[Visceral Vein Thrombosis of Myeloproliferative Neoplasm --Review].

Xia Zhang, Jie Yang, Hong-Ling Hao
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Abstract

Classical myeloproliferative neoplasm (MPN) related thrombosis mainly affects elderly patients and often involves arterial circulation, while, MPN-visceral venous thrombosis (SVT) mainly affects young women, and is closely associated with JAK2V617F mutation but not closely with CALR mutation. The pathogenesis of MPN-SVT is not only related to JAK2V617F mutation and vascular endothelial damage, but also needs further research to determine the machanism. JAK2V617F mutation is the most common in MPN-SVT clinically. Patients with non-cirrhotic SVT need to detect MPN mutation, while the detection of CALR or MPL mutation needs to be combined with clinical judgment. At present, the main treatment strategies of MPN-SVT are JAK inhibitors, supplementation of anticoagulants and treatment of portal hypertension. This article reviews the latest research progress on the epidemiology, pathogenesis, diagnosis and treatment strategies of MPN-SVT.

【骨髓增殖性肿瘤的内脏静脉血栓——综述】。
经典骨髓增生性肿瘤(MPN)相关血栓形成主要影响老年人,常累及动脉循环,而MPN-肺源性静脉血栓形成(SVT)主要影响年轻女性,与JAK2V617F突变密切相关,但与CALR突变关系不密切。MPN-SVT的发病机制不仅与JAK2V617F突变和血管内皮损伤有关,还需要进一步研究确定其机制。JAK2V617F突变在MPN-SVT中最为常见。非肝硬化SVT患者需要检测MPN突变,而CALR或MPL突变的检测需要结合临床判断。目前MPN-SVT的主要治疗策略是JAK抑制剂、补充抗凝剂和治疗门静脉高压症。本文就MPN-SVT的流行病学、发病机制、诊断及治疗策略等方面的最新研究进展作一综述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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