Princess Margaret Hospital, University of Toronto, Ontario, Canada.
Blood. 2012 Aug 16;120(7):1367-79. doi: 10.1182/blood-2012-05-399048. Epub 2012 Jun 14.
The discovery of JAK2617F mutation paved the way for the development of small molecule inhibitors of JAK1/2 resulting in first approved JAK1/2 inhibitor, ruxolitinib, for the treatment of patients with myelofibrosis (MF). Although JAK1/2 inhibitor therapy is effective in decreasing the burden of symptoms associated with splenomegaly and MF-related constitutional symptoms, it is neither curative nor effective in reducing the risk of leukemic transformation. Presently, allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for MF. A significant risk of regimen-related toxicities, graft failure, and GVHD are major barriers to the success of HCT in MF. Because of significant HCT-associated morbidity and mortality, divergent opinions regarding its appropriate role in this clinical situation have emerged. In this review, the risk-benefit ratios of modern drug therapy compared with HCT in MF patients are analyzed. A risk-adapted approach individualized to each patient's biologic characteristics and comorbidities is described, which is currently warranted in determining optimal treatment strategies for patients with MF. Inclusion of JAK1/2 inhibitor therapy in future transplant conditioning regimens may provide an opportunity to overcome some of these barriers, resulting in greater success with HCT for MF patients.
JAK2617F 突变的发现为 JAK1/2 的小分子抑制剂的开发铺平了道路,从而导致了首个获批的 JAK1/2 抑制剂鲁索利替尼,用于治疗骨髓纤维化 (MF) 患者。虽然 JAK1/2 抑制剂治疗可有效减轻与脾肿大和 MF 相关的全身症状相关的负担,但它既不能治愈,也不能有效降低白血病转化的风险。目前,异基因造血细胞移植 (HCT) 是 MF 的唯一治愈性治疗方法。HCT 在 MF 中成功的主要障碍是与方案相关的毒性、移植物衰竭和 GVHD 的显著风险。由于 HCT 相关发病率和死亡率较高,对于其在这种临床情况下的适当作用,出现了不同的观点。在这篇综述中,分析了现代药物治疗与 MF 患者 HCT 的风险效益比。描述了一种针对每个患者的生物学特征和合并症的个体化风险适应方法,目前在确定 MF 患者的最佳治疗策略方面是必要的。在未来的移植预处理方案中纳入 JAK1/2 抑制剂治疗可能为克服这些障碍提供机会,从而使 MF 患者的 HCT 获得更大的成功。