[JAK inhibitors for myelofibrosis: ruxolitinib and momelotinib].

Hiroshi Kosugi
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引用次数: 0

Abstract

Myelofibrosis should be diagnosed according to the WHO classification (2022, 5th Ed.) and International Consensus Conference 2022 criteria. Testing for driver mutations in the three genes JAK2, CALR, and MPL is recommended to ensure a definitive diagnosis. Ruxolitinib is the only JAK inhibitor currently approved in Japan, but momelotinib is under regulatory review. The MOMENTUM study showed similar spleen volume reduction at 24 weeks and MFSAF-TSS reduction as the COMFORT study of ruxolitinib. Momelotinib acts on ACVR1 and, therefore, improves anemia through suppression of hepcidin. Anemia and/or transfusion dependency are known to be associated with overall survival duration. Consequently, supportive care measures such as ESA and danazol in lieu of transfusion should be considered in addition to JAK inhibitor selection. Mean survival after discontinuation of JAK inhibitors is 11 to 14 months. Pacritinib (not approved in Japan) is suitable for MF patients with thrombocytopenia. JAK inhibitor selection and supportive care by ESA or danazol in lieu of transfusion should be considered. Many classes of drugs other than JAK inhibitors for myelofibrosis are under investigation.

[治疗骨髓纤维化的 JAK 抑制剂:鲁索利替尼和莫美罗替尼]。
骨髓纤维化应根据世卫组织分类(2022年,第5版)和2022年国际共识会议标准进行诊断。建议检测 JAK2、CALR 和 MPL 三种基因的驱动突变,以确保确诊。鲁索利替尼是目前日本批准的唯一一种 JAK 抑制剂,但莫美洛替尼正在接受监管审查。MOMENTUM研究显示,24周时脾脏体积缩小和MFSAF-TSS缩小的情况与Ruxolitinib的COMFORT研究相似。莫迈罗替尼作用于 ACVR1,因此可通过抑制血色素改善贫血。众所周知,贫血和/或输血依赖与总生存期有关。因此,除了选择JAK抑制剂外,还应考虑ESA和达那唑等支持性治疗措施来代替输血。停用JAK抑制剂后的平均生存期为11至14个月。帕克替尼(未在日本获批)适用于血小板减少的 MF 患者。应考虑选择JAK抑制剂,并使用ESA或达那唑代替输血进行支持治疗。除JAK抑制剂外,还有许多治疗骨髓纤维化的药物正在研究中。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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