The results of the treatment of acute myeloid leukemia (AML) in pediatric patients remain poor. The modern programs of treatment allow achieving long-term survival in only 6570% of patients and the event-free survival rates are lower and make up approximately 55%. The development of protocols for high-intensity therapy, which are based on the block principle, followed by long-term maintenance therapy has led to the significant progress in the treatment of AML. As supportive care has improved, it became possible to escalate the doses of chemotherapy, which contributed to the increase of the treatment effectiveness. So far, doses and drugs administration regimens have approached the tolerated level, which necessitates the research into new therapeutic targets. An investifation of the epigenetic processes underlying leukemogenesis made it possible to identify those targets DNA methylation and histone deacetylation. This review presents the results of the use of epigenetic drugs in the treatment of AML in children. The possibilities of including epigenetic agents in standard polychemotherapy protocols are presented.

The recent chemotherapeutic approaches to acute myeloid leukemia (AML) management reached the limits, achieving overall survival rate of approximately 70 %. An intensification of chemotherapeutic regimens is barely possible due to high level of toxicity and risk of lifethreatening complications. The modernization of program therapy of AML involves the clinical application of achievements in molecular biology, immunology and cytogenetic of the tumor cell. The researches in fundamental oncology revealed the phases of leukemogenesis and defined selective ways of targeted therapy in the treatment of AML.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare hematologic malignancy. Our view of the cellular origins of this kind of tumor has been changing dramatically with the emergence of new data on the molecular biological and immunological characteristics of the tumor. This article discusses the clinical features of BPDCN, as well as the cytological, morphological-immunological and molecular genetic criteria for BPDCN diagnosis. Taking into account the rare incidence of BPDCN, as well as its rather complex diagnostic procedure, which requires an extended diagnostic antibody panel, standard methods of therapy have not been developed. Chemotherapy protocols for acute lymphoblastic leukemia and acute myeloid leukemia are used, with/without subsequent autologous/allogeneic bone marrow transplantation, but the results remain unsatisfactory. For the first time in Russian cancer research, this article provides a description of BPDCN in a 14-year-old child. A detailed clinical analysis of this rare tumor is provided, as well as dermatoscopy results and a description of the histological, immunological and molecular features of BPDCN, from the point of view of differential diagnosis. Parents patients agreed to use personal data in research and publications.