EV/1-Positive Leukemias and Myelodysplastic Syndromes: Theoretical and Clinical Aspects (Literature Review)

Abstract

The present review provides the analysis of theoretical background and therapy of prognostically poorest EV/1-positive myeloid leukemias and myelodysplastic syndromes which is performed at the RM Gorbacheva Scientific Research Institute of Pediatric Oncology, Hematology and Transplantation. The focus is on the evidence of the dominating role of EV/1 gene in impaired epigenetic regulation of hematopoiesis and, thus, on the feasibility of allogeneic hematopoietic stem cell transplantation with hypomethylating agents and/ or trans-retinoic acid used for these diseases treatment.

Evaluation of BAALC- and WT1-expressing leukemic cell precursors in pediatric and adult patients with EVI1-positive AML by means of quantitative real-time polymerase chain reaction (RT-qPCR)

“…Meanwhile, EVI1-positive AML with presumable rearrangements of KMT2A gene was found in one-fourth of pediatric patients [8][9][10], thus also presenting essential age-dependent differences in responses to therapy and HSCT. At the same time, an excellent response to retinoid treatment has been recently revealed in all types of EVI1-positive AML [11], which seems to be associated with their direct action upon functional activity of stem cells [12].…”