Weill Cornell Medicine, 1305 York Avenue, 8th Floor, Room Y-811, New York, NY 10021, USA.
Hematol Oncol Clin North Am. 2021 Apr;35(2):305-324. doi: 10.1016/j.hoc.2020.11.006. Epub 2021 Jan 5.
Thrombotic, vascular, and bleeding complications are the most frequent causes of morbidity and mortality in myeloproliferative neoplasms (MPNs). The interplay and reciprocal amplification between two factors are considered to lead to thrombosis in MPNs: (1) circulating blood cell-intrinsic abnormalities caused by an MPN driver mutation in their hematopoietic progenitor/stem cells, interacting with vascular endothelial cells, show prothrombotic and proadhesive phenotypes; and (2) a state of usually subclinical systemic inflammation that fuels the thrombotic tendency. Prevention and treatment require maintenance of hematocrit less than 45% and cytoreductive therapy in patients with a high risk for thrombotic and vascular complications.
血栓形成、血管和出血并发症是骨髓增殖性肿瘤（MPN）发病和死亡的最常见原因。两种因素的相互作用和相互放大被认为导致了 MPN 的血栓形成：（1）造血祖细胞/干细胞中 MPN 驱动突变引起的循环血细胞内在异常，与血管内皮细胞相互作用，表现出促血栓形成和促黏附表型；（2）通常为亚临床系统性炎症状态，加剧了血栓形成倾向。预防和治疗需要维持血细胞比容低于 45%，并对有血栓和血管并发症高危风险的患者进行细胞减少治疗。
