Myelofibrosis is a myeloproliferative neoplasm associated with constitutional symptoms, increasing splenomegaly, and worsening cytopenias. Janus kinase (JAK) inhibitors have been used for the treatment of myelofibrosis for several years, but there is a lack of comparative information between those treatments. A systematic review and network meta-analysis was performed on randomized controlled trials in patients with myelofibrosis receiving JAK inhibitor or placebo or control. Primary outcomes were efficacy on spleen volume reduction and total symptom score reduction. Additional analyses were conducted on anemia and thrombopenia events. Seven studies were included in the network meta-analysis including 1953 patients randomly assigned to four JAK inhibitors—ruxolitinib, fedratinib, pacritinib, momelotinib—or control. In first-line therapy, momelotinib and fedratinib were associated with comparable efficacy to ruxolitinib, and with less toxicity on erythrocytes and platelets, respectively. Pacritinib was less effective on splenomegaly than ruxolitinib as a first-line treatment but seemed effective in second line, after ruxolitinib exposure. Fedratinib and ruxolitinib that are FDA approved in myelofibrosis have both confirmed being valuable option to treat splenomegaly and constitutional symptoms, and their slightly different tolerance-profiles can guide therapeutic choice for first-line treatment, according to patient profile. Momelotinib could be another option especially due to its positive effect on anemia.

骨髓纤维化是一种骨髓增生性肿瘤，伴有全身症状、脾肿大增加和血细胞减少恶化。Janus 激酶 (JAK) 抑制剂多年来一直用于治疗骨髓纤维化，但缺乏这些治疗方法之间的比较信息。对接受 JAK 抑制剂或安慰剂或对照的骨髓纤维化患者的随机对照试验进行了系统评价和网络荟萃分析。主要结果是对脾脏体积减少和总症状评分降低的疗效。对贫血和血小板减少事件进行了额外的分析。七项研究被纳入网络荟萃分析，其中 1953 名患者被随机分配到四种 JAK 抑制剂（鲁索替尼、费德拉替尼、帕克替尼、莫米洛替尼）或对照组。在一线治疗中，momelotinib 和 fedratinib 的疗效与 ruxolitinib 相当，对红细胞和血小板的毒性分别较小。作为一线治疗，帕克替尼对脾肿大的疗效不如鲁索替尼，但在鲁索替尼暴露后，在二线治疗中似乎有效。根据患者情况，FDA 批准用于治疗骨髓纤维化的 Fedratinib 和 ruxolitinib 均已证实是治疗脾肿大和全身症状的有价值选择，它们的耐受性略有不同，可以指导一线治疗的治疗选择。莫莫替尼可能是另一种选择，尤其是由于其对贫血的积极作用。作为一线治疗，帕克替尼对脾肿大的疗效不如鲁索替尼，但在鲁索替尼暴露后，在二线治疗中似乎有效。根据患者情况，FDA 批准用于治疗骨髓纤维化的 Fedratinib 和 ruxolitinib 均已证实是治疗脾肿大和全身症状的有价值选择，它们的耐受性略有不同，可以指导一线治疗的治疗选择。莫莫替尼可能是另一种选择，尤其是由于其对贫血的积极作用。作为一线治疗，帕克替尼对脾肿大的疗效不如鲁索替尼，但在鲁索替尼暴露后，在二线治疗中似乎有效。根据患者情况，FDA 批准用于治疗骨髓纤维化的 Fedratinib 和 ruxolitinib 均已证实是治疗脾肿大和全身症状的有价值选择，它们的耐受性略有不同，可以指导一线治疗的治疗选择。莫莫替尼可能是另一种选择，尤其是由于其对贫血的积极作用。根据患者的情况，它们稍有不同的耐受情况可以指导一线治疗的治疗选择。莫莫替尼可能是另一种选择，尤其是由于其对贫血的积极作用。根据患者的情况，它们稍有不同的耐受情况可以指导一线治疗的治疗选择。莫莫替尼可能是另一种选择，尤其是由于其对贫血的积极作用。