Graeme Greenfield, Ross McMullan, Nuala Robson, Julie McGimpsey, Mark Catherwood, Mary Frances McMullin
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引用次数: 10
摘要
背景:CML发病机制中的BCR-ABL1融合基因可产生于多种断点。由分别位于BCR基因外显子13和外显子14附近的断点形成的e13a2和e14a2转录本是最常见的。方法:我们使用当地实验室数据库和电子患者护理记录对本地区人群中69例e13a2或e14a2转录型CML患者进行回顾性审核。结果:与e14a2组相比，e13a2组明显年轻(45.0岁vs 54.5岁)，平均白细胞计数较高(189.8 × 109/l vs 92.40 × 109/l)，血小板计数较低(308 × 109/l vs 644 × 109/l)，这表明它们是不同的生物实体。e13a2组和e14a2组的平均随访时间分别为33.8个月和27.2个月，我们观察到e13a2组对伊马替尼的分子反应较差。e13a2组在12个月治疗时达到欧洲白血病网最佳反应标准的患者数量明显较低(17.64% v 50.0%)，并且获得MR4或MR4.5深层分子反应的速度较慢。结论:在我们的地区人群中，e13a2转录本患者对伊马替尼的分子反应较差。
Response to Imatinib therapy is inferior for e13a2 BCR-ABL1 transcript type in comparison to e14a2 transcript type in chronic myeloid leukaemia.
Background: The BCR-ABL1 fusion gene underlying the pathogenesis of CML can arise from a variety of breakpoints. The e13a2 and e14a2 transcripts formed by breakpoints occurring around exon 13 and exon 14 of the BCR gene respectively are the most common.
Methods: We undertook a retrospective audit using local laboratory database and electronic patient care records of 69 CML patients with an e13a2 or e14a2 transcript type identified in our regional population.
Results: The e13a2 group was on average significantly younger (45.0 years v 54.5 years), had a higher average white cell count (189.8 × 109/l v 92.40 × 109/l) and lower platelet count (308 × 109/l v 644 × 109/l) in comparison to the e14a2 group suggesting that these are distinct biological entities. Over an average follow-up of 33.8 months and 27.2 months for the e13a2 and e14a2 groups we observed an inferior molecular response to imatinib in the e13a2 group. A significantly lower number of patients in the e13a2 arm met European Leukemia Net criteria for optimal response at 12 months therapy (17.64% v 50.0%) and were slower to obtain deep molecular responses MR4 or MR4.5.
Conclusion: Patients with an e13a2 transcript demonstrate an inferior molecular response to imatinib in our regional population.
期刊介绍：
BMC Hematology is an open access, peer-reviewed journal that considers articles on basic, experimental and clinical research related to hematology. The journal welcomes submissions on non-malignant and malignant hematological diseases, hemostasis and thrombosis, hematopoiesis, stem cells and transplantation.
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