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分子和细胞遗传学反应对慢性髓性白血病儿童和青少年长期预后的影响:一项来自印度的回顾性研究

Impact of Molecular and Cytogenetic Responses on Long-Term Outcomes in Children and Adolescents With Chronic Myeloid Leukemia: A Retrospective Study From India.

作者信息

Dave Dipesh, Trivedi Maharshi, Doctor Chinmay, Parikh Biren, Panchal Harsha P, Yadav Rajan

机构信息

Pediatric Oncology, The Gujarat Cancer and Research Institute, Ahmedabad, IND.

Oncopathology, The Gujarat Cancer and Research Institute, Ahmedabad, IND.

出版信息

Cureus. 2025 Aug 4;17(8):e89359. doi: 10.7759/cureus.89359. eCollection 2025 Aug.

DOI:10.7759/cureus.89359
PMID:40909024
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12407574/
Abstract

Introduction Chronic myelogenous leukemia (CML) in pediatric and adolescent populations is relatively rare. The present study provides an integrated approach to evaluate the impact of molecular and cytogenetic response on long-term outcomes in these populations by incorporating demographic factors and hematological parameters, and to explore their clinical relevance in resource-limited settings. Material and methods A retrospective analysis was conducted on patients <18 years with newly diagnosed CML from January 2014 to December 2023 at the Gujarat Cancer and Research Institute in India. Data were retrieved from the hospital database, including demographics, clinical details, hematological parameters, CML phase at diagnosis, and cytogenetic and molecular studies. Of 73 diagnosed patients, eighteen were not subjected to molecular, cytogenetic, and complete hematological response (CHR) analysis due to factors such as irregular follow-up, testing constraints, and financial or logistic barriers. Results Of the 73 patients, seventeen (23%) were below 10 years of age, and 56 (77%) were above 10 years at the time of diagnosis. The male-to-female ratio was 1.8:1. Seventy (96%) of the 73 patients were initially diagnosed with CML in the chronic phase, and three patients (4%) were diagnosed with CML in blast crisis. Response evaluation was conducted on 55 patients. Of these, CHR was observed in 41 (74%) patients at 3 months. At 12 months, forty (73%) achieved complete cytogenetic response (CCyR), and 25 (45%) achieved major molecular response (MMR). Among the 55 patients, those who achieved CCyR and MMR at 12 months had a 100% five-year survival, compared to 66.7% and 83%, respectively, in those who did not achieve a response. Patients who achieved early molecular response (EMR) had a 97.4% five-year survival. The Kaplan-Meier estimated overall survival was 91.6% at 3 years and 87.6% at 5 years. Fifty of the 55 patients (91%) were alive at long-term follow-up, with a median duration of follow-up of 62 months (range: 0-128 months). Patients aged 0-10 years and >10 years had five-year survival rates of 100% and 88%, respectively. Conclusion The achievement of molecular and cytogenetic responses has a significant impact on long-term outcomes in pediatric and adolescent patients diagnosed with CML, highlighting their role as crucial predictors in clinical practice. Patients who achieved both MMR and CCyR demonstrated 100% five-year overall survival (OS), while those with EMR showed a 97.4% five-year survival. These findings support the utility of early response monitoring to inform prognosis and guide therapy, particularly in low- and middle-income countries (LMICs). However, treatment abandonment and poor compliance remain major obstacles in LMICs. Further research with multicentric prospective trials in LMICs is essential.

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/608d6fcd89ba/cureus-0017-00000089359-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/08eddb40a8cb/cureus-0017-00000089359-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/8136878994bf/cureus-0017-00000089359-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/4f708b43020b/cureus-0017-00000089359-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/608d6fcd89ba/cureus-0017-00000089359-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/08eddb40a8cb/cureus-0017-00000089359-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/8136878994bf/cureus-0017-00000089359-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/4f708b43020b/cureus-0017-00000089359-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea6c/12407574/608d6fcd89ba/cureus-0017-00000089359-i04.jpg
摘要

引言 儿童和青少年慢性髓性白血病(CML)相对罕见。本研究提供了一种综合方法,通过纳入人口统计学因素和血液学参数来评估分子和细胞遗传学反应对这些人群长期预后的影响,并探讨其在资源有限环境中的临床相关性。

材料和方法 对2014年1月至2023年12月在印度古吉拉特癌症与研究所新诊断为CML的18岁以下患者进行回顾性分析。数据从医院数据库中检索,包括人口统计学、临床细节、血液学参数、诊断时的CML分期以及细胞遗传学和分子研究。在73例确诊患者中,18例因随访不规律、检测限制以及经济或后勤障碍等因素未进行分子、细胞遗传学和完全血液学缓解(CHR)分析。

结果 在73例患者中,17例(23%)诊断时年龄小于10岁,56例(77%)诊断时年龄大于10岁。男女比例为1.8:1。73例患者中有70例(96%)最初诊断为慢性期CML,3例(4%)诊断为急变期CML。对55例患者进行了缓解评估。其中,41例(74%)患者在3个月时达到CHR。12个月时,40例(73%)达到完全细胞遗传学缓解(CCyR),25例(45%)达到主要分子缓解(MMR)。在这55例患者中,12个月时达到CCyR和MMR的患者五年生存率为100%,未达到缓解的患者分别为66.7%和83%。达到早期分子缓解(EMR)的患者五年生存率为97.4%。Kaplan-Meier估计的总生存率3年时为91.6%,5年时为87.6%。55例患者中有50例(91%)在长期随访时存活,中位随访时间为62个月(范围:0 - 128个月)。0 - 10岁和大于10岁的患者五年生存率分别为100%和88%。

结论 分子和细胞遗传学反应的实现对诊断为CML 的儿童和青少年患者的长期预后有显著影响,突出了它们在临床实践中作为关键预测指标的作用。同时达到MMR和CCyR的患者五年总生存率(OS)为100%,而达到EMR的患者五年生存率为97.4%。这些发现支持早期缓解监测对判断预后和指导治疗的实用性,特别是在低收入和中等收入国家(LMICs)。然而,在LMICs中,治疗放弃和依从性差仍然是主要障碍。在LMICs中进行多中心前瞻性试验的进一步研究至关重要。

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