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儿童急性淋巴细胞白血病的诱导化疗反应及其与细胞遗传学和分子特征的相关性。

Induction Chemotherapy Response in Childhood Acute Lymphoblastic Leukaemia and its Correlation with Cytogenetic and Molecular Features.

机构信息

Department of Haematology, Armed Forces Institute of Pathology, Rawalpindi, Pakistan.

Outpatient Department, Armed Forces Institute of Pathology, Rawalpindi, Pakistan.

出版信息

J Coll Physicians Surg Pak. 2022 Nov;32(11):1430-1434. doi: 10.29271/jcpsp.2022.11.1430.

Abstract

OBJECTIVE

To study the correlation of cytogenetic and molecular abnormalities on induction chemotherapy in childhood acute lymphoblastic leukaemia (ALL).

STUDY DESIGN

Analytical study.

PLACE AND DURATION OF STUDY

Department of Haematology, Armed Forces Institute of Pathology (AFIP), from March 2021 to August 2021.

METHODOLOGY

Patients aged 1-18 years with newly diagnosed acute lymphoblastic leukaemia were inducted. Patients aged less than 1 year and more than 18 years were excluded from the study. The diagnosis was based on morphology, cytochemistry, flow cytometry, and cytogenetic/molecular analysis. Risk stratification was done on the basis of age, TLC, and cytogenetic/molecular defects. The UKALL 2011 protocol was used for treatment with regimen-A for standard risk and regimen-B for high-risk patients. Bone marrow was repeated on day 29 of induction therapy and blast percentage was assessed to establish post-induction remission. Association between cytogenetic / molecular abnormalities and post-induction remission status was analysed using chi-square test.

RESULTS

There were total 142 patients with mean age of 6.4 + 3.6 years and a male- to-female ratio of 2.7:1. Immunophenotyping revealed 85.9% cases as B-cell ALL and 14.1% as T-cell ALL. The most frequent cytogenetic and molecular abnormalities were hyperdiploidy (19%), t(9;22)/BCR-ABL1(p190) (10.6%), complex karyotype (5.6%), E2A-PBX1 (8.5%), and TEL-AML1 (4.9%). A total of 127/142 (89.4%) achieved haematological remission after induction therapy with two deaths during induction therapy (1.4%). Post-induction remission rate in patients with favorable cytogenetic/molecular defects was 100% and in children with bad prognostic changes, the rate of remission was 69.2%. Chi-square test showed a significant association between cytogenetic/molecular abnormalities and post-induction remission (p-value <0.001).

CONCLUSION

Cytogenetic and molecular abnormalities have a significant association with post-induction remission in children with acute lymphoblastic leukaemia.

KEY WORDS

Acute lymphoblastic leukaemia, Cytogenetics, Chemotherapy, Induction, Remission.

摘要

目的

研究儿童急性淋巴细胞白血病(ALL)诱导化疗中细胞遗传学和分子异常的相关性。

研究设计

分析性研究。

地点和研究时间

武装部队病理学研究所(AFIP)血液科,2021 年 3 月至 2021 年 8 月。

方法

对新诊断为急性淋巴细胞白血病的 1-18 岁患者进行诱导。排除年龄小于 1 岁和大于 18 岁的患者。诊断基于形态学、细胞化学、流式细胞术和细胞遗传学/分子分析。根据年龄、TLC 和细胞遗传学/分子缺陷进行风险分层。采用 UKALL 2011 方案治疗标准风险患者采用方案-A,高风险患者采用方案-B。在诱导治疗第 29 天重复骨髓检查,并评估 blast 百分比以确定诱导后缓解情况。采用卡方检验分析细胞遗传学/分子异常与诱导后缓解状态的关系。

结果

共 142 例患者,平均年龄 6.4+3.6 岁,男女比例为 2.7:1。免疫表型显示 85.9%的病例为 B 细胞 ALL,14.1%的病例为 T 细胞 ALL。最常见的细胞遗传学和分子异常为超二倍体(19%)、t(9;22)/BCR-ABL1(p190)(10.6%)、复杂核型(5.6%)、E2A-PBX1(8.5%)和 TEL-AML1(4.9%)。诱导治疗后 142 例中有 127 例(89.4%)达到血液学缓解,诱导治疗期间有 2 例死亡(1.4%)。具有良好细胞遗传学/分子特征的患者诱导后缓解率为 100%,具有不良预后变化的患儿缓解率为 69.2%。卡方检验显示细胞遗传学/分子异常与诱导后缓解有显著相关性(p 值<0.001)。

结论

细胞遗传学和分子异常与儿童急性淋巴细胞白血病诱导后缓解有显著相关性。

关键词

急性淋巴细胞白血病;细胞遗传学;化学疗法;诱导;缓解。

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