Department of Hematologic Oncology and Blood Disorders, Atrium Health Levine Cancer Institute, Wake Forest University School of Medicine, Charlotte, NC, USA.
Department of Biostatistics and Data Sciences, Levine Cancer Institute, Atrium Health, Charlotte, NC, USA.
Ann Hematol. 2024 Aug;103(8):2837-2843. doi: 10.1007/s00277-024-05735-7. Epub 2024 Apr 25.
Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by unregulated red blood cell production resulting in elevated hemoglobin and/or hematocrit levels. Patients often have symptoms such as fatigue, pruritus, and painful splenomegaly, but are also at risk of thrombosis, both venous and arterial. Ruxolitinib, a selective Janus kinase inhibitor, is approved by the US Food and Drug Administration as second-line cytoreductive treatment after intolerance or inadequate response to hydroxyurea. Although ruxolitinib has been widely used in this setting, limited data exist in the literature on ruxolitinib treatment patterns and outcomes among patients with PV in routine clinical practice. We report a retrospective, observational, cohort study of patients treated for PV with ruxolitinib across three US centers (academic and regional practice) from December 2014-December 2019. The study included 69 patients, with a median follow-up duration of 3.7 years (95% CI, 2.9-4.4). Our data demonstrate very high rates of hematocrit control (88% of patients by three months and 89% by six months); few patients required dose adjustments or suspension. No arterial thromboses were observed; however, the follow-up duration does not allow for the generation of meaningful conclusions from this. Three patients had thrombotic events; one was in the setting of a second malignancy, one post-operative, and a third related to prolonged immobility. We also found that 28% of patients initiated ruxolitinib as a result of poorly controlled platelet counts, second only to hydroxyurea intolerance (46%) as a reason to start therapy. In clinical practice, ruxolitinib continues to be effective in controlling hematocrit levels after three and six months of treatment in patients and is associated with low thrombotic risk.
真性红细胞增多症 (PV) 是一种骨髓增殖性肿瘤,其特征是红细胞不受调控地生成,导致血红蛋白和/或血细胞比容水平升高。患者常出现疲劳、瘙痒和脾肿大疼痛等症状,但也有静脉和动脉血栓形成的风险。鲁索利替尼是一种选择性 Janus 激酶抑制剂,已被美国食品和药物管理局批准为羟基脲不耐受或治疗反应不足的二线细胞减少治疗药物。尽管鲁索利替尼已在该领域广泛应用,但在常规临床实践中,关于 PV 患者接受鲁索利替尼治疗的模式和结果的文献数据有限。我们报告了一项回顾性、观察性队列研究,该研究纳入了 2014 年 12 月至 2019 年 12 月期间在三个美国中心(学术和区域实践)接受鲁索利替尼治疗的 PV 患者。该研究纳入了 69 名患者,中位随访时间为 3.7 年(95%CI,2.9-4.4)。我们的数据显示,血细胞比容控制率非常高(三个月时 88%的患者达到控制目标,六个月时 89%的患者达到控制目标);很少有患者需要调整剂量或暂停治疗。未观察到动脉血栓形成;然而,随访时间不足以从中得出有意义的结论。有 3 名患者发生了血栓事件;其中 1 例发生在第二恶性肿瘤的背景下,1 例发生在术后,第 3 例与长时间不动有关。我们还发现,有 28%的患者因血小板计数控制不佳而开始使用鲁索利替尼,仅次于因羟基脲不耐受(46%)而开始治疗。在临床实践中,鲁索利替尼在治疗 3 个月和 6 个月后继续有效地控制患者的血细胞比容水平,且血栓形成风险较低。