Todor Samuel Bogdan, Ichim Cristian, Boicean Adrian, Mihaila Romeo Gabriel
Faculty of Medicine, Lucian Blaga University of Sibiu, 550169 Sibiu, Romania.
Curr Issues Mol Biol. 2024 Aug 2;46(8):8407-8423. doi: 10.3390/cimb46080496.
Myeloproliferative neoplasms (MPNs), encompassing disorders like polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are characterized by clonal hematopoiesis without the Philadelphia chromosome. The JAK2 V617F mutation is prevalent in PV, ET, and PMF, while mutations in MPL and CALR also play significant roles. These conditions predispose patients to thrombotic events, with PMF exhibiting the lowest survival among MPNs. Chronic inflammation, driven by cytokine release from aberrant leukocytes and platelets, amplifies cardiovascular risk through various mechanisms, including atherosclerosis and vascular remodeling. Additionally, MPN-related complications like pulmonary hypertension and cardiac fibrosis contribute to cardiovascular morbidity and mortality. This review consolidates recent research on MPNs' cardiovascular implications, emphasizing thrombotic risk, chronic inflammation, and vascular stiffness. Understanding these associations is crucial for developing targeted therapies and improving outcomes in MPN patients.
骨髓增殖性肿瘤（MPN）包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF）等疾病，其特征是无费城染色体的克隆性造血。JAK2 V617F突变在PV、ET和PMF中普遍存在，而MPL和CALR的突变也起着重要作用。这些疾病使患者易发生血栓事件，其中PMF在MPN中生存率最低。由异常白细胞和血小板释放的细胞因子驱动的慢性炎症，通过多种机制（包括动脉粥样硬化和血管重塑）增加心血管风险。此外，MPN相关并发症如肺动脉高压和心脏纤维化会导致心血管疾病的发病率和死亡率。本综述整合了近期关于MPN心血管影响的研究，重点强调血栓形成风险、慢性炎症和血管僵硬。了解这些关联对于开发针对性治疗方法和改善MPN患者的治疗结果至关重要。
