CHRONIC MYELOID LEUKEMIA IN CHILDREN OF THE SAKHA REPUBLIC (YAKUTIA)

Abstract

This article analyzes the course, treatment and outcomes of chronic myeloid leukemia (CML) in children in the Sakha Republic (Yakutia) (SR (Ya)). This disease occurs very rarely in children: over 21 years, only three children with such a diagnosis have been registered in the SR(Ya). The clinical and laboratory picture of CML in children does not differ from that in adults. Two children, except for the first patient, who was diagnosed in 1997, had a cytogenetic study of the bone marrow. With standard karyotyping, patient 2 was found to have translocation t(4;9;22) (q28;q34;q11) - Philadelphia’s Ph-chromosome; the FISH study revealed the presence of chimeric BCR/ABL gene in 100 % of the cells. Patient 3 only underwent karyotyping, and the Ph-chromosome, t (9; 22) (q34; q11) was also detected. At present, tyrosine kinase inhibitors are the main standard for the treatment of CML. Patient 1 became ill before the introduction of tyrosine kinase inhibitors into CML therapy, her life expectancy from the onset of the disease was only 2 years. Two children are treated with imatinib, a first generation tyrosine kinase inhibitor. The duration of the disease in them was 11 months and 9 years 3 months. In Patient 2, after 7 months from the start of therapy, a pronounced molecular response was achieved, in Patient 3 - a loss of the molecular response due to long interruptions in treatment. The side effects of ICT therapy were insignificant.