Acute Myeloid Leukemia with Core Binding Factor Rearrangements: A 10-Year Cancer Center Experience.

IF 2.5 3区医学 Q3 ONCOLOGY Oncology Pub Date : 2025-02-17 DOI:10.1159/000544049

Shehab Fareed, Dina Sameh Soliman, Abdulrahman F Al-Mashdali, Amna Gameil, Yahya Mulikandathil, Awni Alshurafa, ElMustafa Abdalla, Abdallah Fadul, Sarah Aldali, Deena Mudawi, Kaplana Singh, Feryal Ibrahim, Anas Hamad, Honar Cherif, Mohamed Yassin

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Abstract

Background: Core-binding factor acute myeloid leukemia (CBF-AML) is characterized by t(8;21) or inv(16)/t(16;16) chromosomal rearrangements.

Methods: In this retrospective study of 71 CBF-AML cases from Qatar's National Center for Cancer Care & Research (2013-2022), we analyzed clinicopathological characteristics and survival outcomes.

Results: The cohort was predominantly male (76%) with a median age of 40 years, and 96% were de novo AML. The t(8;21) translocation was more frequent (69%) than inv(16)/t(16;16). Patients under 40 years showed higher white blood cell counts and blast percentages. Standard "3+7" induction chemotherapy (used in 69% of cases) achieved remission in 67% of patients. Median overall survival was 81% at 37 months, with median progression-free survival of 34 months. No significant survival differences were observed based on FLT3-ITD status, translocation type, complex karyotype, KIT mutation status, or allogeneic stem cell transplantation (performed in 19% of patients), though patients under 60 years demonstrated better survival outcomes.

Conclusions: This study highlights CBF-AML heterogeneity and challenges established prognostic markers, suggesting a need for risk stratification reassessment, treatment strategy optimization, ELN guidelines implementation, and continuous molecular monitoring.

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背景：核心结合因子急性髓性白血病（CBF-AML）以t（8;21）或inv（16）/t（16;16）染色体重排为特征：在这项回顾性研究中，我们分析了卡塔尔国家癌症治疗与研究中心（2013-2022年）的71例CBF-AML临床病理特征和生存结果：患者主要为男性（76%），中位年龄为40岁，96%为新发急性髓细胞性白血病。与inv(16)/t(16;16)相比，t(8;21)易位更为常见（69%）。40岁以下的患者白细胞计数和出血率均较高。标准的 "3+7 "诱导化疗（用于69%的病例）使67%的患者病情得到缓解。中位总生存期为 81%，37 个月，中位无进展生存期为 34 个月。根据FLT3-ITD状态、易位类型、复杂核型、KIT突变状态或异体干细胞移植（19%的患者进行了异体干细胞移植），未观察到明显的生存率差异，但60岁以下的患者生存率更高：这项研究强调了CBF-AML的异质性，并对既有的预后指标提出了挑战，表明需要重新评估风险分层、优化治疗策略、实施ELN指南并进行持续的分子监测。

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

Oncology 医学-肿瘤学

CiteScore

6.00

自引率

2.90%

发文量

审稿时长

6-12 weeks

期刊介绍： Although laboratory and clinical cancer research need to be closely linked, observations at the basic level often remain removed from medical applications. This journal works to accelerate the translation of experimental results into the clinic, and back again into the laboratory for further investigation. The fundamental purpose of this effort is to advance clinically-relevant knowledge of cancer, and improve the outcome of prevention, diagnosis and treatment of malignant disease. The journal publishes significant clinical studies from cancer programs around the world, along with important translational laboratory findings, mini-reviews (invited and submitted) and in-depth discussions of evolving and controversial topics in the oncology arena. A unique feature of the journal is a new section which focuses on rapid peer-review and subsequent publication of short reports of phase 1 and phase 2 clinical cancer trials, with a goal of insuring that high-quality clinical cancer research quickly enters the public domain, regardless of the trial’s ultimate conclusions regarding efficacy or toxicity.