Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA.
Curr Hematol Malig Rep. 2024 Jun;19(3):111-119. doi: 10.1007/s11899-024-00729-8. Epub 2024 Mar 5.
Summarize best practices for management of patients with early myelofibrosis (MF).
Myelofibrosis is a progressive myeloproliferative neoplasm (MPN) that generally produces burdensome symptoms and ultimately leads to worse overall survival than that observed in healthy controls or patients with other MPNs. Several Janus kinase inhibitors and various interferon formulations are now available for treatment of MF, with ruxolitinib notable for extending overall survival in addition to improving MF signs and symptoms. The chronic nature of the disease can lead some patients to avoid immediate treatment in favor of a watch-and-wait approach. This review summarizes the patient management approach taken in my practice, providing guidance and a discussion of best practices with an emphasis on the importance and clinical benefits of active treatment in early MF. In particular, a case is made to consider treatment with ruxolitinib for patients with intermediate-1 risk disease and to minimize delay between diagnosis and treatment initiation for patients with intermediate or high-risk disease.
总结早期骨髓纤维化(MF)患者管理的最佳实践。
MF 是一种进行性骨髓增生性肿瘤(MPN),通常会产生沉重的症状,最终导致总生存率比健康对照或其他 MPN 患者更差。目前有几种 Janus 激酶抑制剂和多种干扰素制剂可用于治疗 MF,其中芦可替尼除了改善 MF 体征和症状外,还能延长总生存率。该疾病的慢性性质可能导致一些患者避免立即治疗,而倾向于观望等待的方法。本综述总结了我在实践中采取的患者管理方法,提供了指导和最佳实践的讨论,重点强调了早期 MF 中积极治疗的重要性和临床益处。特别是,对于中危-1 风险疾病的患者,建议考虑使用芦可替尼治疗,并对于中危或高危疾病的患者,尽量减少诊断与治疗开始之间的时间延迟。