Ishikawa Yuichi, Kiyoi Hitoshi, Tsujimura Akane, Miyawaki Shuichi, Miyazaki Yasushi, Kuriyama Kazutaka, Tomonaga Masao, Naoe Tomoki
Department of Infectious Diseases, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Eur J Haematol. 2009 Aug;83(2):90-8. doi: 10.1111/j.1600-0609.2009.01261.x. Epub 2009 Mar 21.
Acute myeloid leukemia (AML) has been thought to be the consequence of two broad complementation classes of mutations: class I and class II. However, overlap-mutations between them or within the same class and the position of TP53 mutation are not fully analyzed. We comprehensively analyzed the FLT3, cKIT, N-RAS, C/EBPA, AML1, MLL, NPM1, and TP53 mutations in 144 newly diagnosed de novo AML. We found 103 of 165 identified mutations were overlapped with other mutations, and most overlap-mutations consisted of class I and class II mutations. Although overlap-mutations within the same class were found in seven patients, five of them additionally had the other class mutation. These results suggest that most overlap-mutations within the same class might be the consequence of acquiring an additional mutation after the completion both of class I and class II mutations. However, mutated genes overlapped with the same class were limited in N-RAS, TP53, MLL-PTD, and NPM1, suggesting the possibility that these irregular overlap-mutations might cooperatively participate in the development of AML. Notably, TP53 mutation was overlapped with both class I and class II mutations, and associated with morphologic multilineage dysplasia and complex karyotype. The genotype consisting of complex karyotype and TP53 mutation was an unfavorable prognostic factor in entire AML patients, indicating this genotype generates a disease entity in de novo AML. These results collectively suggest that TP53 mutation might be a functionally distinguishable class of mutation.
急性髓系白血病(AML)被认为是两类广泛的互补性突变的结果:I类和II类。然而,它们之间或同一类内的重叠突变以及TP53突变的位置尚未得到充分分析。我们全面分析了144例新诊断的初发AML患者的FLT3、cKIT、N-RAS、C/EBPA、AML1、MLL、NPM1和TP53突变。我们发现,在165个已鉴定的突变中,有103个与其他突变重叠,且大多数重叠突变由I类和II类突变组成。虽然在7例患者中发现了同一类内的重叠突变,但其中5例还存在另一类突变。这些结果表明,同一类内的大多数重叠突变可能是在I类和II类突变均完成后获得额外突变的结果。然而,与同一类重叠的突变基因在N-RAS、TP53、MLL-PTD和NPM1中有限,提示这些不规则的重叠突变可能协同参与AML的发生发展。值得注意的是,TP53突变与I类和II类突变均有重叠,并与形态学多系发育异常和复杂核型相关。由复杂核型和TP53突变组成的基因型是所有AML患者的不良预后因素,表明这种基因型在初发AML中产生了一种疾病实体。这些结果共同表明,TP53突变可能是一类功能上可区分的突变。