Abstract

Acute myeloid leukemia (AML) occurs in frequency 1.5 per 100 000 children and occupy 15–20 % in the structure of pediatric acute leukemia. In comparison with adults, pediatric AML usually a primary disease; clonal evolution of myeloproliferative disorders is rare situation in clinical course of the disease. Pediatric AML classification based on combination of morphology, cytochemistry, immune phenotyping and molecular genetics and situated in the process of permanent revision. Doctrine of AML treatment, excluding acute promyelocytic leukemia, was not changed during last 30 years, but the survival rate increased from 40 to 60–65 % because of stratification of patients on risk groups, intensification of chemotherapy, application of epigenetic/target therapy, improvement of supportive care and widening of indications for hematopoietic stem cell transplantation in the clinical program of treatment. Children in the age under 2 years old with AML is the unique group of patients with different biology of process, genetic aberrations, concentration of unfavorable risk factors and expressed predisposition to therapeutic toxicity. Progress of the treatment for this subgroup with usage of risk-based protocols of treatment during the last 10 years leads to change of prognosis – now the prognosis is the same in comparison with more adult groups of patients. Thus, 25–30 % of patients with AML in the age of 0–18 years old can be faced with relapse of leukemia, 5–10 % will die because of complications of disease and/or side effects of therapy. Prospects of treatment optimization for children are connected with target therapy of different genetic variants of AML, like for promyelocytic leukemia or chronic myeloid leukemia, as well as in decreasing of the toxicity of therapy. Review of international studies for AML treatment for children is presented in this article as well as discussion on prospect of optimization of treatment.

Highlights

  • Pediatric Acute myeloid leukemia (AML) classification based on combination of morphology, cytochemistry, immune phenotyping and molecular genetics and situated in the process of permanent revision

  • Children in the age under 2 years old with AML is the unique group of patients with different biology of process, genetic aberrations, concentration of unfavorable risk factors and expressed predisposition to therapeutic toxicity

  • 25–30 % of patients with AML in the age of 0–18 years old can be faced with relapse of leukemia, 5–10 % will die because of complications of disease and/or side effects of therapy

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Summary

ГЕМАТОЛОГИИ и ОНКОЛОГИИ

Острый миелобластный лейкоз (ОМЛ) встречается с частотой 1,5 на 100 000 детей и занимает 15–20 % в структуре детских острых лейкозов. Перспективы оптимизации лечения детей связаны с таргетной терапией различных генетических вариантов ОМЛ по аналогии с острым промиелоцитарным и хроническим миелолейкозом, а также снижением токсичности терапии. В статье представлен обзор международных исследований по лечению ОМЛ у детей и обсуждены перспективы оптимизации лечения больных. Ключевые слова: острый миелобластный лейкоз у детей, биология, цитогенетика, молекулярные перестройки, перспективы оптимизации лечения. Children in the age under 2 years old with AML is the unique group of patients with different biology of process, genetic aberrations, concentration of unfavorable risk factors and expressed predisposition to therapeutic toxicity. Prospects of treatment optimization for children are connected with target therapy of different genetic variants of AML, like for promyelocytic leukemia or chronic myeloid leukemia, as well as in decreasing of the toxicity of therapy. Наиболее важные разделы настоящей классификации включают ОМЛ с рекуррентными генетическими аномалиями, ОМЛ с миелодиспластическим синдромом, ятрогенный и неклассифицируемый ОМЛ

ОМЛ с рекуррентными генетическими аномалиями
Новообразование из бластных плазмоцитоидных дендритных клеток
Перспективы оптимизации лечения острого миелобластного лейкоза
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