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.
题目: 骨髓增殖性肿瘤的内脏静脉血栓形成.
摘要: 经典的骨髓增殖性肿瘤（MPN）相关血栓主要影响老年患者并且常累及动脉循环，但MPN患者相关内脏静脉血栓形成（MPN-SVT）主要影响年轻女性，且与JAK2V617F突变密切相关，而与CALR突变的关联性很低。MPN-SVT的发病除了与JAK2V617F突变和血管内皮损伤相关之外，尚需进一步研究确定其他参与机制。临床上JAK2V617F突变在MPN-SVT中最常见。非肝硬化SVT患者需检测MPN突变，而CALR或MPL突变的检测需要结合临床。目前MPN-SVT的主要治疗策略为应用JAK抑制剂、抗凝剂和治疗门静脉高压症。本文就有关MPN-SVT的流行病学、发病机制、诊断和治疗策略的最新研究进展作一综述.
Keywords: JAK2V617F mutation; myeloproliferative neoplasm; polycythemia vera; visceral venous thrombosis.