骨髓增殖性肿瘤的内脏静脉血栓形成

Visceral Vein Thrombosis of Myeloproliferative Neoplasm——Review

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。

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.