10.5 % (n = 30). Conclusions. ILCP/PCP with cytostatics is a method of complex therapy that can improve the quality and increase the life expectancy of patients, especially with the exhausted possibility of other treatment options.

Chemotherapy (CT) has significantly increased the chance of cure for patients with resectable osteogenic sarcoma and is now an integral part of their treatment. At the same time, the standard approach is represented by administration of the same СT (doxorubicin, cisplatin, methotrexate) to all patients both before and after the surgery has remained unchanged over the past decades. Unfortunately, the results of its application in patients with a poor pathologic response of a tumor to preoperative CT are not satisfactory, and recently published studies have not shown their improvement due to the intensification of postoperative CT. As a result, the standard of treatment for these patients remains a continuation after surgery the same CT, which has proved ineffective at the preoperative stage. The article is devoted to a critical analysis of the feasibility of such practices and an assessment of the possible positive and negative consequences of the rejection of postoperative CT in patients with poor pathologic response.

The review article provides information about metastases of solid tumors in the lungs: from pathogenesis and the first experience of metastasectomy to a combined local method of therapeutic treatment. According to the literature, lung metastasectomy is part of the standard treatment for adults, but information about the surgical treatment of metastatic lesions in children remains poorly understood. As a rule, metastatic lesion accompanies the progression of the tumor process. Since the 1960s, methods of isolated lung perfusion have been developed, the results of which are reflected in a few articles, but most of the studies are aimed at exploring the possibilities of use and at the direct results of the implementation of this technique.

Adenovirus infection is one of the causes of transplant-associated mortality after allogeneic hematopoietic stem cell transplantation. Fulminant liver failure with adenovirus infection is a diagnostic and therapeutic problem due to its aggressive clinical course and extremely poor prognosis, while modern methods of treating adenovirus infection are based on the use of virostatic drugs and virus-specific lymphocytes and are associated with severe side effects. The article presents a rare clinical case of elimination of disseminated adenoviral infection with fulminantly developing liver failure in a patient after allogeneic bone marrow transplantation from a haploidentical donor.

Introduction. Today, the problem of nutritional deficiency (ND) in children undergoing conditioning therapy and after hematopoietic stem cell transplantation (HSCT) remains relevant. The main goal for success is the interaction of doctors of various specialties, nurses and clinical psychologists. In the treatment process, a nurse occupies one of the key places; the process of passing nutritional support and returning to normal nutritional status depend on her knowledge, qualifications and skills. Materials and methods. Thirty-eight immunocompromised patients were included in the study (patients of the HSCT unit who received conditioning therapy). According to nutritional status: in 18 patients, ND was noted on day –1 before HSCT, in 10 patients, ND was noted on + 5–7th days after HSCT, and in 10 children, normal nutritional status remained. In terms of the incidence of adverse complications of chemotherapy (CT): nausea, vomiting, mucositis, and diarrhea were noted mainly in the age group up to 3 years; the presence of pain is more pronounced in children from 3 to 10 years; A change in the taste of the food intake was more present in the age group from 10 to 18 years. Results. The majority of patients (75 %) who received conditioning therapy and after HSCT had ND. Between the day +1 and + 7th day, mixed nutritional support was assigned after the HSCT. With the development of complications after chemotherapy, recommendations and measures were developed to reduce the effects of the treatment. Findings. Due to screening for ND and a multidisciplinary approach, nutritional support is a necessary component when conducting various types of antitumor treatment, which in turn improves the results of treatment and the quality of life of patients. On the part of the nursing staff, recommendations on the management of patients with ND and direct training of their legal representatives were actively applied.

This article is devoted to the analysis of problems related to the state of the nervous system in patients who have survived malignant neoplasm in childhood. The main diseases and pathological conditions that can develop in this population are considered. The risks of the development of pathological changes in the nervous system, treatment and prevention are described. Separately analyzed issues of peripheral neuropathy, Raynaud’s syndrome, chronic pain.