Clinical characteristics and prognostic significance of chronic myeloid leukemia with rare BCR-ABL1 transcripts
Clinical characteristics and prognostic significance of chronic myeloid leukemia with rare BCR-ABL1 transcripts
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罕见BCR-ABL1转录本的慢性粒细胞白血病的临床特征及预后意义
DOI：
10.1080/10428194.2019.1607329
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发表时间：
2019-06-27
影响因子：
2.6
通讯作者：
Chen, Suning
中科院分区：
文献类型：
--
作者：
Xue, Mengxing;Wang, Qinrong;Chen, Suning
摘要在酪氨酸激酶抑制剂（TKI）时代，罕见BCR-ABL 1转录本的预后意义尚不确定。在这项回顾性研究中，从2331例慢性粒细胞白血病（CML）患者队列中确定了40例（1.7%）罕见BCR-ABL 1转录本患者;确定了4种罕见转录本，包括e1 a2（0.9%）、e19 a2（0.4%）、e13 a3（0.1%）和e14 a3（0.3%）。与具有典型转录本的患者相比，具有e1 a2转录本的患者对TKI的反应较差，结果更差。携带e19 a2转录本的患者对TKI的早期最佳应答率较高，但其中大多数患者后来因BCR-ABL 1突变而丧失了完全细胞遗传学应答（CCyR），导致预后不良。携带e13 a3/e14 a3转录本的患者对TKI反应良好，结局良好。这些发现表明，在TKI时代确定CML患者的治疗时，应考虑BCR-ABL 1转录本的类型。
Abstract The prognostic significance of rare BCR-ABL1 transcripts is uncertain in the tyrosine kinase inhibitor (TKI) era. In this retrospective study, 40 (1.7%) patients with rare BCR-ABL1 transcripts were identified from a cohort of 2331 chronic myeloid leukemia (CML) patients; 4 types of rare transcripts were identified, including e1a2 (0.9%), e19a2 (0.4%), e13a3 (0.1%), and e14a3 (0.3%). Compared to patients with the typical transcript, those with the e1a2 transcript had an inferior response to TKIs and a worse outcome. Patients with the e19a2 transcript had a high rate of early optimal response to TKIs, but most of them later lost the complete cytogenetic response (CCyR) due to BCR-ABL1 mutations, resulting in a poor prognosis. Patients with the e13a3/e14a3 transcript responded well to TKIs and had a good outcome. These findings indicate that the type of BCR-ABL1 transcript should be considered when determining the treatment for CML patients in the TKI era.