赖氨酸甲基转移酶2A(KMT2A)基因重排(KMT2A-r)是儿童急性淋巴细胞白血病(ALL)的一种高危基因亚型，复发率高，预后不良，使用传统化疗的疗效欠佳，改善儿童KMT2A-r ALL的治疗和预后仍是亟待解决的问题。近年来，随着人们对KMT2A-r机制的深入了解，将KMT2A-r ALL进行了更准确的风险分层，同时在免疫和靶向治疗方面也有了长足的进步。现就儿童KMT2A-r ALL的遗传和生物学特征、风险分层、治疗策略及预后进行综述，以期为临床工作和未来研究提供理论支持。 Lysine methyltransferase 2A(KMT2A) rearrangement (KMT2A-r) is a high-risk gene subtype of acute lymphoblastic leukemia (ALL) in children with a high relapse rate， poor prognosis and suboptimal response to conventional chemotherapy。Improving the treatment and prognosis of childhood KMT2A-r ALL is an urgent problem。In recent years， with an in-depth understanding of the mechanism of KMT2A-r， more accurate risk stratification of KMT2A-r ALL has been carried out， and great progress has also been made in its immune and targeted therapy。In this article， the genetic and biological characteristics， risk stratification， treatment strategies and prognosis of children KMT2A-r ALL were reviewed in order to provide theoretical support for clinical work and future research。
Advances ofKMT2A rearrangment acute lymphoblastic leukemia in children
Lysine methyltransferase 2A(KMT2A) rearrangement (KMT2A-r) is a high-risk gene subtype of acute lymphoblastic leukemia (ALL) in children with a high relapse rate, poor prognosis and suboptimal response to conventional chemotherapy.Improving the treatment and prognosis of childhood KMT2A-r ALL is an urgent problem.In recent years, with an in-depth understanding of the mechanism of KMT2A-r, more accurate risk stratification of KMT2A-r ALL has been carried out, and great progress has also been made in its immune and targeted therapy.In this article, the genetic and biological characteristics, risk stratification, treatment strategies and prognosis of children KMT2A-r ALL were reviewed in order to provide theoretical support for clinical work and future research.
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