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MLL-rearranged infant leukaemia: A 'thorn in the side' of a remarkable success story.
Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms ( IF 2.6 ) Pub Date : 2020-05-03 , DOI: 10.1016/j.bbagrm.2020.194564
Siobhan Rice 1 , Anindita Roy 2
Affiliation  

Advances in treatment of childhood leukaemia has led to vastly improved survival rates, however some subtypes such as those characterised by MLL gene rearrangement (MLL-r), especially in infants, continue to have high relapse rates and poor survival. Natural history and molecular studies indicate that infant acute lymphoblastic leukaemia (ALL) originates in utero, is distinct from childhood ALL, and most cases are caused by MLL-r resulting in an oncogenic MLL fusion protein. Unlike childhood ALL, only a very small number of additional mutations are present in infant ALL, indicating that MLL-r alone may be sufficient to give rise to this rapid onset, aggressive leukaemia in an appropriate fetal cell context. Despite modifications in treatment approaches, the outcome of MLL-r infant ALL has remained dismal and a clear understanding of the underlying biology of the disease is required in order to develop appropriate disease models and more effective therapeutic strategies.

中文翻译:

MLL 重排婴儿白血病:一个非凡成功故事的“刺”。

儿童白血病治疗的进步极大地提高了存活率,但是一些亚型,例如以 MLL 基因重排 (MLL-r) 为特征的亚型,尤其是在婴儿中,仍然具有高复发率和较差的存活率。自然史和分子研究表明,婴儿急性淋巴细胞白血病 (ALL) 起源于子宫,与儿童 ALL 不同,大多数病例是由 MLL-r 导致致癌 MLL 融合蛋白引起的。与儿童 ALL 不同,婴儿 ALL 中仅存在极少数额外突变,表明仅 MLL-r 可能足以在适当的胎儿细胞环境中引起这种快速发作的侵袭性白血病。尽管治疗方法有所改变,
更新日期:2020-05-03
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