McKenzie Shirlyn B
Department of Clinical Laboratory Sciences, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229-3900, USA.
Clin Lab Sci. 2005 Winter;18(1):28-37.
Acute myelocytic leukemia (AML) is a malignant neoplasm of hematopoietic cells characterized by an abnormal proliferation of myeloid precursor cells, decreased rate of self-destruction and an arrest in cellular differentiation. The leukemic cells have an abnormal survival advantage. Thus, the bone marrow and peripheral blood are characterized by leukocytosis with a predominance of immature cells, primarily blasts. As the immature cells accumulate in the bone marrow, they replace the normal myelocytic cells, megakaryocytes, and erythrocytic cells. This leads to a loss of normal bone marrow function and associated complications of bleeding, anemia, and infection. The incidence of AML increases with age, peaking in the sixth decade of life. In the United States, there are about 10,000 new cases of AML and 7,000 deaths in those with an AML diagnosis per year. Current molecular studies of AML demonstrate that it is a heterogeneous disorder of the myeloid cell lineage. This paper will discuss the most recent understanding and research of the cellular origin of AML and associated common genetic mutations that fuel the neoplastic process. Also discussed are how these advances have impacted the classification, selection of therapy, and definition of complete remission in AML. Promyelocytic leukemia will be discussed in detail as this AML subtype reveals how our understanding of the biology and genetics of the disease has led to targeted therapy that results in a cure in up to 80% of patients.
急性髓细胞白血病（AML）是一种造血细胞的恶性肿瘤，其特征为髓系前体细胞异常增殖、自我毁灭速率降低以及细胞分化停滞。白血病细胞具有异常的生存优势。因此，骨髓和外周血的特征是白细胞增多，以不成熟细胞为主，主要是原始细胞。随着不成熟细胞在骨髓中积累，它们取代了正常的髓细胞、巨核细胞和红细胞。这导致正常骨髓功能丧失以及出血、贫血和感染等相关并发症。AML的发病率随年龄增长而增加，在60岁时达到峰值。在美国，每年约有10000例新的AML病例，且有7000例AML诊断患者死亡。目前对AML的分子研究表明，它是髓系细胞谱系的一种异质性疾病。本文将讨论对AML细胞起源的最新认识和研究以及驱动肿瘤形成过程的相关常见基因突变。还将讨论这些进展如何影响AML的分类、治疗选择和完全缓解的定义。将详细讨论早幼粒细胞白血病，因为这种AML亚型揭示了我们对该疾病生物学和遗传学的理解如何导致靶向治疗，使高达80%的患者得以治愈。
