CLINICAL FEATURES AND RESULTS OF THERAPY IN INFANTS AND CHILDREN AGED 1 TO 3 YEARS OLD WITH ACUTE MYELOID LEUKEMIA

Q4 Medicine Pediatriya - Zhurnal im G.N. Speranskogo Pub Date : 2023-06-01 DOI:10.24110/0031-403x-2023-102-3-8-15

A.K. Ignatova, I.I. Kalinina, D.A. Venyov, D.A. Evseev, T.Yu. Salimova, M.N. Sadovskaya, O.V. Goronkova, E.V. Suntsova, V.E. Matveev, U.N. Petrova, K.S. Antonova, D.D. Baydildina, K.A. Voronin, P.A. Levin, M.E. Dubrovina, T.V. Konyukhova, Yu.V. Olshanskaya, E.A. Zerkalenkova, M.V. Gaskova, A.M. Popov, S.A. Kashpor, M.A. Maschan, A.A. Maschan

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Abstract

Infants of the first year of life represent a unique group of patients with acute myeloid leukemia (AML). Materials and methods of the research: the characteristics of 492 patients with newly diagnosed AML aged 10 DoL-18 y/o who received intensive chemotherapy according to the AML-MM-2006 and AML-MRD-2018 guidelines in Apr. 2007-Apr. 2021 were analyzed. The analysis was carried out separately for infants (<1 y/o, 58/12%), young children (1-3 y/o, 99/20%) and children aged 3-18 y/o (335/68%). Results: the infant group was characterized by a higher incidence of hyperleukocytosis, extramedullary lesions, the predominance of monoblast/monocytic (63%) and megakaryoblast (24%) subvariants and chromosomal aberrations involving the 11q23 locus (KMT2A gene) (53%). “CBF leukemias” were virtually non-existent in infants (0% for t(8;21)(q22;q22.1)/RUNX1::RUNX1T1 and 2% for inv(16)(p13.1q22)/CBF::MYH11) and the rates increased with age. Activating mutations in the FLT3 gene were extremely rare (2% vs. 13.5% in patients >/=3 y/o). Patients of the 1-3 y/o group were similar in morphological and cytogenetic characteristics to the infants’ group but had a smaller tumor mass and higher survival rates. The 5-year overall survival of infants was statistically significantly lower than in older patients, 52% vs. 67% (p<0.001), primarily due to high mortality prior to remission, which in its turn was caused by complications associated with hyperleukocytosis. Conclusions: given the vulnerability of this category of patients, it is reasonable to transfer them to larger medical facilities with extensive experience in the field in order to conduct the remission induction stage (especially cytoreduction) within the intensive care unit.

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1 ~ 3岁婴幼儿急性髓性白血病的临床特点和治疗结果

一岁的婴儿是急性髓性白血病(AML)患者的一个独特群体。研究材料与方法:2007年4月至4月，492例10 dol18 y/o新诊断AML患者根据AML- mm -2006和AML- mrd -2018指南接受强化化疗的特点。2021年进行了分析。分别对婴儿(1岁，58/12%)、幼儿(1-3岁，99/20%)和3-18岁儿童(335/68%)进行分析。结果:婴儿组的特点是白细胞增多、髓外病变发生率较高，单核细胞/单核细胞(63%)和巨核细胞(24%)亚变异体占主导地位，涉及11q23位点(KMT2A基因)的染色体畸变(53%)。“CBF白血病”在婴儿中几乎不存在(t(8;21)(q22;q22.1)/RUNX1::RUNX1T1为0%，inv(16)(p13.1q22)/CBF::MYH11为2%)，并且随着年龄的增长而增加。FLT3基因的激活突变极为罕见(2% vs. 13.5%)。1-3岁组患者的形态学和细胞遗传学特征与婴儿组相似，但肿瘤体积较小，生存率较高。婴儿的5年总生存率在统计学上显著低于老年患者，52% vs. 67% (p<0.001)，这主要是由于缓解前的高死亡率，而缓解前的高死亡率又由白细胞增多症相关的并发症引起。结论:鉴于这类患者的脆弱性，合理的做法是将他们转移到在该领域有丰富经验的大型医疗机构，以便在重症监护病房内进行缓解诱导阶段(特别是细胞减少)。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatriya - Zhurnal im G.N. Speranskogo Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.60

自引率

0.00%

发文量

150

期刊介绍： Journal “Pediatria” named after G.N. Speransky (the official short names of the Journal are “Journal «Pediatria»,” “Pediatria,” and “«Pediatria,» the Journal”) is the oldest Soviet-and-Russian (in the Russian Federation, the CIS and former Soviet Union) scientific and practical medical periodical assigned for pediatricians that is published continuously since May, 1922, and distributed worldwide. Our mission statement specifies that we aim to the ‘raising the level of skills and education of pediatricians, organizers of children’s health protection services, medicine scientists, lecturers and students of medical institutes for higher education, universities and colleges worldwide with an emphasis on Russian-speaking audience and specific, topical problems of children’s healthcare in Russia, the CIS, Baltic States and former Soviet Union Countries and their determination with the use of the World’s best practices in pediatrics.’ As part of this objective, the Editorial of the Journal «Pediatria» named after G.N. Speransky itself adopts a neutral position on issues treated within the Journal. The Journal serves to further academic discussions of topics, irrespective of their nature - whether religious, racial-, gender-based, environmental, ethical, political or other potentially or topically contentious subjects. The Journal is registered with the ISSN, - the international identifier for serials and other continuing resources, in the electronic and print world: ISSN 0031-403X (Print), and ISSN 1990-2182 (Online). The Journal was founded by the Academician, Dr. Georgiy Nestorovich SPERANSKY, in May, 1922. Now (since 1973) the Journal bears his honorary name.