临床血液学和细胞遗传学特征在哈萨克斯坦共和国儿童b细胞白血病方案治疗中的作用

L. Manzhuova, G. Tashenova, A. Tulebayeva, E. Sarsekbaev, A. Zhailaubaeva, G. Omarova, D. Yeginbergenova
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引用次数: 0

摘要

相关性:研究儿童白血病的免疫学和分子遗传学特征以及急性b细胞淋巴细胞白血病(B-ALL)肿瘤群体的生物学特征对治疗有效性的影响,对哈萨克斯坦共和国具有特别重要的意义。该研究旨在根据b细胞白血病的生物学特性评估现代方案化疗在儿童中的有效性。方法:本研究分析了2016-2018年在哈萨克斯坦阿拉木图儿科外科科学中心JSC (Almaty, Republic of Kazakhstan)住院治疗的154名6个月至15岁原发性B-ALL患儿的数据。在确定事件时,我们以ALL-BFM组的协议标准为指导。结果:B-ALL暴露最多的年龄组为3-7岁(43.5%),反映了所谓的婴儿高峰。在这类急性淋巴细胞白血病的临床表现中,75.3%的患者在表现期伴有中毒综合征。首发期的临床多态性决定了“面具”的诊断最多样化。41例(26.6%)患儿出现肝脏功能衰竭,12例(7.8%)患儿出现呼吸衰竭,5例(3.2%)患儿出现心血管功能衰竭,3例(1.9%)患儿出现急性肾损伤,5例(3.2%)患儿出现中枢神经系统损伤。B-ALL的免疫变异分布如下:B1 - 9 (5.8%), B2 - 123 (79.8%), B3 - 18 (11.7%), B4 - 4(2.6%), 1例患者有b细胞淋巴瘤白血病(0.6%)。在定量异常组中,12例(7.8%)检测到高倍体。在定性异常中,6例(3.9%)患者出现t(12;21) (p13;q22),这是一个有利的预后因素(有缓解记录)。合并异常3例(1.9%)21号染色体三体合并唐氏综合征(7号同染色体,4、6、15、17号三体,易位t(9;22)(q34;q11)的检出率为1.3%。易位t(1;19)(q23;p13.3)占5.8%,9号染色体缺陷占3.2%。结论:对治疗的反应和长期预后在很大程度上取决于肿瘤的细胞遗传学特征、对强的松的敏感性以及侵袭程度等生物学因素,表现为明显的淋巴细胞增生和白细胞增多症。研究表明,现代ALL-BFM方案治疗儿童的疗效很高。
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THE ROLE OF CLINICAL-HEMATOLOGICAL AND CYTOGENETIC CHARACTERISTICS IN THE PROGRAM THERAPY OF B-CELL LEUKEMIA IN CHILDREN IN THE REPUBLIC OF KAZAKHSTAN
Relevance: The study of immunological and molecular genetic characteristics of leukemia in children and the influence of biological features of the tumor population of acute B-cell lymphoblastic leukemia (B-ALL) on the effectiveness of therapy is particularly relevant for the Republic of Kazakhstan. The study aimed to evaluate the effectiveness of modern program chemotherapy in children depending on the biological characteristics of B-cell leukemias. Methods: The study analyzed the data of 154 children aged six months to 15 years with primary B-ALL on inpatient treatment at the Scientific Center of Pediatrics and Pediatric Surgery JSC (Almaty, the Republic of Kazakhstan) in 2016-2018. When determining events, we were guided by the criteria of the protocols of the ALL-BFM group. Results: The age groups most exposed to B-ALL were 3-7 years old (43.5%), reflecting the so-called infant peak. In the clinical picture of this type of ALL, intoxication syndrome accompanying the period of manifestation was present in 75.3% of patients. The clinical polymorphism of the debut period determined the most diverse list of diagnoses of “masks.” Damage to organs and systems, in the form of liver failure, was detected in 41 (26.6%) children, with the development of respiratory failure in 12 (7.8%), cardiovascular failure in 5 patients (3.2%), acute kidney injury in 3 (1.9%), CNS damage in 5 (3.2%) patients. With B-ALL, the distribution of immunological variants was determined as follows: B1 – 9 (5.8%), B2 – 123 (79.8%), B3 – 18 (11,7%), B4 – 4 (2,6%) and leukemia of B-cell lymphoma was noted in one (0.6%) patient. From the group of quantitative anomalies, hyperploidy was detected in 12 (7.8%) cases. Among qualitative anomalies, t(12;21) (p13;q22) was determined in 6 (3.9%) patients and was a favorable prognostic factor (remission was recorded). Trisomy of chromosome 21 with Down syndrome in 3 (1.9%) patients with combined anomalies (isochromosome 7, trisomy 4, 6, 15, 17, translocation t(9; 22)(q34;q11) was detected in 1.3%. Translocations t(1;19)(q23;p13.3) in 5.8% and del 9-chromosome defect in 3.2% of cases. Conclusion: The response to therapy and long-term prognosis are largely determined by biological factors such as cytogenetic features of the tumor, sensitivity to prednisone, as well as the degree of aggressiveness, which manifests itself in the form of pronounced symptoms of lymphoproliferation and hyperleukocytosis. The research has shown the high efficiency of modern ALL-BFM program therapy in children.
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