Wang Le, Li Julie, Arbitman Leah, Zhang Hailing, Shao Haipeng, Martin Michael, Moscinski Lynn, Song Jinming
Department of Hematology and Oncology, Guthrie Clinic Cancer Center, Robert Packer Hospital, Sayre, PA 18840, USA.
Department of Hematopathology and Lab Medicines, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL 33612, USA.
Cancers (Basel). 2025 May 30;17(11):1834. doi: 10.3390/cancers17111834.
Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers characterized by the excessive production of blood cells in the bone marrow. These disorders arise from acquired genetic driver mutations, with or without underlying genetic predispositions, resulting in the uncontrolled production of red blood cells, white blood cells, or platelets. The excessive cell production and abnormal signaling from driver mutations cause chronic inflammation and a higher risk of blood clots and vascular complications. The primary goals of MPN treatment are to induce remission, improve quality of life and survival, as well as to reduce the risk of complications such as thrombosis, vascular events, and leukemic transformation. This review provides a comprehensive update on the diagnosis and therapeutic advancements in major MPN subtypes, including chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and primary myelofibrosis. It examines these complex diseases from a molecular and evolutionary perspective, highlighting key clinical trials' long-term follow-up and therapies targeting driver mutations that have transformed treatment strategies. Additionally, several important advancements in addressing challenges such as anemia in myelofibrosis, along with promising emerging therapies, are also discussed.
骨髓增殖性肿瘤(MPNs)是一组罕见的血癌,其特征是骨髓中血细胞过度生成。这些疾病源于获得性基因驱动突变,无论有无潜在的遗传易感性,都会导致红细胞、白细胞或血小板的不受控制的生成。驱动突变导致的细胞过度生成和异常信号传导会引起慢性炎症,并增加血栓形成和血管并发症的风险。MPN治疗的主要目标是诱导缓解、改善生活质量和生存率,以及降低诸如血栓形成、血管事件和白血病转化等并发症的风险。本综述全面更新了主要MPN亚型(包括慢性髓性白血病、真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化)的诊断和治疗进展。它从分子和进化的角度审视这些复杂疾病,突出关键临床试验的长期随访以及针对驱动突变的疗法,这些疗法改变了治疗策略。此外,还讨论了在应对诸如骨髓纤维化中的贫血等挑战方面的一些重要进展以及有前景的新兴疗法。