FROM EDITION
SELECTED LECTURES
Оригинальные исследования
MLL-negative (mixed lineage leukemia – MLL) acute lymphoblastic leukemia (ALL) diagnosed in the first year of life is rare. Although infant ALL is often assumed to be fatal, no studies have been published on outcome of MLL-negative infant ALL. The present study reports the clinical characteristics and outcome of 60 patients with MLL-negative infant ALL treated with the series of Moscow-Berlin (MB) protocols, a regimen with reduced chemotherapy. MLL-negative ALL by infants with age < 6 months at the diagnosis was characterized by a higher white blood cell count, splenomegaly and prevalence of girls compared with older infants. Induction failure rate was 3.4 % for the whole group of patients and was not significantly different between infants with less or more 6 month old. EFS and OS for the whole group of patients were 62 ± 7 % and 59 ± 7 % with relapse rate of 28.7 %. But relapse rate was significantly higher in MLL-negative infant ALL patients with age < 6 month compared of that of pts with age 6 month (cumulative incidence was 60.7 ± 16.5 % vs 19.1 ± 6.7 %, p < 0.005). EFS and OS of MLL-negative infant ALL patients were significantly worse compared of that for the old infants (10 ± 9% vs 79 ± 7 % for EFS). The treatment according new MB regimens significantly improve the outcomes for the whole group and also for children with < 6 months age at the diagnosis.
Article represents analysis of clinical and biological characteristics and results of treatment of children of first year of life with acute myeloid leukemia. Retrospective analysis included 18 patients under the first year of life received treatment in Republican research center of pediatric oncology, hematology and immunology (Minsk, Belarus Republic). Probability of long-term overall survival was 33 ± 11 %; event-free survival – 28 ± 11 %; cumulative frequency of deaths related to treatment – 66.7 ± 11.7 %; cumulative frequency of relapse – 5.6 ± 5.6 %. Conclusions on age-related correction of cytarabine required.
Rationale. Executive functions (EF) can be considered as interrelated group of cognitive skills responsible for target behavior, including both higher and lower functions. Higher functions include inhibition, switching, cognitive flexibility, planning, working memory and solving of problems. Lower level of EF is a speed of processing and cognitive effectiveness/fluency which serve EF. Decreasing of EF and slowing of processing at patients received complex therapy in case of tumors of posterior cranial fossa lead to decreasing of quality of life and social functioning. It is supposed that disorder of EF and processing are connected to inaccuracies of saccadic system. Materials and methods. Five patients at age 10–14 years old with tumors of posterior cranial fossa were enrolled to this pilot study. These patients received rehabilitation and complex of hardware correction with the help of Dynavision D2 and Neurotracker equipment aimed to modify EF and processing. Two diagnostic sessions realized by itracking method and CANTAB battery to diagnose condition of saccadic system before and after rehabilitation process. Results. First experience of usage of a complex of hardware methods for EF correction showed improvement of function of saccadic system despite of different disturbances of visual analyzer which were diagnosed at all children. These results indicate the promising ways in solving of this actual problem.
Обзоры литературы
The article presents a review of the literature on the state of the problem of diagnosis and treatment of congenital central nervous system (CNS) tumors in children. Oncological alertness and awareness of specialists supervising pregnant women, parturient women, and young children, diagnostic features and the level of technological assistance to children with congenital CNS tumors determine the survival and prognosis of the disease. Modern methods of diagnosis and treatment make it possible to achieve satisfactory results in this category of patients. In the management team of patients and families, it is necessary to involve a palliative specialist, especially in cases of detection of noncurable tumors. Own clinical observations of patients with congenital CNS tumors are presented.
Primary immune deficiencies (PID) are the heterogeneous group of genetically associated disorders developed due to defects of more than 300 different genes. Modern classification of PID based on main pathogenic mechanism of disease, but to a large extent relies on presence of any genetic defect. Thus, role of molecular and genetic methods of investigation of patients comes to the fore and often determines approaches to a target conservative or curative therapy of PIDs. Despite of successful application of new therapeutic approaches, therapy with the help of intravenous immune globulins remains actual for majority of patients with PID.
CLINICAL CASES
Chronic myeloid leukemia (CML) is a rare disease in childhood. Long-term use of tyrosine kinase inhibitors has become the standard for the treatment of the chronic phase of CML. The article describes the case of development of CML in a child at the age of two years and raised the issue of the need for lifelong drug intake and the problems that arise in connection with this.
Clinical recommendations
QUESTIONS OF PRACTICAL HEMATOLOGY/ONCOLOGY
Article is dedicated on actual section of modern rehabilitology – organization of rehabilitation of patients of first year of life with oncohematological and immunological diseases. Aim of this study was to analyze the work of innovative direction of rehabilitation/abilitation in department of treatment of children of first year of life in Clinical Rehabilitation Research Center “Russkoe pole” of Dmitry Rogachev Center (Rehabilitation Center). Results of analysis confirm the dominance of embryonal tumors in the structure of diseases among 335 patients admitted to department in 2016. Medical help for patients performing by multidisciplinary team composed of employees of Rehabilitation Center and consultative department of Dmitry Rogachev Center. Volume of involvement of each participant of multidisciplinary team to rehabilitation process is determined by the spectrum of clinical problems of a patient. Results allowed authors to conclude that future integration of programs of recover medicine in practice of pediatric hematologists/oncologists provides both improvement of patients’ life parameters and decreasing of “population load” due to increased number of children and adolescents cured of malignant neoplasms.
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ISSN 2413-5496 (Online)