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急性髓系白血病:流行病学与病因学

Acute myeloid leukemia: epidemiology and etiology.

作者信息

Deschler Barbara, Lübbert Michael

机构信息

Department of Hematology/Oncology, University of Freiburg, Freiburg, Germany.

出版信息

Cancer. 2006 Nov 1;107(9):2099-107. doi: 10.1002/cncr.22233.

Abstract

Acute myeloid leukemias (AMLs) are infrequent, yet highly malignant neoplasms responsible for a large number of cancer-related deaths. The incidence has been near stable over the last years. It continuously shows 2 peaks in occurrence in early childhood and later adulthood. With an incidence of 3.7 per 100,000 persons and an age-dependent mortality of 2.7 to nearly 18 per 100,000 persons, there is a rising awareness in the Western world of AML's special attributes resulting from an ever-aging population. To objectively describe epidemiologic data on this patient population, recent publications were evaluated to make transparent the current trends and facts. A review of the literature is presented, reflecting highlights of current research with respect to AML etiology. To estimate outcome and discuss informed treatment decisions with AML patients of different age groups and different biologic risk categories, it is mandatory to consider that the outcome results reported in clinical trials were until now heavily biased toward younger patients, whereas the overall dismal prognosis documented in population-based studies most likely reflects the exclusion of older patients from aggressive treatment. The etiology for most cases of AML is unclear, but a growing knowledge concerning leukemogenenic agents within chemotherapy regimens for other malignancies is already available. This includes specific associations of the most frequent balanced translocations in AML, including the "good-risk" abnormalities comprised by the core binding factor leukemias (i.e., AML with the translocation (8;21) and inversion of chromosome 16, and acute promyelocytic leukemia with the translocation (15;17)). In contrast to these genetic alterations, epigenetic lesions, e.g., promoter silencing by hypermethylation of the p15/INK4b and other genes, are increasingly recognized as important in the pathogenesis of AML.

摘要

急性髓系白血病(AML)并不常见,但却是高度恶性的肿瘤,导致大量癌症相关死亡。过去几年其发病率一直接近稳定。在幼儿期和成年后期,发病率持续呈现两个高峰。AML的发病率为每10万人中有3.7例,年龄依赖性死亡率为每10万人中有2.7至近18例。在西方世界,由于人口老龄化,人们对AML的特殊属性的认识日益提高。为了客观描述该患者群体的流行病学数据,对近期发表的文献进行了评估,以明确当前的趋势和事实。本文对文献进行了综述,反映了当前AML病因学研究的重点。为了评估不同年龄组和不同生物学风险类别的AML患者的预后并讨论明智的治疗决策,必须考虑到目前临床试验报告的结果严重偏向年轻患者,而基于人群的研究中记录的总体预后不佳很可能反映了老年患者被排除在积极治疗之外。大多数AML病例的病因尚不清楚,但对于其他恶性肿瘤化疗方案中的致白血病因素的认识正在不断增加。这包括AML中最常见的平衡易位的特定关联,包括核心结合因子白血病所包含的“低风险”异常(即伴有(8;21)易位和16号染色体倒位的AML,以及伴有(15;17)易位的急性早幼粒细胞白血病)。与这些基因改变相反,表观遗传损伤,例如p15/INK4b和其他基因的高甲基化导致的启动子沉默,在AML发病机制中的重要性日益得到认可。

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