4.2 Article

Acute myeloid leukemia carrying ETV6 mutations: biologic and clinical features

Journal

HEMATOLOGY
Volume 23, Issue 9, Pages 608-612

Publisher

TAYLOR & FRANCIS LTD
DOI: 10.1080/10245332.2018.1482051

Keywords

ETV6 mutation; AML; fusion gene; ETV6-NTRK3; ETV6-LPXN; ETV6-ANLN; molecular target; targeted therapy

Categories

Funding

  1. National Natural Science Foundation of China [81170492, 81370673]
  2. National High Technology Research and Development Program 863 of People's Republic of China [2012AA022703]
  3. National Key Basic Research Program 973 of People's Republic of China [2010CB732404]
  4. Key Medical Projects of Jiangsu Province [BL2014078]
  5. Key Medical of Jiangsu Province [ZDXKB2016020]

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Objectives: E26 transformation-specific variant 6 gene (ETV6) is one of the most consistently rearranged genes in acute leukaemia. It encodes a principal hematopoietic transcription factor. Methods: We performed a systematic review focusing on the mechanisms responsible for etv6 acquisition, and its effect on the development of AML. We also review the Characteristics of ETV6 mutations and its fusion genes. Finally, for using ETV6 as a molecular target, we discuss future therapeutic approaches available to mitigate the associated disease. Results: ETV6 rearrangements often accompany other molecular mutations. Thirty-three distinct partner bands of ETV6 that contain various fusion genes were detected which plays a vital role in obtaining information about leukaemia genesis. RXDX-101 and PKC412 were reported to be inhibitors of ETV6-NTRK3. Discussion: Future researches are needed to explain how ETV6 mutations act within the microenvironment of leukemic cells and how it affects the progression of leukaemia.

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