骨髓纤维化（MF）是一种寿命缩短的骨髓增生性肿瘤，具有多种特征，如克隆增殖、纤维化和脾肿大。直到最近，Janus Kinase (JAK) 1/2 抑制剂鲁索利尼布是唯一一种被批准用于MF中无法进行移植的患者并需要治疗症状和/或脾肿大的靶向治疗。然而，鲁索利尼布在3至5年的停药率很高，主要是由于失去疗效或毒性，这些患者没有随后的治疗。在2019年8月，选择性JAK2抑制剂费特拉替尼被美国食品和药物管理局批准用于中度2或高危主要或继发性MF的治疗，无论是否先前接受JAK抑制剂治疗，并用于管理症状和脾肿大。在本文中，我们讨论了费特拉替尼的开发、药理学和药代动力学以及临床开发和未来方向。我们使用pubmed搜索与费特拉替尼和骨髓纤维化相关的文章。费特拉替尼为无法继续使用鲁索利尼布的MF患者提供了第二线治疗。新的JAK抑制剂与其他靶向治疗的组合是必不可少的，以改善MF的管理。

: Myelofibrosis (MF) is a life shortening myeloproliferative neoplasm that has multiple features such as clonal proliferation, fibrosis and splenomegaly. Until recently, ruxolitinib, a Janus Kinase (JAK) 1/2 inhibitor was the only targeted therapy approved for transplant-ineligible patients with MF and who require treatment for symptoms and/or splenomegaly. However, the discontinuation rate with ruxolitinib at 3 to 5 years is high and mostly due to loss of response or toxicity and these patients had no subsequent treatment.: Fedratinib, a selective JAK2 inhibitor, was approved by the Food and Drug Administration (FDA) in August 2019 for the treatment of intermediate-2 or high-risk primary or secondary MF regardless of prior JAK inhibitor treatment for the management of symptoms and splenomegaly. We discuss herein the development of fedratinib, its pharmacology and pharmacokinetics as well as the clinical development and the future directions. We used pubmed for the search of articles related to fedratinib and myelofibrosis.: Fedratinib provided a second-line treatment for patients with MF who failed or discontinued ruxolitinib. New combinations of JAK inhibitors with other targeted therapies are a must in order to improve the management of MF..