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青少年及青年慢性髓系白血病:临床病理变量与预后

Chronic Myeloid Leukemia in Adolescents and Young Adults: Clinicopathological Variables and Outcomes.

作者信息

Abdulla Mohammad A J, Aldapt Mahmood B, Chandra Prem, El Akiki Susanna, Alshurafa Awni, Nashwan Abdulqadir J, Fernyhough Liam J, Sardar Sundus, Chapra Ammar, Yassin Mohamed A

机构信息

Department of Hematology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

Internal Medicine Department, Ibn Sina Specialized Hospital, Jenin, Palestine.

出版信息

Oncology. 2024;102(12):1018-1028. doi: 10.1159/000539982. Epub 2024 Jul 16.

Abstract

INTRODUCTION

Adolescents and young adults (AYAs) diagnosed with chronic myeloid leukemia (CML) constitute a significant demographic group, particularly in regions with youthful populations like Qatar. Despite the global median age of CML diagnosis being 65 years, Qatar's age distribution reflects a younger cohort. This study investigates whether AYAs with CML exhibit distinct clinicopathological characteristics or outcomes compared to older age groups.

METHODS

A total of 224 CML patients were enrolled, including 114 AYAs (defined as ages 15 through 39). Demographic and clinical parameters, including gender, BMI, BCR-ABL1 transcript type, white blood cell (WBC) count, hemoglobin level, platelet count, and spleen size, were compared between AYAs and older patients. Prognostic scoring systems (Sokal, Hasford, EUTOS, and ELTS) and molecular response rates (MMR and DMR) were also evaluated.

RESULTS

AYAs demonstrated higher WBC counts at diagnosis (median 142.3 vs. 120; p = 0.037) and lower hemoglobin levels (10.5 vs. 11.40; p = 0.004) compared to older patients. Spleen size was significantly larger in AYAs (18.8 vs. 15.5; p = 0.001). While AYAs showed better prognostic scores by Sokal and Hasford criteria, EUTOS and ELTS scores indicated comparable risk stratification. However, AYAs exhibited lower rates of MMR (56.7 vs. 73.4%; p = 0.016) and achieved MMR at a slower pace (median time 130 vs. 103 months; p = 0.064). Similarly, the percentage of DMR was lower in AYAs (37.1 vs. 46.8%; p = 0.175).

CONCLUSION

Despite their younger age, AYAs with CML displayed poorer prognoses compared to older patients. These findings underscore the importance of tailored management strategies for AYAs with CML to optimize outcomes in this distinct patient population.

KEY POINT

AYAs are underrepresented in CML studies and risk scores, so this is the focus of this study.

摘要

引言

被诊断为慢性髓性白血病(CML)的青少年和青年(AYA)构成了一个重要的人群,尤其是在卡塔尔等人口年轻化的地区。尽管全球CML诊断的中位年龄为65岁,但卡塔尔的年龄分布显示出更年轻的队列。本研究调查与老年人群相比,患有CML的AYA是否表现出不同的临床病理特征或预后。

方法

共纳入224例CML患者,其中114例为AYA(定义为年龄15至39岁)。比较了AYA与老年患者的人口统计学和临床参数,包括性别、体重指数、BCR-ABL1转录本类型、白细胞(WBC)计数、血红蛋白水平、血小板计数和脾脏大小。还评估了预后评分系统(Sokal、Hasford、EUTOS和ELTS)和分子反应率(MMR和DMR)。

结果

与老年患者相比,AYA在诊断时白细胞计数更高(中位数142.3对120;p = 0.037),血红蛋白水平更低(10.5对11.40;p = 0.004)。AYA的脾脏大小明显更大(18.8对15.5;p = 0.001)。虽然根据Sokal和Hasford标准,AYA的预后评分更好,但EUTOS和ELTS评分表明风险分层相当。然而,AYA的MMR发生率较低(56.7%对73.4%;p = 0.016),达到MMR的速度较慢(中位时间130对103个月;p = 0.064)。同样,AYA的DMR百分比更低(37.1%对46.8%;p = 0.175)。

结论

尽管年龄较小,但与老年患者相比,患有CML的AYA预后较差。这些发现强调了为患有CML的AYA制定量身定制的管理策略对于优化这一独特患者群体的治疗结果的重要性。

关键点

AYA在CML研究和风险评分中的代表性不足,因此这是本研究的重点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/793e/11614302/a09da5d710f2/ocl-2024-0102-0012-539982_F01.jpg

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