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Aim. To evaluate the efficiency of the original ALL-MB-2002 protocol within the multicenter study of treatment of acute lymphoblastic leukemia (ALL) in children. Subjects and methods. A total of 1873 primary patients with ALL aged 1 to 18 years, of whom 1544 patients were enrolled in this study, were notified at 36 clinics of Russia and Belarus from April 15, 2002, to January 1, 2008. Results. With the median observation of 4.12 years, 7-year event-free survival (EFS) was 73±13%; overall survival (OS) 78±2%; relapse-free survival 82±1%. The rates of EFS and OS were equal and amounted to 76±2 and 80±2% in the standard-risk group (SRG) and intermediate-risk group (ImRG), respectively. In the high-risk group (HRG) patients, EFS and OS were as high as 30±6 and 37±6%, respectively. The frequency of relapses with central nervous system lesion was as much as 4.7% in all the patients, 6-year cumulative risk for isolated neurorecurrences being 2.5% in the SRG patients. Adolescents, patients with the baseline leukocytosis (more than 100109/l), and those with a splenic size of over 4 cm or more from the costal arch margin had substantially worse survival rates. A poor early response to therapy (on induction days 8 and 15) was also associated with its lower efficiency. Conclusion. Despite a considerable rise in the number of centers and a slight increase in the intensity of therapy, the results of the new ALL-MB-2002 protocol are as minimum equivalents obtained in the use of the previous ALL-MB-91 protocol. A significant improvement in the overall results of therapy and a reduction in the cumulative risk for isolated neurorecurrences were noted in the ImRG patients.

Проанализирована значимость прогностических факторов эффективности терапии у детей с острым лимфобластным лейкозом (ОЛЛ), получающих терапию по протоколу ALL-MB-2002, в зависимости от группы риска. В исследование были включены 1544 первичных больных ОЛЛ в возрасте от 1 года до 18 лет, получавших лечение в 36 клиниках России и Беларуси в период с 15 апреля 2002 г. до 1 января 2008 г. Из 1544 больных, включенных в анализ, пациенты группы стандартного риска (SRG) составили 69,2 %, промежуточного риска (ImRG) — 24,5 % и 6,3 % пациентов были отнесены к группе высокого риска. Анализ, проведенный у пациентов SRG, продемонстрировал резкие различия в выживаемости между отдельными подгруппами больных. Выявлены достоверные различия в показателях выживаемости пациентов в зависимости от возраста (старше и младше 10 лет), инициального лейкоцитоза (более и менее 30 × 10 9 /л), инициального пальпаторного увеличения селезенки (более и менее 4 см из-под края реберной дуги) и параметров раннего ответа на терапию (8-й и 15-й день). При ретроспективном определении больных, относящихся к SRG, с использованием новых критериев (инициальный лейкоцитоз

The paper presents the results of a multicenter randomized study of two-dose COLI-ASP regimens in therapy of consolidation of standard risk-group (SRG) children and adolescents with acute lymphoblastic leukemia (ALL) treated by the ALL-MB-2002 protocol. The principal aim of the study was to elucidate whether the dose of COLI-ASP might be reduced without losing the efficiency of therapy. The analysis covered primary ALL patients notified at 36 clinics of Russia and Belarus, enrolled for the study in the period of May 1, 2002, to October 10, 2006. Out of 819 patients to be examined, 414 received COLI-ASP in a dose of 10,000 U/m2 (a control group), and 405 children took it in a dose of 5,000 U/m2 (a study group). No statistically significant differences were found between the patients in gender, age, baseline leukocytosis, and spleen size. The study showed no statistically and clinically significant differences in event-free, overall, and relapse-free survival (EFS, OS, and RFS, respectively) and relapse rates with the use of two-dose COLI-ASP regimens. However, in the control group, the death rate in remission was twice greater than that in the study group (p = 0.02). Detailed analysis of the outcomes of therapy in some subgroups revealed no differences between the control and study groups. The Cox multiple regression analysis has indicated that the factors influencing EFS and RFS are the baseline sizes of the spleen, baseline leukocytosis, age, and a response to treatment in the period of induction, rather than the dose of E. coli asparaginase. The authors have also found that among the SRG patients, there is a subgroup of patients without risk factors, in whom the reduction in the COLI-ASP dose may substantially increase survival rates, by lowering toxicity and mortality in remission.

Purpose of the study. To analyze the physical and neuropsychiatric development of pediatric patients who underwent cranial irradiation in the period from 2015 to 2020 in the radiotherapy department of the National Research Center of Oncology and to assess the risk of post-radiation complications.Materials and methods. 17 children aged from 3 to 17 years were hospitalized under medical supervision in the department of pediatric oncology of the National Medical Research Centre for Oncology. All the children underwent a course of conformal radiation therapy totally on the brain area and the first two cervical vertebrae in the radiotherapy department of the National Medical Research Centre for Oncology. 13 patients (76.7 %) underwent radiation therapy due to the prevention of neuroleukemia with a total dose of 12 Gy (a dose per fraction was 2 Gy), 2 patients with a confirmed relapse of acute lymphoblastic leukaemia (ALL) (11.65 %), 1 patient with a confirmed diagnosis of neuroleukemia (5.8 %) and 1 patient from the high-risk group (5.8 %) – with a total dose of 18 Gy (a dose per fraction was 2 Gy). Further 75 month regular medical checkup was carried out on the basis of the Regional Children's Clinical Hospital for.Results. None of the surviving patients showed growth retardation. Two patients (11.65 %) complained of increased fatigue, decreased concentration; one patient (5.8 %) showed unmotivated irritability and aggression during the examination. Intellectual development corresponded to age in all patients (100 %). One patient (5.8 %) experienced episodes of nausea and vomiting (grade 1 on the CTCAE scale), three patients (17.7 %) suffered from headache (grade 2 on the CTCAE scale), three patients (17.7 %) complained of fever up to 38 °C (1 degree on the CTCAE scale). Two out of 17 ALL patients died due to disease progression.Conclusion. Taking into account the different time intervals between treatment and the moment of the study (from 9 to 75 months), cranial irradiation demonstrates relative safety for patients undergoing treatment during critical periods of development of both physical and neuropsychic spheres. However, an objective assessment of the development prospects is difficult due to the relatively short time after undergoing therapy (from 9 to 75 months) and a small sample of patients.

Mucormycosis of the lungs is a severe infectious complication in patients with acute lymphoblastic leukemia, which develops at the stage of high-dose cytostatic therapy. It is characterized by an extremely aggressive, rapidly progressive course and, without specific treatment, is fatal in a short time. Reliable verification of mucor is necessary due to its resistance to the most commonly used antifungal drugs, particularly to voriconazole.The article presents a clinical case of pulmonary mucormycosis in a 12‑year-old child at the stage of diagnosis of acute lymphoblastic leukemia. The first symptoms of the disease (headaches, malaise and weakness, pallor), changes in the general blood count (hyperleukocytosis up to 200 thousand cells/μl, single platelets). Based on the results of the examination, the main diagnosis was verified for acute lymphoblastic leukemia L2, IFT T-II, CD1a-. At the stage of diagnosis of acute lymphoblastic leukemia, the underlying disease was complicated by the development of right-sided pneumonia according to X-ray examination. To verify the etiology of infiltration of lung tissue, broncho-alveolar lavage was directed to microbiological diagnostics, which included studies: enzyme immunoassay, microscopic and cultural. On the aggregate of all the results obtained, invasive mucormycosis was diagnosed and antifungal therapy was started immediately.

Purpose: To create the algorithm of hepatitis B vaccinal prevention at children with acute lymphoblastic leukemia. Materials and Methods: The data of 181 children with acute lymphoblastic leukemia, from which 99 patients received specific hepatitis B prevention with vaccine «Engerix B», have been investigated. The authors have assessed correlations between efficiency of offered vaccinal prevention and leuco/neutropenia degrees at patients in days of vaccinations. The analysis of hepatitis B infect level at stages of vaccination was carried out. Results: The algorithm of hepatitis B vaccinal prevention at children with acute lymphoblastic leukemia is offered.

The development of progressive myocardial dysfunction is one of the side effects of polychemotherapy. Changes in diastolic filling of the left ventricular is the earliest marker that precedes the full-scaled clinical picture of heart failure. Purpose: Assessment of left ventricle’s diastolic function and the study of the state of lipid peroxidation processes in children with acute leukemia at the stages of polychemotherapy. Materials and Methods: We have examined 54 patients with acute lymphoblastic leukemia at the age ranging from 3 to 17 years old. Results: Complex transthoracic Doppler echocardiography shows the occurrence of diastolic dysfunction at the time when the disease is diagnosed and its progression after the end of polychemotherapy. The development of cardiac complications is accompanied by an increase in the generation of reactive oxygen species, accumulation of oxydated lipoproteins, which suggests the free-radical genesis of myocardial damage. Summary: Early detection of disorder of diastolic function of the left ventricle may allow to appoint cardiotropic therapy on the initial stage and decrease the rate of complications of polychemotherapy.

This paper presents the results of work of Children’s center of oncology and hematology during 18 years. There are presented results of event free survival of patients, treated in accordance with the modern high-technological protocols of the chemotherapy and using the alternative methods of the treatment. We also present our experience of the psychosocial service organization and the rehabilitation of children with the oncology diseases.

The main link in the pathogenesis of ITP is an excessive elimination of platelets in venous sinuses of the spleen. It justifies the splenectomy in this pathology, which increases the immunosuppression, which is caused by earlier courses prednizolonotherapy. A violation of proteinsynthetical detoxifying and liver function plays an essential link in the pathogenesis of postsplenectomic syndrome. It exacerbates immunosuppression. The composition allows saving all the structural elements of viable lymphoid tissue in the spleen for 7 days. Delayed autotransplantation of preserved splenic tissue gives the body a full lymphoid tissue without signs of hyperplasia. Two-moment autotransplantation of preserved splenic tissue in ITP allows to cut short the phenomenon postsplenectomic immunosuppression with normalization of platelet counts in peripheral blood and to avoid disability. The developed medical complex, including splenectomy with followed by a delayed autotransplantation of splenic tissue is technically simple, requires nonexpensive equipment and can be applied in practical public health services in the treatment of children’s ITP