| JAK2V617F突变在骨髓增殖性肿瘤中的研究进展 |
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| 作者姓名： | Chen YX Li Y Zhang LY Liu B |
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| 作者单位： | 1. 山东大学附属省立医院血液科,山东济南,250021
2. 西安交通大学医学院第一附属医院骨科,陕西西安,710061 |
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| 摘 要： | 骨髓增殖性肿瘤(myeloproliferative neoplasms,MPN)是骨髓中一种或多种髓系细胞增殖和外周血中成熟及不成熟细胞计数增加的克隆性造血干/祖细胞疾病.JAK2 V617F突变作为近年MPN研究领域最重要的发现,被认为与MPN发病机制密切相关.突变的JAK2失去自我抑制能力,持续活化导致一系列下游信号转导通路失调,最终造成恶性细胞过度增殖.目前,关于JAK2V617F突变与MPN临床特征及转归方面的研究及针对JAK2 V617F的靶向治疗已取得一定进展.但是仍存在一些待解决问题,如JAK2V617F突变在MPN发病中的作用及具体机制有待进一步研究;应积极开发针对JAK2的有效靶向药物;突变对于临床诊断及疾病检测的应用也亟待深入研究等.本文从JAK2及JAK2 V617F突变介绍JA K2 V617突变在MPN中的致病机制、突变与临床特征及转归之间的关系和相关靶向治疗的发展等,对最新研究进展作一综述.
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| 关 键 词： | 骨髓增殖性肿瘤 JAK2V617F突变 致病机理 临床特征 |
Progress of study on JAK2V617F mutation in myeloproliferative neoplasm |
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| Chen YX,Li Y,Zhang LY,Liu B.Progress of study on JAK2V617F mutation in myeloproliferative neoplasm[J].Journal of Experimental Hematology,2011,19(5):1329-1333. |
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| Authors: | Chen Yi-Xin Li Ying Zhang Ling-Yan Liu Bin |
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| Institution: | (Department of Hematology, Shandong University Affiliated Provincial Hospital, Jinan 250021, Shandong Province, China; 1Department of Orthopaedics, First Affiliated Hospital of Xi′an Jiaotong University Medical School, Xi′an 710061, Shaanxi Province, China) |
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| Abstract: | Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell diseases characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased mature and immature cells in peripheral blood. As the most important discovery in recent studies of MPN, JAk2V617F mutation is considered to closely relate with the pathogenesis of MPN. The mutated JAK2 lost self-inhibition, and then, the sustained activation leads to a series of disorders in downstream signal transduction pathway... |
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| Keywords: | myeloproliferative neoplasms JAK2V617F mutation pathogenesis clinical features |
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