Department of Haematology, The Affiliated Zhuzhou Hospital, XiangYa Medical College, Central South University, Zhuzhou, Hunan, China.
J Gene Med. 2021 Jan;23(1):e3276. doi: 10.1002/jgm.3276. Epub 2020 Oct 1.
The genetic changes in chronic myeloid leukaemia (CML) have been well established, although challenges persist in cases with rare fusion transcripts or complex variant translocations. Here, we present a CML patient with e14a3 BCR-ABL1 transcript and t(9;22;12) variant Philadelphia (Ph) chromosome.
Cytogenetic analysis and fluorescence in situ hybridization (FISH) was performed to identify the chromosomal aberrations and gene fusions. Rare fusion transcript was verified by a reverse transcription-polymerase chain reaction (RT-PCR). Breakpoints were characterized and validated using Oxford Nanopore Technologies (ONT) (Oxford, UK) and Sanger sequencing, respectively.
The karyotype showed the translocation t(9;22;12)(q34;q11.2;q24) [20] and FISH indicated 40% positive BCR-ABL1 fusion signals. The RT-PCR suggested e14a3 type fusion transcript. The ONT sequencing analysis identified specific positions of translocation breakpoints: chr22:23633040-chr9:133729579, chr12:121567595-chr22:24701405, which were confirmed using Sanger sequencing. The patient achieved molecular remission 3 months after imatinib therapy.
The present study indicates Nanopore sequencing as a valid strategy, which can characterize breakpoints precisely in special clinical cases with atypical structural variations. CML patients with e14a3 transcripts may have good clinical course in the tyrosine kinase inhibitor era, as reviewed here.
慢性髓性白血病(CML)的遗传变化已经得到充分证实,尽管在罕见融合转录本或复杂变异易位的情况下仍存在挑战。在这里,我们报告了一例具有 e14a3 BCR-ABL1 转录本和 t(9;22;12)变异费城(Ph)染色体的 CML 患者。
通过细胞遗传学分析和荧光原位杂交(FISH)来鉴定染色体畸变和基因融合。通过逆转录聚合酶链反应(RT-PCR)来验证罕见融合转录本。使用牛津纳米孔技术(ONT)(英国牛津)和 Sanger 测序分别对断点进行特征描述和验证。
核型显示易位 t(9;22;12)(q34;q11.2;q24)[20],FISH 表明 40%的 BCR-ABL1 融合信号阳性。RT-PCR 提示 e14a3 型融合转录本。ONT 测序分析确定了易位断点的特定位置:chr22:23633040-chr9:133729579,chr12:121567595-chr22:24701405,通过 Sanger 测序得到确认。患者在伊马替尼治疗 3 个月后达到分子缓解。
本研究表明纳米孔测序是一种有效的策略,可以在具有非典型结构变异的特殊临床病例中精确地描述断点。如本文所述,具有 e14a3 转录本的 CML 患者在酪氨酸激酶抑制剂时代可能具有良好的临床病程。