Ambayya Angeli, Razali Rozaimi, Sulong Sarina, Zulkefli Ezzanie Suffya, Yap Yee Yee, Sathar Jameela, Hassan Rosline
Department of Haematology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian 16150, Malaysia.
Clinical Haematology Referral Laboratory, Haematology Department, Hospital Ampang, Ministry of Health Malaysia, Ampang 68000, Malaysia.
Cancers (Basel). 2023 Feb 22;15(5):1386. doi: 10.3390/cancers15051386.
Characterising genomic variants is paramount in understanding the pathogenesis and heterogeneity of normal-karyotype acute myeloid leukaemia (AML-NK). In this study, clinically significant genomic biomarkers were ascertained using targeted DNA sequencing and RNA sequencing on eight AML-NK patients' samples collected at disease presentation and after complete remission. In silico and Sanger sequencing validations were performed to validate variants of interest, and they were followed by the performance of functional and pathway enrichment analyses for overrepresentation analysis of genes with somatic variants. Somatic variants involving 26 genes were identified and classified as follows: 18/42 (42.9%) as pathogenic, 4/42 (9.5%) as likely pathogenic, 4/42 (9.5%) as variants of unknown significance, 7/42 (16.7%) as likely benign and 9/42 (21.4%) as benign. Nine novel somatic variants were discovered, of which three were likely pathogenic, in the CEBPA gene with significant association with its upregulation. Transcription misregulation in cancer tops the affected pathways involving upstream genes (CEBPA and RUNX1) that were deregulated in most patients during disease presentation and were closely related to the most enriched molecular function gene ontology category, DNA-binding transcription activator activity RNA polymerase II-specific (GO:0001228). In summary, this study elucidated putative variants and their gene expression profiles along with functional and pathway enrichment in AML-NK patients.
表征基因组变异对于理解正常核型急性髓系白血病(AML-NK)的发病机制和异质性至关重要。在本研究中,通过对8例AML-NK患者疾病初发时和完全缓解后采集的样本进行靶向DNA测序和RNA测序,确定了具有临床意义的基因组生物标志物。进行了计算机模拟和桑格测序验证以验证感兴趣的变异,随后对具有体细胞变异的基因进行功能和通路富集分析以进行过表达分析。鉴定出涉及26个基因的体细胞变异,并分类如下:18/42(42.9%)为致病性变异,4/42(9.5%)为可能致病性变异,4/42(9.5%)为意义未明变异,7/42(16.7%)为可能良性变异,9/42(21.4%)为良性变异。发现了9个新的体细胞变异,其中3个在CEBPA基因中可能具有致病性,且与该基因的上调显著相关。癌症中的转录调控异常在受影响的通路中位居首位,这些通路涉及上游基因(CEBPA和RUNX1),在大多数患者疾病初发时这些基因失调,并且与最富集的分子功能基因本体类别“RNA聚合酶II特异性DNA结合转录激活因子活性”(GO:0001228)密切相关。总之,本研究阐明了AML-NK患者中假定的变异及其基因表达谱以及功能和通路富集情况。