迟 佳,王京华.芦可替尼治疗骨髓纤维化的研究进展[J].转化医学杂志,2021,10(4):272-274
芦可替尼治疗骨髓纤维化的研究进展
Research progress of ruxolitinib in treatment of myelofibrosis
  
DOI:
中文关键词:  骨髓纤维化  芦可替尼  JAK抑制剂  JAK-STAT信号转导  联合治疗
英文关键词:Myelofibrosis  Ruxolitinib  JAK inhibitor  JAK-STAT pathway  Combination therapy
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作者单位
迟 佳 哈尔滨医科大学附属第二医院第六住院部四楼血液科 
王京华 哈尔滨医科大学附属第二医院第六住院部四楼血液科 
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中文摘要:
      骨髓纤维化(myelofibrosis,MF)又称骨髓增殖性肿瘤相关骨髓纤维化,是一组起源于造血干细胞的克隆性疾病,临床上具有脾脏肿大、血细胞计数异常、体质性症状、可向急性白血病转化等特点。其发病机制尚不明确,研究最多的是JAK-信号转导和转录激活因子(Janus kinase-signal transducers and activator of transcription,JAK-STAT)信号通路异常活化及炎症细胞因子异常释放。JAK抑制剂芦可替尼作为首个被批准应用于MF的靶向治疗药物,近十年积累了广泛的临床经验,可显著改善症状、缩小脾脏及延长生存期。以芦可替尼为基础的联合治疗可提高疗效,有望成为治疗MF的新策略。本文就JAK抑制剂芦可替尼治疗MF的研究进展作以综述。
英文摘要:
      Myelofibrosis (MF), which is also known as myeloproliferative neoplasm-associated myelofibrosis is a group of clonal diseases that originated from hematopoietic stem cells, characterized by splenomegaly, abnormal blood cell counts, constitutional symptoms, and risk of acute leukemia. Its pathogenesis is still unclear and there are many studies about the abnormal activation of Janus Kinase-Signal Transducers and Activator of Transcription(JAK-STAT) signal pathway and abnormal release of inflammatory cytokines. Ruxolitinib, a Janus Kinase(JAK) inhibitor, is the first target drug approved for therapy of MF. It could reduce spleen, improve symptoms and prolong survival time. The combination therapy based on ruxolitinib could improve the curative effect, which was expected as a new strategy for MF. This article reviewed the combination of ruxolitinib in the treatment of MF.
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